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Nephrotic syndrome

2017-05-25T15:40:18Z

Olufunmilola:

__NOTOC__
{{Nephrotic syndrome}}

'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''

{{CMG}}; {{APM}} {{AE}}{{OO}} {{CZ}}, [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]

==[[Nephrotic syndrome overview|Overview]]==

==[[Nephrotic syndrome classification|Classification]]==

==[[Nephrotic syndrome pathophysiology|Pathophysiology]]==

==[[Nephrotic syndrome causes|Causes]]==

==[[Nephrotic syndrome differential diagnosis|Differentiating Nephrotic Syndrome from other Diseases]]==

==[[Nephrotic syndrome epidemiology and demographics|Epidemiology and Demographics]]==

==[[Nephrotic syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

==Diagnosis==
[[Nephrotic syndrome history and symptoms| History and Symptoms]] | [[Nephrotic syndrome physical examination | Physical Examination]] | [[Nephrotic syndrome laboratory findings|Laboratory Findings]] | [[Nephrotic syndrome biopsy|Biopsy]] | [[Nephrotic syndrome chest X ray|Chest X ray]] | [[Nephrotic syndrome ultrasound|Ultrasound]] | [[Nephrotic syndrome other imaging findings|Other Imaging Findings]]

==Treatment==
[[Nephrotic syndrome medical therapy|Medical Therapy]] | [[Nephrotic syndrome primary prevention|Primary Prevention]] | [[Nephrotic syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Nephrotic syndrome future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Nephrotic syndrome case study one|Case #1]]
{{Nephrology}}
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

[[de:Nephrotisches Syndrom]]
[[es:Síndrome nefrótico]]
[[fr:Syndrome néphrotique]]
[[hr:Nefrotski sindrom]]
[[nl:Nefrotisch syndroom]]
[[ja:ネフローゼ症候群]]
[[pl:Zespół nerczycowy]]
[[pt:Síndrome nefrótica]]
[[sv:Nefrotiskt syndrom]]
[[zh:肾病综合症]]

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Familial Hematuria & Hereditary Nephritis

2017-03-09T21:24:00Z

Olufunmilola: /* Natural History, Complications and Prognosis */

__NOTOC__
{{SI}}
{{CMG}} {{APM}}

{{SK}} Synonym 1; Synonym 2; Synonym 3

==Overview==

The term 'Familial Hematuria and Hereditary Nephritis' refers to a mixed group of inherited kidney disorders which often present in early childhood and adolescence in form of microscopic hematuria [link to hematuria chapter]. The prognosis and associate co-morbidities are highly variable and dependent on the underlying genetic disease. It can be benign as seen in 'Thin Basement-membrane Disease' or lead to early end stage kidney disease as seen in the X-linked form of Alport syndrome [reference].

Unfortunately the term 'Familial Hematuria and Hereditary Nephritis' has been used variably and can be confusing. While the use of 'nephritis' in the term may imply inflammation, the disorders grouped here are NOT inflammatory in nature, nor due they reflect the involvement of a common pathway, inheritance pattern, or pathophysiology.

==Historical Perspective==
*A familial pattern of early childhood hematuria was first reported by Leonard G. Guthrie in 1902 <ref name="pmiddoi:10.1001">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=doi:10.1001 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. In the subsequent years, reports of early childhood hematuria, kidney failure (some associated to deafness or occular abnormalities), and early death in male offsprings emerged <ref name="pmid4613619">{{cite journal| author=Henderson JR| title=Ludwig Wittgenstein and Guy's Hospital. | journal=Guys Hosp Rep | year= 1973 | volume= 122 | issue= 1-2 | pages= 185-93 | pmid=4613619 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4613619 }} </ref>. Initial reports of this familial pattern of hematuria, and kidney failure thought these presentations to be due to pyelonephritis. Later reports, based on kidney biopsies and autopsies, were proposing a glomerulonephritic process to be the primary cause <ref name="pmidDOI: 10.1056">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=DOI: 10.1056 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. Other reports idnetified familial clustering of kidney disease in association with other abnormalities <ref name="pmid15416035">{{cite journal| author=HAWKINS CF, SMITH OE| title=Renal dysplasia in a family with multiple hereditary abnormalities including iliac horns. | journal=Lancet | year= 1950 | volume= 1 | issue= 6609 | pages= 803-8 | pmid=15416035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15416035 }} </ref>. The ever changing hypothesis of the underlying cause is perhaps a reflection of the various underlying disorders which were responsible in those families describe.

==Classification==
The following disorders are commonly grouped under the term 'Familial Hematuria and Hereditary Nephritis':
*Hereditary Disorders of Collagen Type IV:
**Thin Basement Membrane Disease (Benign Familial Hematuria)
**Alport Syndrome
**Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) <ref name="pmid611773">{{cite journal| author=Marinov G, Tzvetkova T| title=Age related differentiation in the local peculiarities of the terminal vascular bed of the lower limb skin. | journal=Verh Anat Ges | year= 1977 | volume= | issue= 71 Pt 1 | pages= 689-95 | pmid=611773 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=611773 }} </ref>
*Hereditary Disorders of Laminin:
**Pierson Syndrome
*Hereditary Disorder of basement membrane or cytoskletal gene regulation:
**Nail-Patella Syndrome (also known as Onychoosteodysplasia, or Turner-Kieser syndrome, or Fong disease)
*Hereditary Disorders of cytoskeletal protein:
**Epstein Syndrome
**Fechtner Syndrome
It is arbitrary and due to historical reasons, why certain disease are commonly referenced in this category, while other forms of inherited kidney disorders of the glomerular filtration barrier are categorized elsewhere. At best, perhaps an early diagnosis of hematuria can be considered a common denominator, setting these disorders apart from most other forms of inherited kidney disorders.

==Causes==
*Alport syndrome can be due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070)
**Compund heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Homozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
**X-linked mutations in the COL4A5 gene on the X-chromosome (X-linked dominant) (OMIM # 301050)
*Thin Basement Membrane Disease, also known as Thin Basement Membrane Nephropathy or Bening Familial Hematuria is due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070) or
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
**Heterozygous mutations in the COL4A1 gene on the chromosome 13q34 (autosomal dominant) (OMIM # 611773)
* Pierson Syndrome:
** Homozygous or compound heterozygous mutation in the LAMB2 gene on chromosome 3p21 (autosomal recessive) (OMIM # 609049)
*Nail-Patella Syndrome:
**Heterozygous mutation in the LIM-homeodomain protein LMX1B on the chromosome 9q33 (autosomal dominant) (OMIM #161200)
*Epstein Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153650)
*Fechtner Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153640)
==Differentiating Familial Hematuria & Hereditary Nephritis from other Diseases==
*Familial Hematuria & Hereditary Nephritis refers to a mixed group of disorders which can significantly overlap with other categories of inherited glomerular kidney disorders (e.g. genetic forms of FSGS). While this differentiation is arbitrary and due to historical reasons, a clear distinction must be made between the familial forms of hematuria referenced here, and other causes of hematuria and kidney disease observered in infants, children, and adolescents:
:*Genetic forms of FSGS, e.g. autosomal recessive nephrotic syndrome of the Finnish type (NPHS1 mutation), podocin related autosomal recessive proteinuria/ESRD (NPHS2 mutation), autosomal dominant FSGS (α-actinine 4, ACTN4 mutation), and others. In genetic forms of FSGS, proteinuria is the hallmark, and hematuria mostly absent. Decline in renal function and endstage kidney disease is highly variable and based on the underlying genetic defect.
:*Inflammatory glomerular disorders, e.g. lupus nephritis, infection related glomerulonephritis and others. These are often accompanied by other clinical and laboratory findings (e.g. ESR, CRP, complement levels, serologies).
:*Interstitial nephritis: Drugs, infectious agents, as well as autoimmune causes of interstitial nephritis
:*Pyelonephritis: Urine culture, and imaging studies to rule out structural abnormalities of the uro-genital tract which can predispose to reflux, obstruction, and recurrent infection should be performed and rules out.

==Epidemiology and Demographics==
* The prevalence of the individual diseases grouped together under Familial Hematuria & Hereditary Nephritis varies and is:
** Alport Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Thin Basement Membrane Disease: approximately [number or range] per 100,000 individuals worldwide.
** Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps: approximately [number or range] per 100,000 individuals worldwide.
** Pierson Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Epstein Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Fechtner Syndrome: approximately [number or range] per 100,000 individuals worldwide

== Natural History, Complications and Prognosis==
*Alport Syndrome:
**The x-linked form of Alport Syndrome is more severe in males, leading to early end stage kidney disease between the ages 15-35 in the majority of affected individuals <ref name="pmid10752524">{{cite journal| author=Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A et al.| title=X-linked Alport syndrome: natural history in 195 families and genotype- phenotype correlations in males. | journal=J Am Soc Nephrol | year= 2000 | volume= 11 | issue= 4 | pages= 649-57 | pmid=10752524 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10752524 }} </ref>. By age 40 almost all male patients have developed end stage kidney disease. In contrary, only about less than one third of females will go to develop end stage kidney disease <ref name="pmid21071975">{{cite journal| author=Kashtan CE, Segal Y| title=Genetic disorders of glomerular basement membranes. | journal=Nephron Clin Pract | year= 2011 | volume= 118 | issue= 1 | pages= c9-c18 | pmid=21071975 | doi=10.1159/000320876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071975 }} </ref>
**In females the x-linked form of Alport syndrome presents later in life, and is less severe <ref name="pmid14514738">{{cite journal| author=Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A et al.| title=X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: a "European Community Alport Syndrome Concerted Action" study. | journal=J Am Soc Nephrol | year= 2003 | volume= 14 | issue= 10 | pages= 2603-10 | pmid=14514738 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14514738 }} </ref>. Nevertheless, end stage kidney disease can reach a prevalence of to 30% in this patient population.
**The autosomal recessive form of Alport syndrome is more severe and presents earlier in life than the autosomal dominat form <ref name="pmid21071975">{{cite journal| author=Kashtan CE, Segal Y| title=Genetic disorders of glomerular basement membranes. | journal=Nephron Clin Pract | year= 2011 | volume= 118 | issue= 1 | pages= c9-c18 | pmid=21071975 | doi=10.1159/000320876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071975 }} </ref>
*Thin Basement Membrane Disease:
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
*Nail-Patella Syndrome:
*Epstein Syndrome:
**This syndrome has been describe as a familial form of nephritis, associated with macrothrombocytopenia, renal failure, and hearing loss <ref name="pmid5011389">{{cite journal| author=Epstein CJ, Sahud MA, Piel CF, Goodman JR, Bernfield MR, Kushner JH et al.| title=Hereditary macrothrombocytopathia, nephritis and deafness. | journal=Am J Med | year= 1972 | volume= 52 | issue= 3 | pages= 299-310 | pmid=5011389 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5011389 }} </ref>. Presentation resembles that of Alport Syndrome, and genetic testing may be required to distinguish these two disorders.
*Fechtner Syndrome:

== Diagnosis ==
===Diagnostic Criteria===
*'Familial Hematuria' or 'Hereditary Nephritis' are clinical presentations rather than a diagnosis. A familial pattern of hematuria, proteinuria and kidney disease may raise the suspicion of a genetic disorder related to the above mentioned structural components of the glomerular filtration barrier (collagen type IV, laminin, cytoskeletal components). Characteristic findings on a kidney biopsy (e.g. thinning and irregularities of the glomerular basement membrane), with or without ocular defects and hearing impairment may further strengthen the suspicion of a genetic defect in collagen IV, laminin, or myosin heavy chain biosynthesis, or cyteskeletal regulating proteins. Kidney biopsy can strenghte the clinical suspicion, however ultimate diagnosis is made through genetic testing.
=== Physical Findings ===
*The X-linked Alport syndrome in males as well as the autosomal recessive form of Alport syndrome are associated with ocular defects (corneal and retinal defects, and anterior lenticonus), and hearing loss during childhood.
=== Laboratory Findings ===
*
===Imaging Findings===
*

=== Other Diagnostic Studies ===
*

== Treatment ==
=== Medical Therapy ===
*

=== Surgery ===
*

=== Prevention ===
*
==References==
{{Reflist|2}}

[[Category:Pick One of 28 Approved]]

{{WS}}
{{WH}}

Familial Hematuria & Hereditary Nephritis

2017-03-09T21:22:31Z

Olufunmilola: /* Natural History, Complications and Prognosis */

__NOTOC__
{{SI}}
{{CMG}} {{APM}}

{{SK}} Synonym 1; Synonym 2; Synonym 3

==Overview==

The term 'Familial Hematuria and Hereditary Nephritis' refers to a mixed group of inherited kidney disorders which often present in early childhood and adolescence in form of microscopic hematuria [link to hematuria chapter]. The prognosis and associate co-morbidities are highly variable and dependent on the underlying genetic disease. It can be benign as seen in 'Thin Basement-membrane Disease' or lead to early end stage kidney disease as seen in the X-linked form of Alport syndrome [reference].

Unfortunately the term 'Familial Hematuria and Hereditary Nephritis' has been used variably and can be confusing. While the use of 'nephritis' in the term may imply inflammation, the disorders grouped here are NOT inflammatory in nature, nor due they reflect the involvement of a common pathway, inheritance pattern, or pathophysiology.

==Historical Perspective==
*A familial pattern of early childhood hematuria was first reported by Leonard G. Guthrie in 1902 <ref name="pmiddoi:10.1001">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=doi:10.1001 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. In the subsequent years, reports of early childhood hematuria, kidney failure (some associated to deafness or occular abnormalities), and early death in male offsprings emerged <ref name="pmid4613619">{{cite journal| author=Henderson JR| title=Ludwig Wittgenstein and Guy's Hospital. | journal=Guys Hosp Rep | year= 1973 | volume= 122 | issue= 1-2 | pages= 185-93 | pmid=4613619 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4613619 }} </ref>. Initial reports of this familial pattern of hematuria, and kidney failure thought these presentations to be due to pyelonephritis. Later reports, based on kidney biopsies and autopsies, were proposing a glomerulonephritic process to be the primary cause <ref name="pmidDOI: 10.1056">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=DOI: 10.1056 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. Other reports idnetified familial clustering of kidney disease in association with other abnormalities <ref name="pmid15416035">{{cite journal| author=HAWKINS CF, SMITH OE| title=Renal dysplasia in a family with multiple hereditary abnormalities including iliac horns. | journal=Lancet | year= 1950 | volume= 1 | issue= 6609 | pages= 803-8 | pmid=15416035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15416035 }} </ref>. The ever changing hypothesis of the underlying cause is perhaps a reflection of the various underlying disorders which were responsible in those families describe.

==Classification==
The following disorders are commonly grouped under the term 'Familial Hematuria and Hereditary Nephritis':
*Hereditary Disorders of Collagen Type IV:
**Thin Basement Membrane Disease (Benign Familial Hematuria)
**Alport Syndrome
**Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) <ref name="pmid611773">{{cite journal| author=Marinov G, Tzvetkova T| title=Age related differentiation in the local peculiarities of the terminal vascular bed of the lower limb skin. | journal=Verh Anat Ges | year= 1977 | volume= | issue= 71 Pt 1 | pages= 689-95 | pmid=611773 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=611773 }} </ref>
*Hereditary Disorders of Laminin:
**Pierson Syndrome
*Hereditary Disorder of basement membrane or cytoskletal gene regulation:
**Nail-Patella Syndrome (also known as Onychoosteodysplasia, or Turner-Kieser syndrome, or Fong disease)
*Hereditary Disorders of cytoskeletal protein:
**Epstein Syndrome
**Fechtner Syndrome
It is arbitrary and due to historical reasons, why certain disease are commonly referenced in this category, while other forms of inherited kidney disorders of the glomerular filtration barrier are categorized elsewhere. At best, perhaps an early diagnosis of hematuria can be considered a common denominator, setting these disorders apart from most other forms of inherited kidney disorders.

==Causes==
*Alport syndrome can be due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070)
**Compund heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Homozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
**X-linked mutations in the COL4A5 gene on the X-chromosome (X-linked dominant) (OMIM # 301050)
*Thin Basement Membrane Disease, also known as Thin Basement Membrane Nephropathy or Bening Familial Hematuria is due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070) or
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
**Heterozygous mutations in the COL4A1 gene on the chromosome 13q34 (autosomal dominant) (OMIM # 611773)
* Pierson Syndrome:
** Homozygous or compound heterozygous mutation in the LAMB2 gene on chromosome 3p21 (autosomal recessive) (OMIM # 609049)
*Nail-Patella Syndrome:
**Heterozygous mutation in the LIM-homeodomain protein LMX1B on the chromosome 9q33 (autosomal dominant) (OMIM #161200)
*Epstein Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153650)
*Fechtner Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153640)
==Differentiating Familial Hematuria & Hereditary Nephritis from other Diseases==
*Familial Hematuria & Hereditary Nephritis refers to a mixed group of disorders which can significantly overlap with other categories of inherited glomerular kidney disorders (e.g. genetic forms of FSGS). While this differentiation is arbitrary and due to historical reasons, a clear distinction must be made between the familial forms of hematuria referenced here, and other causes of hematuria and kidney disease observered in infants, children, and adolescents:
:*Genetic forms of FSGS, e.g. autosomal recessive nephrotic syndrome of the Finnish type (NPHS1 mutation), podocin related autosomal recessive proteinuria/ESRD (NPHS2 mutation), autosomal dominant FSGS (α-actinine 4, ACTN4 mutation), and others. In genetic forms of FSGS, proteinuria is the hallmark, and hematuria mostly absent. Decline in renal function and endstage kidney disease is highly variable and based on the underlying genetic defect.
:*Inflammatory glomerular disorders, e.g. lupus nephritis, infection related glomerulonephritis and others. These are often accompanied by other clinical and laboratory findings (e.g. ESR, CRP, complement levels, serologies).
:*Interstitial nephritis: Drugs, infectious agents, as well as autoimmune causes of interstitial nephritis
:*Pyelonephritis: Urine culture, and imaging studies to rule out structural abnormalities of the uro-genital tract which can predispose to reflux, obstruction, and recurrent infection should be performed and rules out.

==Epidemiology and Demographics==
* The prevalence of the individual diseases grouped together under Familial Hematuria & Hereditary Nephritis varies and is:
** Alport Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Thin Basement Membrane Disease: approximately [number or range] per 100,000 individuals worldwide.
** Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps: approximately [number or range] per 100,000 individuals worldwide.
** Pierson Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Epstein Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Fechtner Syndrome: approximately [number or range] per 100,000 individuals worldwide

== Natural History, Complications and Prognosis==
*Alport Syndrome:
**The x-linked form of Alport Syndrome is more severe in males, leading to early end stage kidney disease between the ages 15-35 in the majority of affected individuals <ref name="pmid10752524">{{cite journal| author=Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A et al.| title=X-linked Alport syndrome: natural history in 195 families and genotype- phenotype correlations in males. | journal=J Am Soc Nephrol | year= 2000 | volume= 11 | issue= 4 | pages= 649-57 | pmid=10752524 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10752524 }} </ref>. By age 40 almost all male patients have developed end stage kidney disease. In contrary, only about less than one third of females will go to develop end stage kidney disease <ref name="pmid21071975">{{cite journal| author=Kashtan CE, Segal Y| title=Genetic disorders of glomerular basement membranes. | journal=Nephron Clin Pract | year= 2011 | volume= 118 | issue= 1 | pages= c9-c18 | pmid=21071975 | doi=10.1159/000320876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071975 }} </ref>
**In females the x-linked form of Alport syndrome presents later in life, and is less severe <ref name="pmid14514738">{{cite journal| author=Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A et al.| title=X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: a "European Community Alport Syndrome Concerted Action" study. | journal=J Am Soc Nephrol | year= 2003 | volume= 14 | issue= 10 | pages= 2603-10 | pmid=14514738 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14514738 }} </ref>. Nevertheless, end stage kidney disease can reach a prevalence of to 30% in this patient population.
**The autosomal recessive form of Alport syndrome is more severe and presents earlier in life than the autosomal dominat form <ref name="pmid21071975">{{cite journal| author=Kashtan CE, Segal Y| title=Genetic disorders of glomerular basement membranes. | journal=Nephron Clin Pract | year= 2011 | volume= 118 | issue= 1 | pages= c9-c18 | pmid=21071975 | doi=10.1159/000320876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071975 }} </ref>
*Thin Basement Membrane Disease:
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
*Nail-Patella Syndrome:
*Epstein Syndrome:
**This syndrome has been describe as a familial form of nephritis, associated with macrothrombocytopenia, renal failure, and hearing loss [PMID: 5011389]. Presentation resembles that of Alport Syndrome, and genetic testing may be required to distinguish these two disorders.
*Fechtner Syndrome:

== Diagnosis ==
===Diagnostic Criteria===
*'Familial Hematuria' or 'Hereditary Nephritis' are clinical presentations rather than a diagnosis. A familial pattern of hematuria, proteinuria and kidney disease may raise the suspicion of a genetic disorder related to the above mentioned structural components of the glomerular filtration barrier (collagen type IV, laminin, cytoskeletal components). Characteristic findings on a kidney biopsy (e.g. thinning and irregularities of the glomerular basement membrane), with or without ocular defects and hearing impairment may further strengthen the suspicion of a genetic defect in collagen IV, laminin, or myosin heavy chain biosynthesis, or cyteskeletal regulating proteins. Kidney biopsy can strenghte the clinical suspicion, however ultimate diagnosis is made through genetic testing.
=== Physical Findings ===
*The X-linked Alport syndrome in males as well as the autosomal recessive form of Alport syndrome are associated with ocular defects (corneal and retinal defects, and anterior lenticonus), and hearing loss during childhood.
=== Laboratory Findings ===
*
===Imaging Findings===
*

=== Other Diagnostic Studies ===
*

== Treatment ==
=== Medical Therapy ===
*

=== Surgery ===
*

=== Prevention ===
*
==References==
{{Reflist|2}}

[[Category:Pick One of 28 Approved]]

{{WS}}
{{WH}}

Familial Hematuria & Hereditary Nephritis

2017-03-09T21:21:23Z

Olufunmilola: /* Natural History, Complications and Prognosis */

__NOTOC__
{{SI}}
{{CMG}} {{APM}}

{{SK}} Synonym 1; Synonym 2; Synonym 3

==Overview==

The term 'Familial Hematuria and Hereditary Nephritis' refers to a mixed group of inherited kidney disorders which often present in early childhood and adolescence in form of microscopic hematuria [link to hematuria chapter]. The prognosis and associate co-morbidities are highly variable and dependent on the underlying genetic disease. It can be benign as seen in 'Thin Basement-membrane Disease' or lead to early end stage kidney disease as seen in the X-linked form of Alport syndrome [reference].

Unfortunately the term 'Familial Hematuria and Hereditary Nephritis' has been used variably and can be confusing. While the use of 'nephritis' in the term may imply inflammation, the disorders grouped here are NOT inflammatory in nature, nor due they reflect the involvement of a common pathway, inheritance pattern, or pathophysiology.

==Historical Perspective==
*A familial pattern of early childhood hematuria was first reported by Leonard G. Guthrie in 1902 <ref name="pmiddoi:10.1001">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=doi:10.1001 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. In the subsequent years, reports of early childhood hematuria, kidney failure (some associated to deafness or occular abnormalities), and early death in male offsprings emerged <ref name="pmid4613619">{{cite journal| author=Henderson JR| title=Ludwig Wittgenstein and Guy's Hospital. | journal=Guys Hosp Rep | year= 1973 | volume= 122 | issue= 1-2 | pages= 185-93 | pmid=4613619 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4613619 }} </ref>. Initial reports of this familial pattern of hematuria, and kidney failure thought these presentations to be due to pyelonephritis. Later reports, based on kidney biopsies and autopsies, were proposing a glomerulonephritic process to be the primary cause <ref name="pmidDOI: 10.1056">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=DOI: 10.1056 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. Other reports idnetified familial clustering of kidney disease in association with other abnormalities <ref name="pmid15416035">{{cite journal| author=HAWKINS CF, SMITH OE| title=Renal dysplasia in a family with multiple hereditary abnormalities including iliac horns. | journal=Lancet | year= 1950 | volume= 1 | issue= 6609 | pages= 803-8 | pmid=15416035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15416035 }} </ref>. The ever changing hypothesis of the underlying cause is perhaps a reflection of the various underlying disorders which were responsible in those families describe.

==Classification==
The following disorders are commonly grouped under the term 'Familial Hematuria and Hereditary Nephritis':
*Hereditary Disorders of Collagen Type IV:
**Thin Basement Membrane Disease (Benign Familial Hematuria)
**Alport Syndrome
**Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) <ref name="pmid611773">{{cite journal| author=Marinov G, Tzvetkova T| title=Age related differentiation in the local peculiarities of the terminal vascular bed of the lower limb skin. | journal=Verh Anat Ges | year= 1977 | volume= | issue= 71 Pt 1 | pages= 689-95 | pmid=611773 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=611773 }} </ref>
*Hereditary Disorders of Laminin:
**Pierson Syndrome
*Hereditary Disorder of basement membrane or cytoskletal gene regulation:
**Nail-Patella Syndrome (also known as Onychoosteodysplasia, or Turner-Kieser syndrome, or Fong disease)
*Hereditary Disorders of cytoskeletal protein:
**Epstein Syndrome
**Fechtner Syndrome
It is arbitrary and due to historical reasons, why certain disease are commonly referenced in this category, while other forms of inherited kidney disorders of the glomerular filtration barrier are categorized elsewhere. At best, perhaps an early diagnosis of hematuria can be considered a common denominator, setting these disorders apart from most other forms of inherited kidney disorders.

==Causes==
*Alport syndrome can be due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070)
**Compund heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Homozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
**X-linked mutations in the COL4A5 gene on the X-chromosome (X-linked dominant) (OMIM # 301050)
*Thin Basement Membrane Disease, also known as Thin Basement Membrane Nephropathy or Bening Familial Hematuria is due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070) or
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
**Heterozygous mutations in the COL4A1 gene on the chromosome 13q34 (autosomal dominant) (OMIM # 611773)
* Pierson Syndrome:
** Homozygous or compound heterozygous mutation in the LAMB2 gene on chromosome 3p21 (autosomal recessive) (OMIM # 609049)
*Nail-Patella Syndrome:
**Heterozygous mutation in the LIM-homeodomain protein LMX1B on the chromosome 9q33 (autosomal dominant) (OMIM #161200)
*Epstein Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153650)
*Fechtner Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153640)
==Differentiating Familial Hematuria & Hereditary Nephritis from other Diseases==
*Familial Hematuria & Hereditary Nephritis refers to a mixed group of disorders which can significantly overlap with other categories of inherited glomerular kidney disorders (e.g. genetic forms of FSGS). While this differentiation is arbitrary and due to historical reasons, a clear distinction must be made between the familial forms of hematuria referenced here, and other causes of hematuria and kidney disease observered in infants, children, and adolescents:
:*Genetic forms of FSGS, e.g. autosomal recessive nephrotic syndrome of the Finnish type (NPHS1 mutation), podocin related autosomal recessive proteinuria/ESRD (NPHS2 mutation), autosomal dominant FSGS (α-actinine 4, ACTN4 mutation), and others. In genetic forms of FSGS, proteinuria is the hallmark, and hematuria mostly absent. Decline in renal function and endstage kidney disease is highly variable and based on the underlying genetic defect.
:*Inflammatory glomerular disorders, e.g. lupus nephritis, infection related glomerulonephritis and others. These are often accompanied by other clinical and laboratory findings (e.g. ESR, CRP, complement levels, serologies).
:*Interstitial nephritis: Drugs, infectious agents, as well as autoimmune causes of interstitial nephritis
:*Pyelonephritis: Urine culture, and imaging studies to rule out structural abnormalities of the uro-genital tract which can predispose to reflux, obstruction, and recurrent infection should be performed and rules out.

==Epidemiology and Demographics==
* The prevalence of the individual diseases grouped together under Familial Hematuria & Hereditary Nephritis varies and is:
** Alport Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Thin Basement Membrane Disease: approximately [number or range] per 100,000 individuals worldwide.
** Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps: approximately [number or range] per 100,000 individuals worldwide.
** Pierson Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Epstein Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Fechtner Syndrome: approximately [number or range] per 100,000 individuals worldwide

== Natural History, Complications and Prognosis==
*Alport Syndrome:
**The x-linked form of Alport Syndrome is more severe in males, leading to early end stage kidney disease between the ages 15-35 in the majority of affected individuals [PMID: 10752524]. By age 40 almost all male patients have developed end stage kidney disease. In contrary, only about less than one third of females will go to develop end stage kidney disease <ref name="pmid21071975">{{cite journal| author=Kashtan CE, Segal Y| title=Genetic disorders of glomerular basement membranes. | journal=Nephron Clin Pract | year= 2011 | volume= 118 | issue= 1 | pages= c9-c18 | pmid=21071975 | doi=10.1159/000320876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071975 }} </ref>
**In females the x-linked form of Alport syndrome presents later in life, and is less severe <ref name="pmid14514738">{{cite journal| author=Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A et al.| title=X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: a "European Community Alport Syndrome Concerted Action" study. | journal=J Am Soc Nephrol | year= 2003 | volume= 14 | issue= 10 | pages= 2603-10 | pmid=14514738 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14514738 }} </ref>. Nevertheless, end stage kidney disease can reach a prevalence of to 30% in this patient population.
**The autosomal recessive form of Alport syndrome is more severe and presents earlier in life than the autosomal dominat form <ref name="pmid21071975">{{cite journal| author=Kashtan CE, Segal Y| title=Genetic disorders of glomerular basement membranes. | journal=Nephron Clin Pract | year= 2011 | volume= 118 | issue= 1 | pages= c9-c18 | pmid=21071975 | doi=10.1159/000320876 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071975 }} </ref>
*Thin Basement Membrane Disease:
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
*Nail-Patella Syndrome:
*Epstein Syndrome:
**This syndrome has been describe as a familial form of nephritis, associated with macrothrombocytopenia, renal failure, and hearing loss [PMID: 5011389]. Presentation resembles that of Alport Syndrome, and genetic testing may be required to distinguish these two disorders.
*Fechtner Syndrome:

== Diagnosis ==
===Diagnostic Criteria===
*'Familial Hematuria' or 'Hereditary Nephritis' are clinical presentations rather than a diagnosis. A familial pattern of hematuria, proteinuria and kidney disease may raise the suspicion of a genetic disorder related to the above mentioned structural components of the glomerular filtration barrier (collagen type IV, laminin, cytoskeletal components). Characteristic findings on a kidney biopsy (e.g. thinning and irregularities of the glomerular basement membrane), with or without ocular defects and hearing impairment may further strengthen the suspicion of a genetic defect in collagen IV, laminin, or myosin heavy chain biosynthesis, or cyteskeletal regulating proteins. Kidney biopsy can strenghte the clinical suspicion, however ultimate diagnosis is made through genetic testing.
=== Physical Findings ===
*The X-linked Alport syndrome in males as well as the autosomal recessive form of Alport syndrome are associated with ocular defects (corneal and retinal defects, and anterior lenticonus), and hearing loss during childhood.
=== Laboratory Findings ===
*
===Imaging Findings===
*

=== Other Diagnostic Studies ===
*

== Treatment ==
=== Medical Therapy ===
*

=== Surgery ===
*

=== Prevention ===
*
==References==
{{Reflist|2}}

[[Category:Pick One of 28 Approved]]

{{WS}}
{{WH}}

Familial Hematuria & Hereditary Nephritis

2017-03-09T21:09:10Z

Olufunmilola: /* Classification */

__NOTOC__
{{SI}}
{{CMG}} {{APM}}

{{SK}} Synonym 1; Synonym 2; Synonym 3

==Overview==

The term 'Familial Hematuria and Hereditary Nephritis' refers to a mixed group of inherited kidney disorders which often present in early childhood and adolescence in form of microscopic hematuria [link to hematuria chapter]. The prognosis and associate co-morbidities are highly variable and dependent on the underlying genetic disease. It can be benign as seen in 'Thin Basement-membrane Disease' or lead to early end stage kidney disease as seen in the X-linked form of Alport syndrome [reference].

Unfortunately the term 'Familial Hematuria and Hereditary Nephritis' has been used variably and can be confusing. While the use of 'nephritis' in the term may imply inflammation, the disorders grouped here are NOT inflammatory in nature, nor due they reflect the involvement of a common pathway, inheritance pattern, or pathophysiology.

==Historical Perspective==
*A familial pattern of early childhood hematuria was first reported by Leonard G. Guthrie in 1902 <ref name="pmiddoi:10.1001">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=doi:10.1001 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. In the subsequent years, reports of early childhood hematuria, kidney failure (some associated to deafness or occular abnormalities), and early death in male offsprings emerged <ref name="pmid4613619">{{cite journal| author=Henderson JR| title=Ludwig Wittgenstein and Guy's Hospital. | journal=Guys Hosp Rep | year= 1973 | volume= 122 | issue= 1-2 | pages= 185-93 | pmid=4613619 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4613619 }} </ref>. Initial reports of this familial pattern of hematuria, and kidney failure thought these presentations to be due to pyelonephritis. Later reports, based on kidney biopsies and autopsies, were proposing a glomerulonephritic process to be the primary cause <ref name="pmidDOI: 10.1056">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=DOI: 10.1056 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. Other reports idnetified familial clustering of kidney disease in association with other abnormalities <ref name="pmid15416035">{{cite journal| author=HAWKINS CF, SMITH OE| title=Renal dysplasia in a family with multiple hereditary abnormalities including iliac horns. | journal=Lancet | year= 1950 | volume= 1 | issue= 6609 | pages= 803-8 | pmid=15416035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15416035 }} </ref>. The ever changing hypothesis of the underlying cause is perhaps a reflection of the various underlying disorders which were responsible in those families describe.

==Classification==
The following disorders are commonly grouped under the term 'Familial Hematuria and Hereditary Nephritis':
*Hereditary Disorders of Collagen Type IV:
**Thin Basement Membrane Disease (Benign Familial Hematuria)
**Alport Syndrome
**Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) <ref name="pmid611773">{{cite journal| author=Marinov G, Tzvetkova T| title=Age related differentiation in the local peculiarities of the terminal vascular bed of the lower limb skin. | journal=Verh Anat Ges | year= 1977 | volume= | issue= 71 Pt 1 | pages= 689-95 | pmid=611773 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=611773 }} </ref>
*Hereditary Disorders of Laminin:
**Pierson Syndrome
*Hereditary Disorder of basement membrane or cytoskletal gene regulation:
**Nail-Patella Syndrome (also known as Onychoosteodysplasia, or Turner-Kieser syndrome, or Fong disease)
*Hereditary Disorders of cytoskeletal protein:
**Epstein Syndrome
**Fechtner Syndrome
It is arbitrary and due to historical reasons, why certain disease are commonly referenced in this category, while other forms of inherited kidney disorders of the glomerular filtration barrier are categorized elsewhere. At best, perhaps an early diagnosis of hematuria can be considered a common denominator, setting these disorders apart from most other forms of inherited kidney disorders.

==Causes==
*Alport syndrome can be due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070)
**Compund heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Homozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
**X-linked mutations in the COL4A5 gene on the X-chromosome (X-linked dominant) (OMIM # 301050)
*Thin Basement Membrane Disease, also known as Thin Basement Membrane Nephropathy or Bening Familial Hematuria is due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070) or
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
**Heterozygous mutations in the COL4A1 gene on the chromosome 13q34 (autosomal dominant) (OMIM # 611773)
* Pierson Syndrome:
** Homozygous or compound heterozygous mutation in the LAMB2 gene on chromosome 3p21 (autosomal recessive) (OMIM # 609049)
*Nail-Patella Syndrome:
**Heterozygous mutation in the LIM-homeodomain protein LMX1B on the chromosome 9q33 (autosomal dominant) (OMIM #161200)
*Epstein Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153650)
*Fechtner Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153640)
==Differentiating Familial Hematuria & Hereditary Nephritis from other Diseases==
*Familial Hematuria & Hereditary Nephritis refers to a mixed group of disorders which can significantly overlap with other categories of inherited glomerular kidney disorders (e.g. genetic forms of FSGS). While this differentiation is arbitrary and due to historical reasons, a clear distinction must be made between the familial forms of hematuria referenced here, and other causes of hematuria and kidney disease observered in infants, children, and adolescents:
:*Genetic forms of FSGS, e.g. autosomal recessive nephrotic syndrome of the Finnish type (NPHS1 mutation), podocin related autosomal recessive proteinuria/ESRD (NPHS2 mutation), autosomal dominant FSGS (α-actinine 4, ACTN4 mutation), and others. In genetic forms of FSGS, proteinuria is the hallmark, and hematuria mostly absent. Decline in renal function and endstage kidney disease is highly variable and based on the underlying genetic defect.
:*Inflammatory glomerular disorders, e.g. lupus nephritis, infection related glomerulonephritis and others. These are often accompanied by other clinical and laboratory findings (e.g. ESR, CRP, complement levels, serologies).
:*Interstitial nephritis: Drugs, infectious agents, as well as autoimmune causes of interstitial nephritis
:*Pyelonephritis: Urine culture, and imaging studies to rule out structural abnormalities of the uro-genital tract which can predispose to reflux, obstruction, and recurrent infection should be performed and rules out.

==Epidemiology and Demographics==
* The prevalence of the individual diseases grouped together under Familial Hematuria & Hereditary Nephritis varies and is:
** Alport Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Thin Basement Membrane Disease: approximately [number or range] per 100,000 individuals worldwide.
** Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps: approximately [number or range] per 100,000 individuals worldwide.
** Pierson Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Epstein Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Fechtner Syndrome: approximately [number or range] per 100,000 individuals worldwide

== Natural History, Complications and Prognosis==
*Alport Syndrome:
**The x-linked form of Alport Syndrome is more severe in males, leading to early end stage kidney disease between the ages 15-35 in the majority of affected individuals [PMID: 10752524]. By age 40 almost all male patients have developed end stage kidney disease. In contrary, only about less than one third of females will go to develop end stage kidney disease [PMID: 21071975]
**In females the x-linked form of Alport syndrome presents later in life, and is less severe [PMID: 14514738]. Nevertheless, end stage kidney disease can reach a prevalence of to 30% in this patient population.
**The autosomal recessive form of Alport syndrome is more severe and presents earlier in life than the autosomal dominat form [PMID: 21071975].
*Thin Basement Membrane Disease:
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
*Nail-Patella Syndrome:
*Epstein Syndrome:
**This syndrome has been describe as a familial form of nephritis, associated with macrothrombocytopenia, renal failure, and hearing loss [PMID: 5011389]. Presentation resembles that of Alport Syndrome, and genetic testing may be required to distinguish these two disorders.
*Fechtner Syndrome:

== Diagnosis ==
===Diagnostic Criteria===
*'Familial Hematuria' or 'Hereditary Nephritis' are clinical presentations rather than a diagnosis. A familial pattern of hematuria, proteinuria and kidney disease may raise the suspicion of a genetic disorder related to the above mentioned structural components of the glomerular filtration barrier (collagen type IV, laminin, cytoskeletal components). Characteristic findings on a kidney biopsy (e.g. thinning and irregularities of the glomerular basement membrane), with or without ocular defects and hearing impairment may further strengthen the suspicion of a genetic defect in collagen IV, laminin, or myosin heavy chain biosynthesis, or cyteskeletal regulating proteins. Kidney biopsy can strenghte the clinical suspicion, however ultimate diagnosis is made through genetic testing.
=== Physical Findings ===
*The X-linked Alport syndrome in males as well as the autosomal recessive form of Alport syndrome are associated with ocular defects (corneal and retinal defects, and anterior lenticonus), and hearing loss during childhood.
=== Laboratory Findings ===
*
===Imaging Findings===
*

=== Other Diagnostic Studies ===
*

== Treatment ==
=== Medical Therapy ===
*

=== Surgery ===
*

=== Prevention ===
*
==References==
{{Reflist|2}}

[[Category:Pick One of 28 Approved]]

{{WS}}
{{WH}}

Familial Hematuria & Hereditary Nephritis

2017-03-09T21:08:45Z

Olufunmilola: /* Classification */

__NOTOC__
{{SI}}
{{CMG}} {{APM}}

{{SK}} Synonym 1; Synonym 2; Synonym 3

==Overview==

The term 'Familial Hematuria and Hereditary Nephritis' refers to a mixed group of inherited kidney disorders which often present in early childhood and adolescence in form of microscopic hematuria [link to hematuria chapter]. The prognosis and associate co-morbidities are highly variable and dependent on the underlying genetic disease. It can be benign as seen in 'Thin Basement-membrane Disease' or lead to early end stage kidney disease as seen in the X-linked form of Alport syndrome [reference].

Unfortunately the term 'Familial Hematuria and Hereditary Nephritis' has been used variably and can be confusing. While the use of 'nephritis' in the term may imply inflammation, the disorders grouped here are NOT inflammatory in nature, nor due they reflect the involvement of a common pathway, inheritance pattern, or pathophysiology.

==Historical Perspective==
*A familial pattern of early childhood hematuria was first reported by Leonard G. Guthrie in 1902 <ref name="pmiddoi:10.1001">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=doi:10.1001 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. In the subsequent years, reports of early childhood hematuria, kidney failure (some associated to deafness or occular abnormalities), and early death in male offsprings emerged <ref name="pmid4613619">{{cite journal| author=Henderson JR| title=Ludwig Wittgenstein and Guy's Hospital. | journal=Guys Hosp Rep | year= 1973 | volume= 122 | issue= 1-2 | pages= 185-93 | pmid=4613619 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4613619 }} </ref>. Initial reports of this familial pattern of hematuria, and kidney failure thought these presentations to be due to pyelonephritis. Later reports, based on kidney biopsies and autopsies, were proposing a glomerulonephritic process to be the primary cause <ref name="pmidDOI: 10.1056">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=DOI: 10.1056 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. Other reports idnetified familial clustering of kidney disease in association with other abnormalities <ref name="pmid15416035">{{cite journal| author=HAWKINS CF, SMITH OE| title=Renal dysplasia in a family with multiple hereditary abnormalities including iliac horns. | journal=Lancet | year= 1950 | volume= 1 | issue= 6609 | pages= 803-8 | pmid=15416035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15416035 }} </ref>. The ever changing hypothesis of the underlying cause is perhaps a reflection of the various underlying disorders which were responsible in those families describe.

==Classification==
The following disorders are commonly grouped under the term 'Familial Hematuria and Hereditary Nephritis':
*Hereditary Disorders of Collagen Type IV:
**Thin Basement Membrane Disease (Benign Familial Hematuria)
**Alport Syndrome
**Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) (OMIM # 611773)
*Hereditary Disorders of Laminin:
**Pierson Syndrome
*Hereditary Disorder of basement membrane or cytoskletal gene regulation:
**Nail-Patella Syndrome (also known as Onychoosteodysplasia, or Turner-Kieser syndrome, or Fong disease)
*Hereditary Disorders of cytoskeletal protein:
**Epstein Syndrome
**Fechtner Syndrome
It is arbitrary and due to historical reasons, why certain disease are commonly referenced in this category, while other forms of inherited kidney disorders of the glomerular filtration barrier are categorized elsewhere. At best, perhaps an early diagnosis of hematuria can be considered a common denominator, setting these disorders apart from most other forms of inherited kidney disorders.

==Causes==
*Alport syndrome can be due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070)
**Compund heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Homozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
**X-linked mutations in the COL4A5 gene on the X-chromosome (X-linked dominant) (OMIM # 301050)
*Thin Basement Membrane Disease, also known as Thin Basement Membrane Nephropathy or Bening Familial Hematuria is due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070) or
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
**Heterozygous mutations in the COL4A1 gene on the chromosome 13q34 (autosomal dominant) (OMIM # 611773)
* Pierson Syndrome:
** Homozygous or compound heterozygous mutation in the LAMB2 gene on chromosome 3p21 (autosomal recessive) (OMIM # 609049)
*Nail-Patella Syndrome:
**Heterozygous mutation in the LIM-homeodomain protein LMX1B on the chromosome 9q33 (autosomal dominant) (OMIM #161200)
*Epstein Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153650)
*Fechtner Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153640)
==Differentiating Familial Hematuria & Hereditary Nephritis from other Diseases==
*Familial Hematuria & Hereditary Nephritis refers to a mixed group of disorders which can significantly overlap with other categories of inherited glomerular kidney disorders (e.g. genetic forms of FSGS). While this differentiation is arbitrary and due to historical reasons, a clear distinction must be made between the familial forms of hematuria referenced here, and other causes of hematuria and kidney disease observered in infants, children, and adolescents:
:*Genetic forms of FSGS, e.g. autosomal recessive nephrotic syndrome of the Finnish type (NPHS1 mutation), podocin related autosomal recessive proteinuria/ESRD (NPHS2 mutation), autosomal dominant FSGS (α-actinine 4, ACTN4 mutation), and others. In genetic forms of FSGS, proteinuria is the hallmark, and hematuria mostly absent. Decline in renal function and endstage kidney disease is highly variable and based on the underlying genetic defect.
:*Inflammatory glomerular disorders, e.g. lupus nephritis, infection related glomerulonephritis and others. These are often accompanied by other clinical and laboratory findings (e.g. ESR, CRP, complement levels, serologies).
:*Interstitial nephritis: Drugs, infectious agents, as well as autoimmune causes of interstitial nephritis
:*Pyelonephritis: Urine culture, and imaging studies to rule out structural abnormalities of the uro-genital tract which can predispose to reflux, obstruction, and recurrent infection should be performed and rules out.

==Epidemiology and Demographics==
* The prevalence of the individual diseases grouped together under Familial Hematuria & Hereditary Nephritis varies and is:
** Alport Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Thin Basement Membrane Disease: approximately [number or range] per 100,000 individuals worldwide.
** Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps: approximately [number or range] per 100,000 individuals worldwide.
** Pierson Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Epstein Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Fechtner Syndrome: approximately [number or range] per 100,000 individuals worldwide

== Natural History, Complications and Prognosis==
*Alport Syndrome:
**The x-linked form of Alport Syndrome is more severe in males, leading to early end stage kidney disease between the ages 15-35 in the majority of affected individuals [PMID: 10752524]. By age 40 almost all male patients have developed end stage kidney disease. In contrary, only about less than one third of females will go to develop end stage kidney disease [PMID: 21071975]
**In females the x-linked form of Alport syndrome presents later in life, and is less severe [PMID: 14514738]. Nevertheless, end stage kidney disease can reach a prevalence of to 30% in this patient population.
**The autosomal recessive form of Alport syndrome is more severe and presents earlier in life than the autosomal dominat form [PMID: 21071975].
*Thin Basement Membrane Disease:
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
*Nail-Patella Syndrome:
*Epstein Syndrome:
**This syndrome has been describe as a familial form of nephritis, associated with macrothrombocytopenia, renal failure, and hearing loss [PMID: 5011389]. Presentation resembles that of Alport Syndrome, and genetic testing may be required to distinguish these two disorders.
*Fechtner Syndrome:

== Diagnosis ==
===Diagnostic Criteria===
*'Familial Hematuria' or 'Hereditary Nephritis' are clinical presentations rather than a diagnosis. A familial pattern of hematuria, proteinuria and kidney disease may raise the suspicion of a genetic disorder related to the above mentioned structural components of the glomerular filtration barrier (collagen type IV, laminin, cytoskeletal components). Characteristic findings on a kidney biopsy (e.g. thinning and irregularities of the glomerular basement membrane), with or without ocular defects and hearing impairment may further strengthen the suspicion of a genetic defect in collagen IV, laminin, or myosin heavy chain biosynthesis, or cyteskeletal regulating proteins. Kidney biopsy can strenghte the clinical suspicion, however ultimate diagnosis is made through genetic testing.
=== Physical Findings ===
*The X-linked Alport syndrome in males as well as the autosomal recessive form of Alport syndrome are associated with ocular defects (corneal and retinal defects, and anterior lenticonus), and hearing loss during childhood.
=== Laboratory Findings ===
*
===Imaging Findings===
*

=== Other Diagnostic Studies ===
*

== Treatment ==
=== Medical Therapy ===
*

=== Surgery ===
*

=== Prevention ===
*
==References==
{{Reflist|2}}

[[Category:Pick One of 28 Approved]]

{{WS}}
{{WH}}

Familial Hematuria & Hereditary Nephritis

2017-03-09T21:07:04Z

Olufunmilola: /* Historical Perspective */

__NOTOC__
{{SI}}
{{CMG}} {{APM}}

{{SK}} Synonym 1; Synonym 2; Synonym 3

==Overview==

The term 'Familial Hematuria and Hereditary Nephritis' refers to a mixed group of inherited kidney disorders which often present in early childhood and adolescence in form of microscopic hematuria [link to hematuria chapter]. The prognosis and associate co-morbidities are highly variable and dependent on the underlying genetic disease. It can be benign as seen in 'Thin Basement-membrane Disease' or lead to early end stage kidney disease as seen in the X-linked form of Alport syndrome [reference].

Unfortunately the term 'Familial Hematuria and Hereditary Nephritis' has been used variably and can be confusing. While the use of 'nephritis' in the term may imply inflammation, the disorders grouped here are NOT inflammatory in nature, nor due they reflect the involvement of a common pathway, inheritance pattern, or pathophysiology.

==Historical Perspective==
*A familial pattern of early childhood hematuria was first reported by Leonard G. Guthrie in 1902 <ref name="pmiddoi:10.1001">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=doi:10.1001 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. In the subsequent years, reports of early childhood hematuria, kidney failure (some associated to deafness or occular abnormalities), and early death in male offsprings emerged <ref name="pmid4613619">{{cite journal| author=Henderson JR| title=Ludwig Wittgenstein and Guy's Hospital. | journal=Guys Hosp Rep | year= 1973 | volume= 122 | issue= 1-2 | pages= 185-93 | pmid=4613619 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4613619 }} </ref>. Initial reports of this familial pattern of hematuria, and kidney failure thought these presentations to be due to pyelonephritis. Later reports, based on kidney biopsies and autopsies, were proposing a glomerulonephritic process to be the primary cause <ref name="pmidDOI: 10.1056">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=DOI: 10.1056 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref>. Other reports idnetified familial clustering of kidney disease in association with other abnormalities <ref name="pmid15416035">{{cite journal| author=HAWKINS CF, SMITH OE| title=Renal dysplasia in a family with multiple hereditary abnormalities including iliac horns. | journal=Lancet | year= 1950 | volume= 1 | issue= 6609 | pages= 803-8 | pmid=15416035 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15416035 }} </ref>. The ever changing hypothesis of the underlying cause is perhaps a reflection of the various underlying disorders which were responsible in those families describe.

==Classification==
The following disorders are commonly grouped under the term 'Familial Hematuria and Hereditary Nephritis':
*Hereditary Disorders of Collagen Type IV:
**Thin Basement Membrane Disease (Benign Familial Hematuria)
**Alport Syndrome
**Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC) (OMIM # 611773)
*Hereditary Disorders of Laminin:
**Pierson Syndrome
*Hereditary Disorder of basement membrane or cytoskletal gene regulation:
**Nail-Patella Syndrome (also known as Onychoosteodysplasia, or Turner-Kieser syndrome, or Fong disease)
*Hereditary Disorders of cytoskeletal protein:
**Epstein Syndrome
**Fechtner Syndrome
It is arbitrary and due to historical reasons, why certain disease are commonly referenced in this category, while other forms of inherited kidney disorders of the glomerular filtration barrier are categorized elsewhere. At best, perhaps an early diagnosis of hematuria can be considered a common denominator, setting these disorders apart from most other forms of inherited kidney disorders.
==Causes==
*Alport syndrome can be due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070)
**Compund heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Homozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal recessive) (OMIM # 120070)
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
**X-linked mutations in the COL4A5 gene on the X-chromosome (X-linked dominant) (OMIM # 301050)
*Thin Basement Membrane Disease, also known as Thin Basement Membrane Nephropathy or Bening Familial Hematuria is due to:
**Heterozygous mutations in the COL4A3 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120070) or
**Heterozygous mutations in the COL4A4 gene on the chromosome 2q36 (autosomal dominant) (OMIM # 120131)
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
**Heterozygous mutations in the COL4A1 gene on the chromosome 13q34 (autosomal dominant) (OMIM # 611773)
* Pierson Syndrome:
** Homozygous or compound heterozygous mutation in the LAMB2 gene on chromosome 3p21 (autosomal recessive) (OMIM # 609049)
*Nail-Patella Syndrome:
**Heterozygous mutation in the LIM-homeodomain protein LMX1B on the chromosome 9q33 (autosomal dominant) (OMIM #161200)
*Epstein Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153650)
*Fechtner Syndrome:
**Heterozygous mutation in the MYH9 gene on chromosome 22q12 (autosomal dominant) (OMIM # 153640)
==Differentiating Familial Hematuria & Hereditary Nephritis from other Diseases==
*Familial Hematuria & Hereditary Nephritis refers to a mixed group of disorders which can significantly overlap with other categories of inherited glomerular kidney disorders (e.g. genetic forms of FSGS). While this differentiation is arbitrary and due to historical reasons, a clear distinction must be made between the familial forms of hematuria referenced here, and other causes of hematuria and kidney disease observered in infants, children, and adolescents:
:*Genetic forms of FSGS, e.g. autosomal recessive nephrotic syndrome of the Finnish type (NPHS1 mutation), podocin related autosomal recessive proteinuria/ESRD (NPHS2 mutation), autosomal dominant FSGS (α-actinine 4, ACTN4 mutation), and others. In genetic forms of FSGS, proteinuria is the hallmark, and hematuria mostly absent. Decline in renal function and endstage kidney disease is highly variable and based on the underlying genetic defect.
:*Inflammatory glomerular disorders, e.g. lupus nephritis, infection related glomerulonephritis and others. These are often accompanied by other clinical and laboratory findings (e.g. ESR, CRP, complement levels, serologies).
:*Interstitial nephritis: Drugs, infectious agents, as well as autoimmune causes of interstitial nephritis
:*Pyelonephritis: Urine culture, and imaging studies to rule out structural abnormalities of the uro-genital tract which can predispose to reflux, obstruction, and recurrent infection should be performed and rules out.

==Epidemiology and Demographics==
* The prevalence of the individual diseases grouped together under Familial Hematuria & Hereditary Nephritis varies and is:
** Alport Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Thin Basement Membrane Disease: approximately [number or range] per 100,000 individuals worldwide.
** Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps: approximately [number or range] per 100,000 individuals worldwide.
** Pierson Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Epstein Syndrome: approximately [number or range] per 100,000 individuals worldwide.
** Fechtner Syndrome: approximately [number or range] per 100,000 individuals worldwide

== Natural History, Complications and Prognosis==
*Alport Syndrome:
**The x-linked form of Alport Syndrome is more severe in males, leading to early end stage kidney disease between the ages 15-35 in the majority of affected individuals [PMID: 10752524]. By age 40 almost all male patients have developed end stage kidney disease. In contrary, only about less than one third of females will go to develop end stage kidney disease [PMID: 21071975]
**In females the x-linked form of Alport syndrome presents later in life, and is less severe [PMID: 14514738]. Nevertheless, end stage kidney disease can reach a prevalence of to 30% in this patient population.
**The autosomal recessive form of Alport syndrome is more severe and presents earlier in life than the autosomal dominat form [PMID: 21071975].
*Thin Basement Membrane Disease:
*Hereditary Angiopathy with Nephropathy, Aneurysms, and Muscle Cramps (HANAC):
*Nail-Patella Syndrome:
*Epstein Syndrome:
**This syndrome has been describe as a familial form of nephritis, associated with macrothrombocytopenia, renal failure, and hearing loss [PMID: 5011389]. Presentation resembles that of Alport Syndrome, and genetic testing may be required to distinguish these two disorders.
*Fechtner Syndrome:

== Diagnosis ==
===Diagnostic Criteria===
*'Familial Hematuria' or 'Hereditary Nephritis' are clinical presentations rather than a diagnosis. A familial pattern of hematuria, proteinuria and kidney disease may raise the suspicion of a genetic disorder related to the above mentioned structural components of the glomerular filtration barrier (collagen type IV, laminin, cytoskeletal components). Characteristic findings on a kidney biopsy (e.g. thinning and irregularities of the glomerular basement membrane), with or without ocular defects and hearing impairment may further strengthen the suspicion of a genetic defect in collagen IV, laminin, or myosin heavy chain biosynthesis, or cyteskeletal regulating proteins. Kidney biopsy can strenghte the clinical suspicion, however ultimate diagnosis is made through genetic testing.
=== Physical Findings ===
*The X-linked Alport syndrome in males as well as the autosomal recessive form of Alport syndrome are associated with ocular defects (corneal and retinal defects, and anterior lenticonus), and hearing loss during childhood.
=== Laboratory Findings ===
*
===Imaging Findings===
*

=== Other Diagnostic Studies ===
*

== Treatment ==
=== Medical Therapy ===
*

=== Surgery ===
*

=== Prevention ===
*
==References==
{{Reflist|2}}

[[Category:Pick One of 28 Approved]]

{{WS}}
{{WH}}

Membranoproliferative glomerulonephritis pathophysiology

2017-01-30T20:12:01Z

Olufunmilola: /* Membranoproliferative glomerulonephritis type I */

__NOTOC__
{{Membranoproliferative glomerulonephritis}}
{{CMG}}{{APM}} {{AE}} {{OO}}
==Overview==
==Pathophysiology==
==Histologic Findings==

===Light microscopy===

Glomeruli generally are enlarged and hypercellular, with an increase in mesangial cellularity and matrix. Mesangial increase, when generalized throughout the glomeruli, causes an exaggeration of their lobular form, giving rise to the alternative name of lobular nephritis. Infiltrating neutrophils and monocytes contribute to glomerular hypercellularity.

The capillary basement membranes are thickened by interposition of mesangial cells and matrix into the capillary wall. This gives rise to the tram-track or double-contoured appearance of the capillary wall, best appreciated with the methenamine silver stain or the periodic acid-Schiff reagent.

Crescents may be visible in 10% of patient biopsy specimens. Interstitial changes, including inflammation, interstitial fibrosis, and tubular atrophy, are observed in patients with progressive decline in GFR.

https://commons.wikimedia.org/wiki/File:Membranoproliferative_glomerulonephritis_-_very_high_mag.jpg

===Membranoproliferative glomerulonephritis type I===

The most common type of MPGN is type1. It is described as Immune complex mediated MPGN, with circulating immune complexes present in approximately 33% of patients with MPGN type I. In all patients with type I, immune complexes are found in the mesangium and subendothelial spaces. The most common causes of immune complex MPGN include chronic infections, autoimmune diseases, and monoclonal gammopathies. Monoclonal gammopathies have been recently recognized as an important cause of MPGN, especially in older adults, with or without cryoglobulins <ref name="pmid20185597">{{cite journal| author=Sethi S, Zand L, Leung N, Smith RJ, Jevremonic D, Herrmann SS et al.| title=Membranoproliferative glomerulonephritis secondary to monoclonal gammopathy. | journal=Clin J Am Soc Nephrol | year= 2010 | volume= 5 | issue= 5 | pages= 770-82 | pmid=20185597 | doi=10.2215/CJN.06760909 | pmc=2863981 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20185597 }} </ref>. About 41% of patients without chronic infections or autoimmune diseases had serum and/or urine electrophoresis studies positive for a monoclonal gammopathy <ref name="pmid20185597">{{cite journal| author=Sethi S, Zand L, Leung N, Smith RJ, Jevremonic D, Herrmann SS et al.| title=Membranoproliferative glomerulonephritis secondary to monoclonal gammopathy. | journal=Clin J Am Soc Nephrol | year= 2010 | volume= 5 | issue= 5 | pages= 770-82 | pmid=20185597 | doi=10.2215/CJN.06760909 | pmc=2863981 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20185597 }} </ref>. Most of them had a monoclonal gammopathy of undetermined significance

On electron microscopy, electron dense deposits in subendothelial sites are characteristic of this disease. Mesangial and occasional subepithelial deposits also may be present. Irregular new basement membrane material is formed around the subendothelial deposits and mesangial projections, producing the tram-track appearance on light microscopy.

By immunofluorescence, prominent C3 deposition in a granular pattern is noted in the capillary walls, with variable mesangial C3 deposits. Early components of complement, immunoglobulin G (IgG), and, less commonly, immunoglobulin M (IgM) may be found in a distribution similar to C3.

===Membranoproliferative glomerulonephritis type II or dense deposit disease===

The basement membranes of the glomerulus, Bowman capsule, tubules, and peritubular capillaries are thickened. The basement membrane appears irregular and ribbonlike on special stains (eg, periodic acid-Schiff, thioflavine-T, toluidine blue).

On electron microscopy, the basement membrane is thickened by discontinuous, amorphous electron dense deposits that reside in the lamina densa layer, hence the alternative name of dense deposit disease. Mesangial and subepithelial dense deposits may be noted.

Immunofluorescence reveals complement component C3 deposited in an irregular granular pattern in the basement membranes on either side but not within the dense deposits or in nodular ring forms in the mesangium. Little or no deposition of immunoglobulins occurs in the glomeruli.

===Membranoproliferative glomerulonephritis type III===

This variant of MPGN, also called the Burkholder variant, displays combined features of MPGN type I and membranous nephropathy.

Subepithelial, subendothelial, and mesangial deposits are present on electron microscopy. Successive generations of subendothelial and subepithelial deposits disrupt the basement membrane, and concurrent formation of new lamina densa material is present, giving the basement membrane a complex laminated appearance.

Immunohistology shows granular deposition of C3, C5, properdin, IgG, and IgM, predominantly in the capillary walls.

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Disease]]
[[Category:Nephrology]]

Membranoproliferative glomerulonephritis causes

2017-01-30T19:08:14Z

Olufunmilola:

__NOTOC__
{{Membranoproliferative glomerulonephritis}}
{{CMG}}{{APM}} {{AE}}{{OO}}
==Overview==
==Causes==

Conditions associated with a membranoproliferative pattern of injury are listed as follows:

* Immune complex–mediated disease
:*Idiopathic forms of MPGN or of unknown association
:*:* MPGN type I
:*:* MPGN type II or dense deposit disease and PLD
:*:* MPGN type III
:* Autoimmune diseases
:*:* Systemic lupus erythematosus (SLE)
:*:* Sjögren syndrome
:*:* Rheumatoid arthritis
:*:* Inherited complement deficiencies, in particular, C2 deficiency
:*:* Scleroderma
:*:* Celiac disease
:* Chronic infections
:*:* Viral - Hepatitis B, hepatitis C, and cryoglobulinemia type II
:*:* Bacterial - Endocarditis, infected ventriculoatrial (or jugular) shunt, multiple visceral abscesses, leprosy
:*:* Protozoal - Malaria, schistosomiasis
:*:* Other infections - Mycoplasma
:* Miscellaneous - Chronic liver disease (cirrhosis and alpha1-antitrypsin deficiency)
* Chronic and recovered thrombotic microangiopathies
:* Healing phase of hemolytic uremic syndrome and/or thrombotic thrombocytopenic purpura
:* Syndromes of circulating antiphospholipid (anticardiolipin) antibodies
:* Radiation nephritis
:* Nephropathy associated with bone marrow transplantation
:* Sickle cell anemia and polycythemia
:* Transplant glomerulopathy
* Paraprotein deposition diseases
:* Glomerulonephropathies associated with cryoglobulinemia type I
:* Waldenström macroglobulinemia
:* Immunotactoid glomerulopathy
:* Immunoglobulin light chain or heavy chain deposition diseases
:* Fibrillary glomerulonephritis
* Malignant neoplasms
:* Lymphoma
:* Leukemia
:* Carcinoma

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Disease]]
[[Category:Nephrology]]

Membranoproliferative glomerulonephritis classification

2017-01-30T19:05:12Z

Olufunmilola: /* Overview */

__NOTOC__
{{Membranoproliferative glomerulonephritis}}
{{CMG}} {{AE}} {{APM}} {{OO}}
==Overview==

Like many forms of glomerulopathies, membranoproliferative glomerulonephritis (glomerulopathy) has been a diagnosis of tissue pathology rather the diagnosis of a specific disease entity. Therefore the term membranoploriferative glomerulonephritis (MPGN) relates to a pattern of glomerular injury which can be caused by many disease states. Historically the nephropathologists divided MPGN into 3 distinctive categories to shed light into what may be causing this type of kidney injury:
MPGN type 1: mesangial and subendothelial electron dense deposits
MPGN type 2: electron dense material in the glomerular basement membrane
MPGN type 3: subepithelial deposits with basement membrane spikes

This categorization, however is now obsolete. The recognition of several new disorders as the underlying cause of MPGN, and the lack of clinical, prognostic, or therapeutic relevance made this categorization less useful. For completeness and to help better accommodate the transition from old to new classification, below both classifications are reviewed.

Classification of MPGN based on immunofluorescence microscopy is a result of all advances in the understanding of the pathogenesis of the disease. Based on this advanced techniques, there are three types of MPGN <ref name="pmid20185597">{{cite journal| author=Sethi S, Zand L, Leung N, Smith RJ, Jevremonic D, Herrmann SS et al.| title=Membranoproliferative glomerulonephritis secondary to monoclonal gammopathy. | journal=Clin J Am Soc Nephrol | year= 2010 | volume= 5 | issue= 5 | pages= 770-82 | pmid=20185597 | doi=10.2215/CJN.06760909 | pmc=2863981 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20185597 }} </ref>:

*Immune-complex-mediated MPGN (Type I)
*Complement-mediated MPGN (Type II)
*Non-Ig/complement-mediated MPGN (Type III)

==Classification==
Classification of MPGN based on immunofluorescence microscopy is a result of all advances in the understanding of the pathogenesis of the disease. Based on this advanced techniques, there are three types of MPGN <ref name="pmid21839367">{{cite journal| author=Sethi S, Fervenza FC| title=Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. | journal=Semin Nephrol | year= 2011 | volume= 31 | issue= 4 | pages= 341-8 | pmid=21839367 | doi=10.1016/j.semnephrol.2011.06.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21839367 }} </ref>;
*Immune-complex-mediated MPGN (Type I)
*Complement-mediated MPGN (Type II)
*Non-Ig/complement-mediated MPGN (Type III)

===Type I===

The most common type.

Circulating immune complexes are present in approximately 33% of patients with MPGN type I. In all patients with type I, immune complexes are found in the mesangium and subendothelial spaces, and they trigger complement activation and the release of cytokines and chemokines. The release of inflammatory mediators causes an influx of inflammatory cells and leads to mesangial and endothelial cell proliferation. Most patients with circulating immune complexes do not develop MPGN; thus, additional pathogenic factors (eg, nature of the antigen, size of complexes, type and charge on antibodies, local glomerular factors) must play a role. In addition to circulating immune complexes becoming entrapped in the glomerular basement membrane (GBM), experimental evidence indicates that complexes may be formed in situ when antigens adhere to the GBM and antibodies subsequently bind to these antigens. Formation of such immune complexes triggers the same cascade as described above.

Activation of complement and the resulting hypocomplementemia may cause defective clearance of circulating immune complexes. The nephritic factor of the classic pathway (ie, NFc or C4NeF) is found in approximately 15% of patients. This nephritic factor stabilizes the classic pathway C3 convertase C4b,2a and potentiates C3 activation and consumption. The role of this nephritic factor in the pathogenesis of MPGN type I is unclear. Approximately 20% of patients have the nephritic factor of the terminal pathway.

===Type II===

Type II (dense deposit disease) is very similar, except the material deposited is not immune complexes and is not yet known.
MPGN type II (or dense deposit disease) is a separate entity that has been conventionally classified with MPGN because of the similarities of light microscopic appearance. The pathogenesis of MPGN type II is not known. This disease is systemic, as evidenced by dense deposits in the kidney, splenic sinusoids, and Bruch membrane of the retina. This disease also has a high incidence of recurrence in renal allografts. The chemical composition and origin of the dense deposits are not known. No circulating immune complexes are observed in MPGN type II.

Dense deposit disease is associated with multiple complement abnormalities, including a persistent reduction of C3 levels. One hypothesis is that the dense deposits cause complement activation. This hypothesis is supported by the tram-track distribution of C3 deposits along the basement membrane.

NFa is present in 80% of patients with dense deposit disease. NFa stabilizes the alternative pathway convertase and results in complement activation and chronic C3 consumption. Deficiencies of factor H or resistance to factor H, described in MPGN, may lead to an accumulation of the alternative pathway convertase and chronic C3 consumption.

Partial lipoid dystrophy (PLD) is associated commonly with MPGN type II and the presence of NFa. Adipocytes produce adipsin, which is identical to complement factor D and is responsible for activating the preconvertase C3b,Bb. NFa causes a lysis of adipocytes that produce adipsin, and the distribution of fat atrophy in PLD follows variations in the amount of adipsin produced by adipocytes. By analogy, NFa may cause damage to glomerular cells that produce complement.

===Type III===

Type III is very rare, it is characterized by a mixture of subepithelial deposits and the typical pathological findings of Type I disease.

The glomerular deposits contain C3, C5, and properdin, indicating activation of the alternative complement pathway. Signs of activation of the classic pathway are minimal, and circulating immune complexes do not appear to play a role in the genesis of this variant.

Changes in the capillary wall are hypothesized to be the primary event leading to activation of the complement pathway. This hypothesis is supported by the deposition of C3Bb2,Bb convertase components in the basement membrane. The deposits of convertase and membrane attack complex may lyse the basement membrane and stimulate new membrane formation. NFt is present in 60-80% of patients with MPGN type III. NFt stabilizes the alternative pathway properdin-dependent C3/C5 convertase (C3Bb2,Bb,P) and also activates the terminal complement components, forming C5b-C9 (ie, the membrane attack complex).

A familial form of MPGN type III with an autosomal dominant pattern of inheritance has been identified with genetic linkage to band 1q31-32. Genes in this area of chromosome 1 code for proteins that regulate the C3 convertase activity.

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Disease]]
[[Category:Nephrology]]

Membranoproliferative glomerulonephritis overview

2017-01-30T18:59:27Z

Olufunmilola: /* Overview */

__NOTOC__
{{Membranoproliferative glomerulonephritis}}
{{CMG}}{{APM}} {{AE}} {{OO}}
==Overview==
'''Membranoproliferative glomerulonephritis''' or MPGN is a type of [[glomerulonephritis]] caused by immune complexes depositing in the kidney glomerular mesangium and basement membrane ([[GBM]]), activating [[complement system|complement]] and damaging the glomeruli. The GBM is rebuilt ontop of the deposits, causing a "tram-tracking" appearance under the microscope.
Membranoproliferative Glomerulonephritis (MPGN) is a relatively uncommon inflammatory glomerulopathy that can cause chronic nephritis. Based on the histological pattern of glomerular injury it has been described as a chronic kidney disease found mostly in children and young adults. Like many forms of glomerulopathies, membranoproliferative glomerulonephritis (glomerulopathy) has been a diagnosis of tissue pathology rather the diagnosis of a specific disease entity. Therefore, the term membranoploriferative glomerulonephritis (MPGN) relates to a pattern of glomerular injury characterized by mesangial proliferation and expansion, lobularization of the glomerular tufts and double contours which can be caused by many disease states<ref name="pmidPMID:27356907">{{cite journal| author=Lionaki S, Gakiopoulou H, Boletis JN| title=Understanding the complement-mediated glomerular diseases: focus on membranoproliferative glomerulonephritis and C3 glomerulopathies. | journal=APMIS | year= 2016 | volume= 124 | issue= 9 | pages= 725-35 | pmid=PMID:27356907 | doi=10.1111/apm.12566 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27356907 }} </ref>.

Glomerular injury occurs due to deposition of immune complexes on the glomerular mesangium or on the glomerular basement membrane. MPGN has been categorized into 3 types based on the histological pattern of glomerular damage. Clinically, MPGN often present with hematuria, varying degrees of proteinuria, with or without Glomerular filtration rate impairment depending on the severity of glomerular injury, and the underlying etiology.

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Disease]]
[[Category:Nephrology]]

IgA nephropathy medical therapy

2017-01-23T19:04:51Z

Olufunmilola:

__NOTOC__
{{IgA nephropathy }}
{{CMG}}{{APM}} {{AE}}{{OO}]
==Overview==
==[[IgA nephropathy|Medical Therapy]]==
According to “The National Kidney Foundation: Kidney Disease - Improving Global Outcomes” (NKF-KDIGO)<ref name="pmid15882273">{{cite journal| author=Haubitz M, Wittke S, Weissinger EM, Walden M, Rupprecht HD, Floege J et al.| title=Urine protein patterns can serve as diagnostic tools in patients with IgA nephropathy. | journal=Kidney Int | year= 2005 | volume= 67 | issue= 6 | pages= 2313-20 | pmid=15882273 | doi=10.1111/j.1523-1755.2005.00335.x | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15882273 }} </ref> in 2012, the management and treatment recommendations of primary IgA nephropathy are as follows:

===Goals of Management===

*Prevent the occurrence of repeated episodes of AKI and persistent macroscopic hematuria<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
*Reduce proteinuria to less than 1 g/24 hrs in adults and to less than 0.5 g/24 hrs in children regardless of baseline proteinuria at diagnosis<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref><ref name="pmid17978307">{{cite journal| author=Reich HN, Troyanov S, Scholey JW, Cattran DC, Toronto Glomerulonephritis Registry| title=Remission of proteinuria improves prognosis in IgA nephropathy. | journal=J Am Soc Nephrol | year= 2007 | volume= 18 | issue= 12 | pages= 3177-83 | pmid=17978307 | doi=10.1681/ASN.2007050526 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17978307 }} </ref>
*Control blood pressure to < 130/80 mmHg if proteinuria is > 0.3 g/24 hrs and more aggressively to <125/75 mmHg if proteinuria is 1g/24 hrs<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
*Reduce avoidable risk factors, such as obesity. Weight loss to a BMI<25 kg/m2 is recommended to patients with IgA nephropathy to reduce obesity and cardiovascular co-morbidities and degree of proteinuria<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>

===Treatment===

====Supportive Measures====

Supportive measures are to performed in IgA nephropathy similar to the measures required for management of ATN<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>

*Indication: Remarkable presence of only ATN and intratubular RBC casts

====Pharmacologic Therapy====
*'''Angiotensin-converting enzyme inhibitor (ACE-I) or angiotensin II-receptor blocker (ARB)'''<ref name="pmid22895519">{{cite journal|author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int| year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280| pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
** To titrate dosages as required and as tolerated<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
**Duration: Chronic
**Indications:
***Proteinuria > 1g/24 hrs in adults or 0.5 g/24 hrs in children
***Blood pressure > 130/80 mmHg if proteinuria < 1g/24 hrs
***Blood pressure > 125/75 mm Hg if proteinuria > 1g/24 hrs

ACE-I or ARB are helpful to reduce proteinuria. Nonetheless, randomized clinical trials (RCT) have not yet been conducted to assess their role in reducing the progression to ESRD. Although combination therapy has been proven more effective in monotherapy in some studies<ref name="pmid10213639">{{cite journal| author=Russo D, Pisani A, Balletta MM, De Nicola L, Savino FA, Andreucci M et al.| title=Additive antiproteinuric effect of converting enzyme inhibitor and losartan in normotensive patients with IgA nephropathy. | journal=Am J Kidney Dis | year= 1999 | volume= 33 | issue= 5 | pages= 851-6 | pmid=10213639 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10213639 }} </ref><ref name="pmid16047643">{{cite journal| author=Yang Y, Ohta K, Shimizu M, Nakai A, Kasahara Y, Yachie A et al.| title=Treatment with low-dose angiotensin-converting enzyme inhibitor (ACEI) plus angiotensin II receptor blocker (ARB) in pediatric patients with IgA nephropathy. | journal=Clin Nephrol | year= 2005 | volume= 64 | issue= 1 | pages= 35-40 | pmid=16047643 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16047643 }} </ref>, KDIGO guidelines have not made recommendations about combination therapy yet pending RCTs.

*'''Corticosteroids'''<ref name="pmid22895519">{{cite journal|author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int| year= 2012 | volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280| pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
**Duration: 6 months
**Indication: GFR > 50 ml/min/1.73m2 and persistent proteinuria > 1g/24 hrs despite ACE-I or ARB and BP control

The use of steroids in patient with a GFR<50 ml/min/1.73m2 has not been studied yet. Specific dosage is not yet recommended according to KDIGO guidelines. There are more observed side-effects with high-dose pulse corticosteroids.

*'''Immunosuppressive agents''' (''cyclophosphamide, azathioprine'')<ref name="pmid22895519">{{cite journal|author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int| year= 2012 |volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280| pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
**Duration: Cyclophosphamide: 3 months and azathrioprine: minimum 2 years.
**Indication: Patients on steroid therapy with rapidly progressive IgA nephropathy and >50% crescent formation on biopsy

Immunosuppressive agents should not be used in patients with low GFR < 30 ml/min/1.73m2 except if indicated as above. The dose of steroid must be reduced when using concomitant immunosuppressive therapy. One RCT showed better kidney function by reducing corticosteroiddose from 40mg/d to 10mg/d with use of cyclophosphamide at 1.5mg/kg/d for 3 months followed by azathioprine at 1.5mg/kg/d for at least 2 years.

*'''Fish oil'''<ref name="pmid22895519">{{cite journal|author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int| year= 2012 |volume= 82 | issue= 8 | pages= 840-56 |pmid=22895519 | doi=10.1038/ki.2012.280| pmc=|url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>
**Indication: Persistent proteinuria > 1g/24 hrs despite 3-6 months of optimal ACE-I or ARB and blood pressure control.

Fish oil at a dose of 12g/d was shown to improve renal outcome in patients with IgA nephropathy in trials by reducing rate of ESRD in 4 years from 40% to only 4%.<ref name="pmid7935657">{{cite journal| author=Donadio JV, Bergstralh EJ, Offord KP, Spencer DC, Holley KE| title=A controlled trial of fish oil in IgA nephropathy. Mayo Nephrology Collaborative Group. | journal=N Engl J Med | year= 1994 | volume= 331 | issue= 18 | pages= 1194-9 | pmid=7935657 | doi=10.1056/NEJM199411033311804 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7935657 }} </ref> In another 6-month study, 3g/d dose also showed better prognosis with less proteinuria.<ref name="pmid10446945">{{cite journal| author=Donadio JV, Grande JP, Bergstralh EJ, Dart RA, Larson TS, Spencer DC| title=The long-term outcome of patients with IgA nephropathy treated with fish oil in a controlled trial. Mayo Nephrology Collaborative Group. | journal=J Am Soc Nephrol | year= 1999 | volume= 10 | issue= 8 | pages= 1772-7 | pmid=10446945 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10446945 }} </ref>

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Nephrology]]
[[Category:Genetic disorders]]

Focal segmental glomerulosclerosis causes

2017-01-23T14:58:03Z

Olufunmilola:

__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}}; '''Associate Editor-In-Chief:’’’{{OO}} {{CZ}}

==Overview==

==Causes==
According to D'Agati and colleagues,<ref name="pmid12704572">{{cite journal| author=D'Agati V| title=Pathologic classification of focal segmental glomerulosclerosis. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 117-34 | pmid=12704572 | doi=10.1053/snep.2003.50012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704572 }} </ref> FSGS may be primary of secondary. Primary FSGS is defined as idiopathic FSGS, whereas secondary FSGS is defined as FSGS other etiologies.<ref name="pmid12704572">{{cite journal| author=D'Agati V| title=Pathologic classification of focal segmental glomerulosclerosis. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 117-34 | pmid=12704572 | doi=10.1053/snep.2003.50012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704572 }} </ref>
The following list shows all the causes of FSGS<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>:
===Primary (Idiopathic) FSGS===
===Secondary FSGS===
====Familial====
*Mutations in [[alpha actinin 4]]
*Mutations in NPHS1 ([[nephrin]])
*Mutations in NPHS2 ([[podocin]])
*Mutations in WT-1
*Mutations in [[TRPC6]]
*Mutations in [[SCARB2]] (LIMP2)
*Mutations in INF2 (formin)
*Mutations in CD2-associated protein
*[[Mitochondrial cytopathies]]

====Virus Associated====
*[[HIV]]
*[[Parvovirus B19]]
*[[CMV]]

====Medication====
*[[Heroin]]
*[[Interferon alpha]]
*[[Lithium]]
*[[Pamidronate]]/aledronate
*[[Anabolic steroids]]

====Adaptive Structural-Functional Responses====
=====Reduced Kidney Size=====
*Oligomeganephronia
*Unilateral kidney agenesis
*Kidney dysplasia
*Cortical necrosis
*[[Reflux nephropathy]]
*Surgical kidney ablation
*Chronic allograft nephropathy
*Any advanced kidney disease with reduction in functioning [[nephrons]]

=====Initially Normal Kidney Mass=====
*[[Diabetes mellitus]]
*[[Hypertension]]
*[[Obesity]]
*[[Cyanotic congenital heart disease]]
*[[Sickle cell anemia]]

====Malignancy====
*[[Lymphoma]]

====Nonspecific Pattern of FSGS Caused by Kidney Scarring in Glomerular Disease====
*Focal proliferative [[glomerulonephritis]]
**[[IgA nephropathy]]
**[[Lupus nephritis]]
**Pauci-immune focal necrotizing and [[crescentic glomerulonephritis]]
*[[Alport's syndrome]] (hereditary nephritis)
*[[Membranous nephropathy]]
*[[Thrombotic microangiopathy]]

==References==
{{Reflist|2}}

{{WH}}
{{WS}}

Focal segmental glomerulosclerosis overview

2017-01-23T14:56:48Z

Olufunmilola:

__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}}; '''Associate Editor-In-Chief:’’’{{OO}} {{CZ}}

==Overview==

'''Focal segmental glomerulosclerosis''' (FSGS) is a cause of [[nephrotic syndrome]] in children and adolescents, as well as an important cause of [[kidney failure]] in adults.<ref name=Robbins_2005>{{cite book | author = Kumar V, Fausto N, Abbas A (editors) | title = Robbins & Cotran Pathologic Basis of Disease | edition = 7th | pages= pp. 982-3 | publisher = Saunders | year = 2003 | id = ISBN 978-0-721-60187-8 }}</ref> Focal Segmental Glomerulosclerosis (FSGS) is a distinct finding and a descriptive report on the glomerular morphology seen in certain glomerular disease conditions. It is considered to be a glomerular podocytopathy <ref name="pmidPMID: 24589721">{{cite journal| author=Sethi S, Glassock RJ, Fervenza FC| title=Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist. | journal=Nephrol Dial Transplant | year= 2015 | volume= 30 | issue= 3 | pages= 375-84 | pmid=PMID: 24589721 | doi=10.1093/ndt/gfu035 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24589721 }} </ref>. [[Minimal change disease]] (MCD) is by far the most common cause of nephrotic syndrome in children: MCD and primary FSGS may have a similar cause.<ref name=Robbins_2005 /> Focal Segmental Glomerulosclerosis (FSGS) is very similar in presentation to Minimal Change Kidney Disease (MCD), in-fact both are podocytopathies but they are histological different with varying disease signs and symptoms.

The individual components of the name refer to the appearance of the kidney tissue on [[biopsy]]: ''focal'' - only some of the [[glomerulus|glomeruli]] are involved (as opposed to diffuse), ''segmental'' - only part of an entire glomerulus is involved (as opposed to global), ''glomerulosclerosis'' - refers to scarring of the [[glomerulus]] (a part of the [[nephron]] (the functional unit of the [[kidney]])).

FSGS is a progressive form of renal disease, it has become the most common cause of GN-related ESRD in patients with End Stage Renal Disease (ESRD) in the United States. <ref name="pmid15492947">{{cite journal| author=Kitiyakara C, Eggers P, Kopp JB| title=Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. | journal=Am J Kidney Dis | year= 2004 | volume= 44 | issue= 5 | pages= 815-25 | pmid=15492947 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15492947 }} </ref> It accounts for about 40% of adults and about 20% of pediatric cases of Nephrotic Syndrome in the United States. <ref name="pmid15492947">{{cite journal| author=Kitiyakara C, Eggers P, Kopp JB| title=Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. | journal=Am J Kidney Dis | year= 2004 | volume= 44 | issue= 5 | pages= 815-25 | pmid=15492947 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15492947 }} </ref>

FSGS can be a primary or secondary cause of Nephrotic Syndrome. Primary FSGS can occur as an epithelial cell disorder characterized by podocytopathy of the glomeruli that may be related etiologically to minimal change disease. Secondary FSGS occurs due to previous glomerular injury or as a response to previous nephron loss from toxic effects of drugs, viral infections and chronic systemic diseases like diabetes mellitus, SLE and other renal affecting autoimmune diseases.

Definitive diagnosis of FSGS is by kidney biopsy. High clinical suspicion is also very important in the diagnosis and differentiation of FSGS from MCD. MCD often presents with features of Nephrotic Syndrome like proteinuria, edema and hyperlipidemia while FSGS may present with hematuria, hypertension and decreased renal function which are common presentations of nephritic syndrome.
==Historical Perspective==
==Classification==
==Pathophysiology==
==Causes==
==Differentials==
==Risk Factors==
==Screening==
==Epidemiology and Demographics==
==Natural History, Complications, and Prognosis==
==Diagnosis==
===History and Symptoms===
===Physical Examination===
===Laboratory findings===
===Imaging Findings===
===Other Diagnostic Studies===
==Treatment==
===Medical Therapy===
===Surgery===
===Prevention===
==References==
{{Reflist|2}}

{{WH}}
{{WS}}

Focal segmental glomerulosclerosis

2017-01-23T14:55:56Z

Olufunmilola:

__NOTOC__
{{DiseaseDisorder infobox |
Name = Focal segmental glomerulosclerosis |
ICD10 = N00-N08 (with .1 suffix) |
ICD9 = {{ICD9|581.1}} |
OMIM = 603278 |
OMIM_mult = {{OMIM2|603965}} {{OMIM2|607832}} |
MedlinePlus =000478 |
eMedicineSubj = |
eMedicineTopic = |
}}
{{Focal segmental glomerulosclerosis}}

'''For patient information on this page, click [[Focal segmental glomerulosclerosis (patient information)|here]]'''

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:’’’{{OO}}{{CZ}}

{{SK}} FSGS

== [[Focal segmental glomerulosclerosis overview|Overview]] ==

== [[Focal segmental glomerulosclerosis historical perspective|Historical Perspective]] ==

== [[Focal segmental glomerulosclerosis classification|Classification]] ==

== [[Focal segmental glomerulosclerosis pathophysiology|Pathophysiology]] ==

== [[Focal segmental glomerulosclerosis causes|Causes]] ==

== [[Focal segmental glomerulosclerosis differential diagnosis|Differentiating Focal segmental glomerulosclerosis from other Diseases]] ==

== [[Focal segmental glomerulosclerosis epidemiology and demographics|Epidemiology and Demographics]] ==

== [[Focal segmental glomerulosclerosis risk factors|Risk Factors]] ==

== [[Focal segmental glomerulosclerosis natural history, complications and prognosis|Natural History, Complications and Prognosis]] ==

== Diagnosis ==

[[Focal segmental glomerulosclerosis history and symptoms|History and Symptoms]] | [[Focal segmental glomerulosclerosis physical examination|Physical Examination]] | [[Focal segmental glomerulosclerosis laboratory findings|Laboratory Findings]] | [[Focal segmental glomerulosclerosis chest x ray|Chest X Ray]] | [[Focal segmental glomerulosclerosis CT|CT]] | [[Focal segmental glomerulosclerosis MRI|MRI]] | [[Focal segmental glomerulosclerosis ultrasound|Ultrasound]] | [[Focal segmental glomerulosclerosis other imaging findings|Other Imaging Findings]] | [[Focal segmental glomerulosclerosis other diagnostic studies|Other Diagnostic Studies]]

== Treatment ==

[[Focal segmental glomerulosclerosis medical therapy|Medical Therapy]] | [[Focal segmental glomerulosclerosis surgery|Surgery]] | [[Focal segmental glomerulosclerosis primary prevention|Primary Prevention]] | [[Focal segmental glomerulosclerosis secondary prevention|Secondary Prevention]] | [[Focal segmental glomerulosclerosis cost-effectiveness of therapy| Cost-Effectiveness of Therapy]] | [[Focal segmental glomerulosclerosis future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Focal segmental glomerulosclerosis case study one|Case #1]]

{{Nephrology}}

[[Category:Nephrology]]

[[ja:巣状糸球体硬化症]]
[[pt: Glomeruloesclerose segmentar e focal]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:57:14Z

Olufunmilola: /* When to seek urgent medical care? */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, or present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH and or
CT Scan of the head and other suspected body area to located masses that may be secreting the hormone causing SIADH.

==Treatment options==
Treatment will be based on diagnostic findings and the level of hyponatremia. Removal of the offending medication and simple Water restriction with some loop diuretic may suffice for treatment in patients with mild disease.

In patients, with moderate to severe SIADH, water restriction may not improve symptoms. Drugs like Conivaptan (an approved vasopressin receptors antagonist), lithium and Demeclocycline may be given to increase the plasma sodium level and diuretics to improve the excretion of free water. Patients can also receive hypertonic saline fluids intravenously to prevent central pontine myelinosis (a very severe complication of hyponatremia) in patients with severe hyponatremia.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
Prognosis of SIADH depends on the severity of the disease and the promptness of treatment. Prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH)also depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:52:50Z

Olufunmilola: /* Treatment options */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH and or
CT Scan of the head and other suspected body area to located masses that may be secreting the hormone causing SIADH.

==Treatment options==
Treatment will be based on diagnostic findings and the level of hyponatremia. Removal of the offending medication and simple Water restriction with some loop diuretic may suffice for treatment in patients with mild disease.

In patients, with moderate to severe SIADH, water restriction may not improve symptoms. Drugs like Conivaptan (an approved vasopressin receptors antagonist), lithium and Demeclocycline may be given to increase the plasma sodium level and diuretics to improve the excretion of free water. Patients can also receive hypertonic saline fluids intravenously to prevent central pontine myelinosis (a very severe complication of hyponatremia) in patients with severe hyponatremia.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
Prognosis of SIADH depends on the severity of the disease and the promptness of treatment. Prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH)also depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:52:23Z

Olufunmilola: /* Treatment options */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH and or
CT Scan of the head and other suspected body area to located masses that may be secreting the hormone causing SIADH.

==Treatment options==
Treatment will be based on diagnostic findings and the level of hyponatremia. Removal of the offending medication and simple Water restriction with some loop diuretic may suffice for treatment in patients with mild disease.
In patients, with moderate to severe SIADH, water restriction may not improve symptoms. Drugs like Conivaptan (an approved vasopressin receptors antagonist), lithium and Demeclocycline may be given to increase the plasma sodium level and diuretics to improve the excretion of free water. Patients can also receive hypertonic saline fluids intravenously to prevent central pontine myelinosis (a very severe complication of hyponatremia) in patients with severe hyponatremia.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
Prognosis of SIADH depends on the severity of the disease and the promptness of treatment. Prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH)also depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:48:07Z

Olufunmilola: /* What to expect (Outlook/Prognosis)? */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH and or
CT Scan of the head and other suspected body area to located masses that may be secreting the hormone causing SIADH.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
Prognosis of SIADH depends on the severity of the disease and the promptness of treatment. Prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH)also depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:46:58Z

Olufunmilola: /* What to expect (Outlook/Prognosis)? */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH and or
CT Scan of the head and other suspected body area to located masses that may be secreting the hormone causing SIADH.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
Prognosis of SIADH depends on the severity of the disease and the promptness of treatment.
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:21:46Z

Olufunmilola: /* Diagnosis */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH and or
CT Scan of the head and other suspected body area to located masses that may be secreting the hormone causing SIADH.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:20:08Z

Olufunmilola: /* Diagnosis */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
Diagnosis of SIADH is based on detailed history (especially drug history), clinical signs and symptoms and extensive physical and neurological examination by a physician.
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Urine osmolality: would be greater than blood osmolality in SIADH
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.
Chest x-ray to look for ectopic causes of SAIDH
CT Scan of the head and other suspected body area

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:10:25Z

Olufunmilola: /* When to seek urgent medical care? */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Signs and Symptoms of SIADH
Depending on the degree of the dilution hyponatremia, presentation of SIADH varies. It can be asymptomatic, present with features of mild hyponatremia like
*fatigue,
*vomiting,
*malaise,
*anorexia,
*headache
or it may progress to worsening or altered mental status, seizures, delirium or coma in severe hyponatremia

Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:06:54Z

Olufunmilola: /* What causes SIADH? */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==

The causes of SIADH are many, and because the disease may present with features of electrolyte disturbances, there are many non-associated diseases that present like SIADH.
The most common cause of SIADH is ectopic secretion of the antidiuretic hormone (also called arginine vasopressin) by cancer cells. Cancers like lung or bronchogenic cancers, lymphoma(s) in any location, head and neck cancers, sarcoma and myriad of other cancers can cause SIADH.
Other causes of SIADH include: Pulmonary diseases, central nervous system disorders, as well as pharmacotherapy with anticancer drugs like vincristine, cyclophosphamide; opioids like morphine, antiepileptic and some antidepressants medications.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:04:57Z

Olufunmilola: /* Overview */

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

Syndrome of inappropriate antidiuretic hormone secretion often presents with clinical features of low blood level of sodium (i.e. hyponatremia). It is the most common cause of hyponatremia seen in clinical practice, as hyponatremia is the most common electrolyte abnormality seen in clinical medicine.
SIADH arises when there is inappropriate secretion of the antidiuretic hormone that cause the kidneys to retain free water rather than excreting it leading to fluid accumulation in the body with low blood level of sodium. The low level of sodium is due to the dilution effect of excess free body water and not a shortage; therefore, treatment of low sodium level in SIADH is geared towards removing the excess free water and improving the level of sodium.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==
The most common cause of ectopic [[ADH]] secretion is cancer. Certain lung cancers, as well as some head and neck tumors, are the most common cancers that cause this problem. In rare cases, many other tumors may cause ectopic ADH secretion.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Syndrome of inappropriate antidiuretic hormone (patient information)

2017-01-19T21:02:40Z

Olufunmilola:

'''For the WikiDoc page for this topic, click [[Syndrome of inappropriate antidiuretic hormone|here]]'''

{{Syndrome of inappropriate antidiuretic hormone (patient information)}}

{{CMG}} {{APM}}; '''Associate Editor-In-Chief:''' {{OO}} Jinhui Wu, M.D.

==Overview==
Syndrome of inappropriate antidiuretic hormone production (SIADH) is a condition in which the body develops an excess of water and a decrease in [[sodium]] concentration. It may be caused by central nervous system diseases, [[cancer]]s, [[pulmonary disease]]s and some drugs. Signs and aymptoms vary widely. Some patients with SIADH may become severely ill, or may have no symptoms at all. Usual symptoms include [[nausea]], [[vomiting]], [[loss of appetite]], [[fatigue]], [[weakness]],even [[consciousness disorder]]s. Blood tests of [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg) may prompt the diagnosis of SIADH. Treatment depends on the causes. Sharp restriction of water intake and addition of a high concentration of sodium may get immediate improvement. Prognosis of SIADH varies widely, depending on the causes.

==What are the symptoms of SIADH?==
Symptoms vary, depending on the degree of abnormality in the serum [[sodium]] concentration and the speed with which this concentration falls. Usual symptoms include:

:*[[Nausea]], [[vomiting]]
:*[[Loss of appetite]]
:*[[Fatigue]] and [[weakness]]
:*[[Irritability]]
:*Personality changes, such as combativeness.
:*[[Seizures]]
:*[[Confusion]]
:*[[Hallucination]]s
:*[[Stupor]]
:*[[Coma]]

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

==Diseases with similar symptoms==
:*[[Adrenal insufficiency]]
:*[[Diabetic Ketoacidosis]]
:*[[Hyperglycemia]]
:*[[Pseudohyponatremia]]
:*[[Psychogenic polydipsia]]
:*[[Hypopituitarism]]
:*[[Hypothyroidism]] or [[myxedema coma]]

==What causes SIADH?==
The most common cause of ectopic [[ADH]] secretion is cancer. Certain lung cancers, as well as some head and neck tumors, are the most common cancers that cause this problem. In rare cases, many other tumors may cause ectopic ADH secretion.

==Who is at highest risk?==
:*Central nervous system disease: [[Brain tumor]]s, [[trauma]], [[infection]] such as [[meningitis]] and [[encephalitis]], [[Guillain-Barré syndrome]], [[delirium tremens]], [[multiple sclerosis]]
:*Pulmonary disease: [[Small cell lung cancer]], [[pneumonia]], [[chronic obstructive pulmonary disease]], [[lung abscess]], [[tuberculosis]], [[cystisc fibrosnis]], positive-apressure ventilation
:*[[Cancer]]: [[Lung cancer]], [[pancreas cancer]], [[thymoma]], [[ovarian cancer]], [[lymphoma]], [[laryngeal cancer]], [[Nasopharyngeal carcinoma]], [[bladder cancer]], [[prostate cancer]], [[breast cancer]], [[melanoma]].
:*[[Drug]]s: [[Nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].
:*[[Surgery]]: Damage to the [[hypothalamus]] or [[pituitary gland]] during or after [[surgery]]
:*[[Idiopathic]]

==When to seek urgent medical care?==
Syndrome of inappropriate antidiuretic hormone may be a clinical condition of diseases such as cancers, damages and pulmonary diseases. When you have the diseases mentioned above, you should be alert of the appearance the signs of syndrome of inappropriate antidiuretic hormone. If you experience either of the following symptoms, seek urgent medical care as soon as possible:

:*[[Dehydration]]
:*Consciousness disorders, such as [[confusion]], [[hallucination]]s, [[stupor]] or [[coma]].

==Diagnosis==
:*Serum tests include electrolytes, BUN, creatinine, glucose levels, and osmolality. Patients with syndrome of inappropriate antidiuretic hormone may be characterised with [[hyponatremia]] ([[sodium]] <135 mEq/L) and low serum [[osmolality]] (<280 mOsm/kg).
:*Urine tests: Patients with syndrome of inappropriate antidiuretic hormone show elevated urinary [[sodium]] level (>20 mmol/L) and urine osmolality (generally >100 mOsm/L).
:*Imaging Studies, such as [[x-ray]], [[CT]] and [[MRI]] may be help find the causes of syndrome of inappropriate antidiuretic hormone.

==Treatment options==
Treatment of syndrome of inappropriate antidiuretic hormone depends on the cause.

:*For immediate improvement, all patients with SIADH require sharp restriction of their daily water intake and addition an intravenous infusion of fluid with a high concentration of sodium. (hypertonic saline solution). And, patients may be treated with diuresis to promote water excretion.
:*The most definitive way to relieve SIADH is to deal with the underlying problem itself. If SIADH resulted from [[cancer]]s, [[surgery]], [[radiation therapy]], or [[chemotherapy]] may be helpful. If SIADH produced by [[drug]]s, then the patient must stop taking the medicine. If some infection may be the cause, the patient needs to controlling them by some [[antibiotics]] or other anti-microbiological drugs.

==Where to find medical care for SIADH?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|syndrome of inappropriate antidiuretic hormone}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed Directions to Hospitals Treating syndrome of inappropriate antidiuretic hormone]

==What to expect (Outlook/Prognosis)?==
The prognosis of Syndrome of inappropriate antidiuretic hormone (SIADH) depends largely on its cause. If the cause is [[drug]]s, SIADH usually improves after stopping a [[drug]]. If SIADH is assiciated with some [[infection]], treating the infection may be important. For those SIADH with [[cancer]]s, the outcomes may be poor.

==Prevention==
:*Tell your doctor and monitor blood and urine levels of [[electrolyte]]s and [[osmolality]] when taking drugs such as [[nonsteroidal anti-inflammatory drug]]s, [[nicotine]], [[diuretic]]s, [[chlorpropamide]], [[carbamazepine]], [[tricyclic antidepressant]]s, [[selective serotonin reuptake inhibitor]]s, [[vincristine]], [[thioridazine]], [[cyclophosphamide]], [[clofibrate]], [[bromocriptine]], [[haloperidol]], [[thiothixene]], exogenous [[oxytocin]].

==Sources==
http://www.nlm.nih.gov/medlineplus/ency/article/000314.htm

[[Category:Endocrinology patient information]]
[[Category:Endocrinology]]
[[Category:Nephrology patient information]]
[[Category:Nephrology]]
[[Category:Patient information]]
[[Category:Mature chapter]]
[[Category:Overview complete]]
[[Category:For review]]

{{WH}}
{{WS}}

Nephrotic syndrome classification

2017-01-12T20:46:10Z

Olufunmilola: /* Primary (Idiopathic) Nephrotic Syndrome */

__NOTOC__
{{Nephrotic syndrome}}
{{CMG}} {{APM}}; {{AE}} {{OO}} [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]

==Overview==
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.

==Classification==
===Etiologic Classification===
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.
<center>
{{familytree/start}}
{{familytree | | | |A11| | | | |A11 =Nephrotic syndrome}}
{{familytree | |,|-|-|^|-|-|.|}}
{{familytree | B11 | | | | B12 |B11=Primary|B12=Secondary}}
{{familytree/end}}
</center>
====Primary (Idiopathic) Nephrotic Syndrome====
Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary glomerular disease. It is a common diagnosis in children. Etiologies of idiopathic nephrotic syndrome include:<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>:
*[[Minimal change disease]]
*[[Focal segmental glomerulosclerosis]] (FSGS)
*[[Membranous nephropathy]]
*[[Membranoproliferative glomerulonephritis]]
*[[IgM nephropathy]]
*C1q nephropathy
* Hereditary Nephropathies: are group of disorders that present with both the features of nephrotic and nephritic syndromes. When features of nephritic syndrome are associated with sensorineural deafness, it is called Alport’s syndrome. Other hereditary nephropathies include IgA Nephropathy, C1q Nephropathy, etc.

====Secondary Nephrotic Syndrome====
Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s. The most common cause of secondary nephrotic syndrome is [[diabetes mellitus]].

{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Common Causes of Secondary Nephrotic Syndrome<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref><ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>'''''
| bgcolor="#d9ff54"|'''Cause''' || bgcolor="#d9ff54"|'''Characteristic Features'''
|-
| bgcolor="#ececec"|'''[[Diabetes Mellitus]]''' ||
*[[Glucosuria]]
*[[Hyperglycemia]]
*[[Polyuria]]
*[[Polydipsia]]
|-
| bgcolor="#ececec"|'''[[Systemic Lupus Erythematosus]] (SLE)''' ||
*[[Anemia]]
*[[Arthralgia]]
*[[Malar rash|Malar]] or discoid rash
*[[Photosensitivity]]
*Neurological, renal, immunologic disease
*[[Pleural effusion]] or [[ascites]]
*Positive [[ANA]] and [[anti-dsDNA]] antibodies
|-
| bgcolor="#ececec"|'''[[Viral Hepatitis]] ([[HBV]] and [[HCV]])''' ||
*Elevated [[liver function test]]s
*History of transfusions
*High-risk sexual encounters
*IV drug use
*Positive HBsAg or [[HCV]] RNA
|-
| bgcolor="#ececec"|'''[[NSAID]]s''' ||
*Associated with minimal change disease (MCD)
|-
|bgcolor="#ececec"|'''[[Amyloidosis]]''' ||

*[[Cardiomyopathy]]
*[[Hepatomegaly]]
*[[Peripheral neuropathy]]
|-
| bgcolor="#ececec"|'''[[Multiple Myeloma]]''' ||
*Abrnomal [[urine protein electrophoresis]] (UPEP) and [[serum protein electrophoresis]] (SPEP)
*[[Back pain]]
*[[Renal failure]]
*[[Anemia]]
*[[Hypercalcemia]]
*Positive serum beta-2-microglobulin
|-
| bgcolor="#ececec"|'''[[HIV]]''' ||
*Reduced [[CD4]] cell counts
*High-risk sexual encounters
*History of transfusions
*History of drug use
*Renal pathology similar to [[focal segmental glomerulosclerosis]] (FSGS)
|-
| bgcolor="#ececec"|'''[[Preeclampsia]]''' ||
*[[High blood pressure]], [[edema]], and [[proteinuria]] during pregnancy
|}

<center>Adapted from Hull RP, Goldsmith DJ. Nephrotic syndrome in adults. BMJ. 2008; 336:1185-9</center>

==References==
{{Reflist|2}}
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Needs overview]]
[[Category:Primary care]]
{{WH}}
{{WS}}

Nephrotic syndrome classification

2017-01-12T20:41:31Z

Olufunmilola:

__NOTOC__
{{Nephrotic syndrome}}
{{CMG}} {{APM}}; {{AE}} {{OO}} [[User:YazanDaaboul|Yazan Daaboul]], [[User:Sergekorjian|Serge Korjian]]

==Overview==
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.

==Classification==
===Etiologic Classification===
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology.
<center>
{{familytree/start}}
{{familytree | | | |A11| | | | |A11 =Nephrotic syndrome}}
{{familytree | |,|-|-|^|-|-|.|}}
{{familytree | B11 | | | | B12 |B11=Primary|B12=Secondary}}
{{familytree/end}}
</center>
====Primary (Idiopathic) Nephrotic Syndrome====
Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary glomerular disease. It is a common diagnosis in children. Etiologies of idiopathic nephrotic syndrome include:<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>:
*[[Minimal change disease]]
*[[Focal segmental glomerulosclerosis]] (FSGS)
*[[Membranous nephropathy]]
*[[Membranoproliferative glomerulonephritis]]
*[[IgM nephropathy]]
*C1q nephropathy

====Secondary Nephrotic Syndrome====
Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s. The most common cause of secondary nephrotic syndrome is [[diabetes mellitus]].

{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Common Causes of Secondary Nephrotic Syndrome<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref><ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>'''''
| bgcolor="#d9ff54"|'''Cause''' || bgcolor="#d9ff54"|'''Characteristic Features'''
|-
| bgcolor="#ececec"|'''[[Diabetes Mellitus]]''' ||
*[[Glucosuria]]
*[[Hyperglycemia]]
*[[Polyuria]]
*[[Polydipsia]]
|-
| bgcolor="#ececec"|'''[[Systemic Lupus Erythematosus]] (SLE)''' ||
*[[Anemia]]
*[[Arthralgia]]
*[[Malar rash|Malar]] or discoid rash
*[[Photosensitivity]]
*Neurological, renal, immunologic disease
*[[Pleural effusion]] or [[ascites]]
*Positive [[ANA]] and [[anti-dsDNA]] antibodies
|-
| bgcolor="#ececec"|'''[[Viral Hepatitis]] ([[HBV]] and [[HCV]])''' ||
*Elevated [[liver function test]]s
*History of transfusions
*High-risk sexual encounters
*IV drug use
*Positive HBsAg or [[HCV]] RNA
|-
| bgcolor="#ececec"|'''[[NSAID]]s''' ||
*Associated with minimal change disease (MCD)
|-
|bgcolor="#ececec"|'''[[Amyloidosis]]''' ||

*[[Cardiomyopathy]]
*[[Hepatomegaly]]
*[[Peripheral neuropathy]]
|-
| bgcolor="#ececec"|'''[[Multiple Myeloma]]''' ||
*Abrnomal [[urine protein electrophoresis]] (UPEP) and [[serum protein electrophoresis]] (SPEP)
*[[Back pain]]
*[[Renal failure]]
*[[Anemia]]
*[[Hypercalcemia]]
*Positive serum beta-2-microglobulin
|-
| bgcolor="#ececec"|'''[[HIV]]''' ||
*Reduced [[CD4]] cell counts
*High-risk sexual encounters
*History of transfusions
*History of drug use
*Renal pathology similar to [[focal segmental glomerulosclerosis]] (FSGS)
|-
| bgcolor="#ececec"|'''[[Preeclampsia]]''' ||
*[[High blood pressure]], [[edema]], and [[proteinuria]] during pregnancy
|}

<center>Adapted from Hull RP, Goldsmith DJ. Nephrotic syndrome in adults. BMJ. 2008; 336:1185-9</center>

==References==
{{Reflist|2}}
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Needs overview]]
[[Category:Primary care]]
{{WH}}
{{WS}}

Nephrotic syndrome overview

2017-01-12T20:40:35Z

Olufunmilola: /* Overview */

__NOTOC__
{{Nephrotic syndrome}}

{{CMG}} {{APM}} {{AE}}{{OO}}

==Overview==
Nephrotic syndrome is group of signs and symptoms resulting from loss of kidney filtration capabilities leading to massive loss of protein in urine (>3.5g of protein in urine per 24hours), generalized or localized body edema, hyperlipidemia and hypoproteinemia (most importantly, hypoalbuminemia). Causes of Nephrotic Syndrome can be primary (idiopathic) or secondary (from a systemic insult or immune mediated).

Nephrotic syndrome (nephrosis) is defined as heavy proteinuria > 3.5 grams per 24 hours in adults. In children, nephrotic syndrome is defined as protein excretion > 40 mg/m2/h. The accurate diagnosis of nephrotic syndrome thus requires 24-hour urine collection. However, in clinical practice, urine dipstick of a qualitative measure of 3+ urinary proteins, or spot urine protein (mg)/creatinine(mg) ratio > 2 may also reflect nephrotic syndrome.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

Clinically, nephrotic syndrome is defined as<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ|title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref>:
*Heavy proteinuria > 3.5 g/24 hrs
*Peripheral edema
*Hypoalbuminemia
*Hyperlipidemia and lipiduria
*Hypercoagulability

==Classification==
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.

==Pathophysiology==
The pathophysiology of [[hypoalbuminemia]] in nephrotic syndrome is multifactorial. [[Proteinuria]] plays an important role in the pathogenesis of [[hyperlipidemia]] in nephrotic syndrome.<ref name="pmid6742202">{{cite journal| author=Kaysen GA, Kirkpatrick WG, Couser WG| title=Albumin homeostasis in the nephrotic rat: nutritional considerations. | journal=Am J Physiol | year= 1984 | volume= 247 | issue= 1 Pt 2 | pages= F192-202 | pmid=6742202 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6742202 }} </ref> Neurohormonal changes in the [[renin-angiotensin-aldosterone system]], [[vasopressin]], [[atrial natriuretic peptide]] ([[ANP]]), and [[sympathetic nervous system]] are is implicated in [[edema]] formation in nephrotic syndrome.<ref name="pmid22718186">{{cite journal| author=Siddall EC, Radhakrishnan J| title=The pathophysiology of edema formation in the nephrotic syndrome. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 6 | pages= 635-42 | pmid=22718186 | doi=10.1038/ki.2012.180 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22718186 }} </ref>

==Causes==
Nephrotic syndrome can be either primary or secondary to diseases. Primary renal disorders, such as primary [[glomerulonephritides]], may cause primary nephrotic syndrome. The most common cause of secondary nephrotic syndrome in adults is [[diabetes mellitus]].

==Differential Diagnosis==
The differential diagnosis of nephrotic syndrome includes other diseases with similar findings on physical examination, such as [[lower extremity edema]], or similar clinical findings, such as [[hypoalbuminemia]]. Differential diagnoses include [[congestive heart failure]], [[liver cirrhosis]], [[protein losing enteropathy]], [[malignancy]], [[lymphedema]], [[venous insufficiency]], and [[malnutrition]].

==Epidemiology and Demographics==
Idiopathic nephrotic syndrome has an incidence of 2-7 cases per 100,000 and a prevalence of 16 cases per 100,000.<ref name="pmid12944064">{{cite journal| author=Eddy AA, Symons JM| title=Nephrotic syndrome in childhood. | journal=Lancet | year= 2003 | volume= 362 | issue= 9384 | pages= 629-39 | pmid=12944064 | doi=10.1016/S0140-6736(03)14184-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12944064 }} </ref> Nephrotic syndrome may affect children and adults alike. There is no age or ethnic predominance. The prevalence of nephrotic syndrome in children has a 2 to 1 male to female ratio.<ref name="pmid7205481">{{cite journal| author=| title=The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. | journal=J Pediatr | year= 1981 | volume= 98 | issue= 4 | pages= 561-4 | pmid=7205481 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7205481 }} </ref>

==Natural History, Complications and Prognosis==
Complications of nephrotic syndrome include [[infection]]s, [[thrombotic]] events, and [[renal failure]]. Mortality and overall prognosis depends on the occurrence of complications and adherence to medications.<ref name="pmid12944064">{{cite journal| author=Eddy AA, Symons JM| title=Nephrotic syndrome in childhood. | journal=Lancet | year= 2003 | volume= 362 | issue= 9384 | pages= 629-39 | pmid=12944064 | doi=10.1016/S0140-6736(03)14184-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12944064 }} </ref>

==Diagnosis==
===History and Symptoms===
Symptoms of nephrotic syndrome include [[edema]], [[dyspnea]], [[abdominal fullness]], [[anorexia]], and [[fatigue]].

===Physical Examination===
A full physical examination should be performed among patients presenting with nephrotic syndrome. Findings on physical examination suggestive of secondary etiologies may be present, such as characteristic [[rash]] in [[systemic lupus erythematosus]] (SLE), or [[peripheral neuropathy]] in [[diabetes mellitus]].

===Laboratory Findings===
Nephrotic syndrome is characterized by the following laboratory findings: [[proteinuria]] > 3.5g/24 hrs on 24-hour urine collection, [[proteinuria]] on urine dipstick, and urine protein/creatinine ratio > 3. When nephrotic syndrome is diagnosed ([[proteinuria]] > 3.5 g/24 hrs), additional laboratory tests are required such as [[serum albumin]] concentration, serum chemistry panel, lipid panel, and serum [[creatinine]] concentration.

===Chest X-Ray===
Chest X-ray may show signs of [[pleural effusion]].<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

===Ultrasound===
Renal and abdominal doppler ultrasound may be required to investigate for renal etiologies and complications of disease, such as [[renal vein thrombosis]].<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref> Kidney size and signs of obstruction during assessment are also important.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref> [[Doppler ultrasound]] of the extremities is indicated if patients with nephrotic syndrome present with suspected [[deep vein thrombosis]].

===Other Imaging Findings===
[[Renal vein thrombosis]], a complication of nephrotic syndrome, may require any of [[venography]], [[CT scan]], or [[MRI]] for appropriate diagnosis.<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref>

===Biopsy===
[[Ultrasound]]-guided renal biopsy for visualization under [[light microscopy]], [[immunofluorescence]] or immunoperoxidase, and [[electron microscopy]] is usually recommended for patients with nephrotic syndrome.<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref> Renal biopsy provides diagnostic and prognostic benefit. However, guidelines that define the timing and the circumstances to perform renal [[biopsy]] are not present. In [[minimal change disease]], the most common primary cause of nephrotic syndrome in children, and in [[diabetic nephropathy]], the most common secondary cause of nephrotic syndrome in adults, renal biopsy is not generally recommended and is not routinely performed.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref> Nonetheless, patients who present with unknown or unsure etiology of nephrotic syndrome are recommended to undergo renal biopsy for definitive diagnosis.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

==Treatment==

===Medical Therapy===
There are currently no guidelines for the management of [[edema]] associated with nephrotic syndrome. The slow reversal of edema is important at a rate of 0.5-1 kg daily to prevent [[electrolyte disturbance]]s, [[hypotension]], ischemic [[acute tubular necrosis]], and hemoconcentration associated with aggressive diuretic therapy.<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref><ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref> Since proteinuria is one of the most significant factors for progression of disease and is associated with outcome<ref name="pmid9573535">{{cite journal| author=Ruggenenti P, Perna A, Mosconi L, Pisoni R, Remuzzi G| title=Urinary protein excretion rate is the best independent predictor of ESRF in non-diabetic proteinuric chronic nephropathies. "Gruppo Italiano di Studi Epidemiologici in Nefrologia" (GISEN). | journal=Kidney Int | year= 1998 | volume= 53 | issue= 5 | pages= 1209-16 | pmid=9573535 | doi=10.1046/j.1523-1755.1998.00874.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9573535 }} </ref><ref name="pmid8710157">{{cite journal| author=Locatelli F, Marcelli D, Comelli M, Alberti D, Graziani G, Buccianti G et al.| title=Proteinuria and blood pressure as causal components of progression to end-stage renal failure. Northern Italian Cooperative Study Group. | journal=Nephrol Dial Transplant | year= 1996 | volume= 11 | issue= 3 | pages= 461-7 | pmid=8710157 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8710157 }} </ref>, treatment of [[proteinuria]] in nephrotic syndrome must always be considered a priority. [[Angiotensin-converting enzyme inhibitors]] (ACE-I), with or without [[angiotensin-II receptor blocker]]s (ARB) have been extensively studied and are well-known to decrease [[proteinuria]] and the risk of progression of renal disease in patients with nephrotic syndrome.<ref name="pmid8643149">{{cite journal| author=Gansevoort RT, Sluiter WJ, Hemmelder MH, de Zeeuw D, de Jong PE| title=Antiproteinuric effect of blood-pressure-lowering agents: a meta-analysis of comparative trials. | journal=Nephrol Dial Transplant | year= 1995 | volume= 10 | issue= 11 | pages= 1963-74 | pmid=8643149 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8643149 }} </ref><ref name="pmid9217756">{{cite journal| author=| title=Randomised placebo-controlled trial of effect of ramipril on decline in glomerular filtration rate and risk of terminal renal failure in proteinuric, non-diabetic nephropathy. The GISEN Group (Gruppo Italiano di Studi Epidemiologici in Nefrologia) | journal=Lancet | year= 1997 | volume= 349 | issue= 9069 | pages= 1857-63 | pmid=9217756 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9217756 }} </ref><ref name="pmid12531578">{{cite journal| author=Nakao N, Yoshimura A, Morita H, Takada M, Kayano T, Ideura T| title=Combination treatment of angiotensin-II receptor blocker and angiotensin-converting-enzyme inhibitor in non-diabetic renal disease (COOPERATE): a randomised controlled trial. | journal=Lancet | year= 2003 | volume= 361 | issue= 9352 | pages= 117-24 | pmid=12531578 | doi=10.1016/S0140-6736(03)12229-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12531578 }} [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12954032 Review in: ACP J Club. 2003 Sep-Oct;139(2):40] </ref><ref name="pmid10692263">{{cite journal| author=Ruggenenti P, Mosconi L, Vendramin G, Moriggi M, Remuzzi A, Sangalli F et al.| title=ACE inhibition improves glomerular size selectivity in patients with idiopathic membranous nephropathy and persistent nephrotic syndrome. | journal=Am J Kidney Dis | year= 2000 | volume= 35 | issue= 3 | pages= 381-91 | pmid=10692263 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10692263 }} </ref><ref name="pmid12704582">{{cite journal| author=Korbet SM| title=Angiotensin antagonists and steroids in the treatment of focal segmental glomerulosclerosis. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 219-28 | pmid=12704582 | doi=10.1053/snep.2003.50020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704582 }} </ref> [[Pneumococcal vaccine]]s are recommended for all patients with nephrotic syndrome.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

===Primary Prevention===
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==References==
{{Reflist|2}}
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]
{{WH}}
{{WS}}

Nephrotic syndrome overview

2017-01-12T20:39:28Z

Olufunmilola:

__NOTOC__
{{Nephrotic syndrome}}

{{CMG}} {{APM}} {{AE}}{{OO}}

==Overview==
Nephrotic syndrome (nephrosis) is defined as heavy proteinuria > 3.5 grams per 24 hours in adults. In children, nephrotic syndrome is defined as protein excretion > 40 mg/m2/h. The accurate diagnosis of nephrotic syndrome thus requires 24-hour urine collection. However, in clinical practice, urine dipstick of a qualitative measure of 3+ urinary proteins, or spot urine protein (mg)/creatinine(mg) ratio > 2 may also reflect nephrotic syndrome.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

Clinically, nephrotic syndrome is defined as<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ|title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref>:
*Heavy proteinuria > 3.5 g/24 hrs
*Peripheral edema
*Hypoalbuminemia
*Hyperlipidemia and lipiduria
*Hypercoagulability

==Classification==
Nephrotic syndrome can be classified into primary or secondary depending on the underlying etiology. Primary (idiopathic) nephrotic syndrome is defined as nephrotic syndrome due to a primary [[glomerular]] disease. Secondary nephrotic syndrome is defined as nephrotic syndrome due to a primary etiology other than glomerular disorders, such as [[infection]]s, [[malignancies]], systemic conditions, and [[medication]]s.

==Pathophysiology==
The pathophysiology of [[hypoalbuminemia]] in nephrotic syndrome is multifactorial. [[Proteinuria]] plays an important role in the pathogenesis of [[hyperlipidemia]] in nephrotic syndrome.<ref name="pmid6742202">{{cite journal| author=Kaysen GA, Kirkpatrick WG, Couser WG| title=Albumin homeostasis in the nephrotic rat: nutritional considerations. | journal=Am J Physiol | year= 1984 | volume= 247 | issue= 1 Pt 2 | pages= F192-202 | pmid=6742202 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6742202 }} </ref> Neurohormonal changes in the [[renin-angiotensin-aldosterone system]], [[vasopressin]], [[atrial natriuretic peptide]] ([[ANP]]), and [[sympathetic nervous system]] are is implicated in [[edema]] formation in nephrotic syndrome.<ref name="pmid22718186">{{cite journal| author=Siddall EC, Radhakrishnan J| title=The pathophysiology of edema formation in the nephrotic syndrome. | journal=Kidney Int | year= 2012 | volume= 82 | issue= 6 | pages= 635-42 | pmid=22718186 | doi=10.1038/ki.2012.180 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22718186 }} </ref>

==Causes==
Nephrotic syndrome can be either primary or secondary to diseases. Primary renal disorders, such as primary [[glomerulonephritides]], may cause primary nephrotic syndrome. The most common cause of secondary nephrotic syndrome in adults is [[diabetes mellitus]].

==Differential Diagnosis==
The differential diagnosis of nephrotic syndrome includes other diseases with similar findings on physical examination, such as [[lower extremity edema]], or similar clinical findings, such as [[hypoalbuminemia]]. Differential diagnoses include [[congestive heart failure]], [[liver cirrhosis]], [[protein losing enteropathy]], [[malignancy]], [[lymphedema]], [[venous insufficiency]], and [[malnutrition]].

==Epidemiology and Demographics==
Idiopathic nephrotic syndrome has an incidence of 2-7 cases per 100,000 and a prevalence of 16 cases per 100,000.<ref name="pmid12944064">{{cite journal| author=Eddy AA, Symons JM| title=Nephrotic syndrome in childhood. | journal=Lancet | year= 2003 | volume= 362 | issue= 9384 | pages= 629-39 | pmid=12944064 | doi=10.1016/S0140-6736(03)14184-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12944064 }} </ref> Nephrotic syndrome may affect children and adults alike. There is no age or ethnic predominance. The prevalence of nephrotic syndrome in children has a 2 to 1 male to female ratio.<ref name="pmid7205481">{{cite journal| author=| title=The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. | journal=J Pediatr | year= 1981 | volume= 98 | issue= 4 | pages= 561-4 | pmid=7205481 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7205481 }} </ref>

==Natural History, Complications and Prognosis==
Complications of nephrotic syndrome include [[infection]]s, [[thrombotic]] events, and [[renal failure]]. Mortality and overall prognosis depends on the occurrence of complications and adherence to medications.<ref name="pmid12944064">{{cite journal| author=Eddy AA, Symons JM| title=Nephrotic syndrome in childhood. | journal=Lancet | year= 2003 | volume= 362 | issue= 9384 | pages= 629-39 | pmid=12944064 | doi=10.1016/S0140-6736(03)14184-0 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12944064 }} </ref>

==Diagnosis==
===History and Symptoms===
Symptoms of nephrotic syndrome include [[edema]], [[dyspnea]], [[abdominal fullness]], [[anorexia]], and [[fatigue]].

===Physical Examination===
A full physical examination should be performed among patients presenting with nephrotic syndrome. Findings on physical examination suggestive of secondary etiologies may be present, such as characteristic [[rash]] in [[systemic lupus erythematosus]] (SLE), or [[peripheral neuropathy]] in [[diabetes mellitus]].

===Laboratory Findings===
Nephrotic syndrome is characterized by the following laboratory findings: [[proteinuria]] > 3.5g/24 hrs on 24-hour urine collection, [[proteinuria]] on urine dipstick, and urine protein/creatinine ratio > 3. When nephrotic syndrome is diagnosed ([[proteinuria]] > 3.5 g/24 hrs), additional laboratory tests are required such as [[serum albumin]] concentration, serum chemistry panel, lipid panel, and serum [[creatinine]] concentration.

===Chest X-Ray===
Chest X-ray may show signs of [[pleural effusion]].<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

===Ultrasound===
Renal and abdominal doppler ultrasound may be required to investigate for renal etiologies and complications of disease, such as [[renal vein thrombosis]].<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref> Kidney size and signs of obstruction during assessment are also important.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref> [[Doppler ultrasound]] of the extremities is indicated if patients with nephrotic syndrome present with suspected [[deep vein thrombosis]].

===Other Imaging Findings===
[[Renal vein thrombosis]], a complication of nephrotic syndrome, may require any of [[venography]], [[CT scan]], or [[MRI]] for appropriate diagnosis.<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref>

===Biopsy===
[[Ultrasound]]-guided renal biopsy for visualization under [[light microscopy]], [[immunofluorescence]] or immunoperoxidase, and [[electron microscopy]] is usually recommended for patients with nephrotic syndrome.<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref> Renal biopsy provides diagnostic and prognostic benefit. However, guidelines that define the timing and the circumstances to perform renal [[biopsy]] are not present. In [[minimal change disease]], the most common primary cause of nephrotic syndrome in children, and in [[diabetic nephropathy]], the most common secondary cause of nephrotic syndrome in adults, renal biopsy is not generally recommended and is not routinely performed.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref> Nonetheless, patients who present with unknown or unsure etiology of nephrotic syndrome are recommended to undergo renal biopsy for definitive diagnosis.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

==Treatment==

===Medical Therapy===
There are currently no guidelines for the management of [[edema]] associated with nephrotic syndrome. The slow reversal of edema is important at a rate of 0.5-1 kg daily to prevent [[electrolyte disturbance]]s, [[hypotension]], ischemic [[acute tubular necrosis]], and hemoconcentration associated with aggressive diuretic therapy.<ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref><ref name="pmid18497417">{{cite journal| author=Hull RP, Goldsmith DJ| title=Nephrotic syndrome in adults. | journal=BMJ | year= 2008 | volume= 336 | issue= 7654 | pages= 1185-9 | pmid=18497417 | doi=10.1136/bmj.39576.709711.80 | pmc=PMC2394708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18497417 }} </ref> Since proteinuria is one of the most significant factors for progression of disease and is associated with outcome<ref name="pmid9573535">{{cite journal| author=Ruggenenti P, Perna A, Mosconi L, Pisoni R, Remuzzi G| title=Urinary protein excretion rate is the best independent predictor of ESRF in non-diabetic proteinuric chronic nephropathies. "Gruppo Italiano di Studi Epidemiologici in Nefrologia" (GISEN). | journal=Kidney Int | year= 1998 | volume= 53 | issue= 5 | pages= 1209-16 | pmid=9573535 | doi=10.1046/j.1523-1755.1998.00874.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9573535 }} </ref><ref name="pmid8710157">{{cite journal| author=Locatelli F, Marcelli D, Comelli M, Alberti D, Graziani G, Buccianti G et al.| title=Proteinuria and blood pressure as causal components of progression to end-stage renal failure. Northern Italian Cooperative Study Group. | journal=Nephrol Dial Transplant | year= 1996 | volume= 11 | issue= 3 | pages= 461-7 | pmid=8710157 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8710157 }} </ref>, treatment of [[proteinuria]] in nephrotic syndrome must always be considered a priority. [[Angiotensin-converting enzyme inhibitors]] (ACE-I), with or without [[angiotensin-II receptor blocker]]s (ARB) have been extensively studied and are well-known to decrease [[proteinuria]] and the risk of progression of renal disease in patients with nephrotic syndrome.<ref name="pmid8643149">{{cite journal| author=Gansevoort RT, Sluiter WJ, Hemmelder MH, de Zeeuw D, de Jong PE| title=Antiproteinuric effect of blood-pressure-lowering agents: a meta-analysis of comparative trials. | journal=Nephrol Dial Transplant | year= 1995 | volume= 10 | issue= 11 | pages= 1963-74 | pmid=8643149 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8643149 }} </ref><ref name="pmid9217756">{{cite journal| author=| title=Randomised placebo-controlled trial of effect of ramipril on decline in glomerular filtration rate and risk of terminal renal failure in proteinuric, non-diabetic nephropathy. The GISEN Group (Gruppo Italiano di Studi Epidemiologici in Nefrologia) | journal=Lancet | year= 1997 | volume= 349 | issue= 9069 | pages= 1857-63 | pmid=9217756 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9217756 }} </ref><ref name="pmid12531578">{{cite journal| author=Nakao N, Yoshimura A, Morita H, Takada M, Kayano T, Ideura T| title=Combination treatment of angiotensin-II receptor blocker and angiotensin-converting-enzyme inhibitor in non-diabetic renal disease (COOPERATE): a randomised controlled trial. | journal=Lancet | year= 2003 | volume= 361 | issue= 9352 | pages= 117-24 | pmid=12531578 | doi=10.1016/S0140-6736(03)12229-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12531578 }} [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12954032 Review in: ACP J Club. 2003 Sep-Oct;139(2):40] </ref><ref name="pmid10692263">{{cite journal| author=Ruggenenti P, Mosconi L, Vendramin G, Moriggi M, Remuzzi A, Sangalli F et al.| title=ACE inhibition improves glomerular size selectivity in patients with idiopathic membranous nephropathy and persistent nephrotic syndrome. | journal=Am J Kidney Dis | year= 2000 | volume= 35 | issue= 3 | pages= 381-91 | pmid=10692263 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10692263 }} </ref><ref name="pmid12704582">{{cite journal| author=Korbet SM| title=Angiotensin antagonists and steroids in the treatment of focal segmental glomerulosclerosis. | journal=Semin Nephrol | year= 2003 | volume= 23 | issue= 2 | pages= 219-28 | pmid=12704582 | doi=10.1053/snep.2003.50020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12704582 }} </ref> [[Pneumococcal vaccine]]s are recommended for all patients with nephrotic syndrome.<ref name="pmid19904897">{{cite journal| author=Kodner C| title=Nephrotic syndrome in adults: diagnosis and management. | journal=Am Fam Physician | year= 2009 | volume= 80 | issue= 10 | pages= 1129-34 | pmid=19904897 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904897 }} </ref>

===Primary Prevention===
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==References==
{{Reflist|2}}
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]
{{WH}}
{{WS}}

Nephrotic syndrome (patient information)

2017-01-12T20:29:22Z

Olufunmilola: /* Prevention */

__NOTOC__
'''For the WikiDoc page for this topic, click [[Nephrotic syndrome|here]]'''

{{Nephrotic syndrome (patient information)}}

'''Editors-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]]{{APM}} '''Associate Editor-In-Chief''': {{OO}} [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]

==Overview==

Nephrotic syndrome is a group of signs and symptoms of a kidney filtering system disease (glomerular disease) which is associated with massive loss of protein in urine (called proteinuria), body or leg swelling (called edema), excessive or abnormal blood clotting (hypercoagulability) and high blood fat content (called hyperlipidemia). The massive loss of protein in the urine results in low level of essential proteins (primarily the essential protein called albumin) in blood leading to hypoalbuminemia.

Key Words and their meanings
*Hyper: excess
*Hypo: little or low level
*Albumin: essential amino acid.
*Amino Acids: building blocks for proteins
*Lipids: Fat
*Glomeruli: basic unit of the kidney filtration system

Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

==What are the symptoms of Nephrotic syndrome?==
Swelling is the most common symptom. It may occur:

*In the face and around the eyes.
*In the arms and legs, especially in the feet and ankles
*In the belly area (swollen abdomen)
Other symptoms include:

*Foamy appearance of the urine
*Weight gain (unintentional) from fluid retention
*Poor appetite
*High blood pressure

'''Patients that experience any of the above signs and symptoms should see their primary care physician immediately.'''

==What causes Nephrotic syndrome?==

Causes of Nephrotic Syndrome can be primary (that is; from the kidney glomeruli) or secondary (from an external insult to the kidney).
Primary cause of Nephrotic Syndrome is often referred to as Idiopathic i.e. there are no known causes for the kidney abnormality. Such is the case in Minimal change kidney disease seen in children.
Primary causes of nephrotic syndrome are usually described by the [[histology]], e.g. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). These diseases are considered to be "[[diagnosis of exclusion|diagnoses of exclusion]]," meaning they are diagnosed only after secondary causes have been excluded.

Secondary Nephrotic Syndrome is nephrotic syndrome that is associated with well-known kidney diseases like autoimmune disease like Henoin Scholein purpura, IgA nephropathy, lupus nephritis etc.
Secondary Nephrotic syndrome is also associated with post streptococcal glomerulonephritis, Alport's syndrome. It has also been associated with allergic reactions, drugs like NSAIDs, vaccination like the influenza and pneumococcal vaccines, tumors and viral infections.

A complete list of possible causes of nephrotic syndrome can be found [[Nephrotic syndrome causes|here]].

==Diagnosis==
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:

*Creatine - blood test
*Blood urea nitrogen (BUN)
*Creatinine clearance
*Albumin blood test - may be low
*Urinalysis - reveals large amounts of urine protein
*Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.

Kidney biopsy may be needed.

Tests to rule out various causes may include the following:

*[[Glucose tolerance test]]
*[[Antinuclear antibody]]
*[[Rheumatoid factor]]
*[[Cryoglobulins]]
*Complement levels
*Hepatitis B and C antibodies
*[[VDRL]] serology
*Serum protein electrophoresis
This disease may also alter the results of the following tests:

*Urinary casts
*Protein electrophoresis - urine
*Serum iron

==When to seek urgent medical care?==
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.

Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.

Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

==Treatment options==
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.

Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.

Corticosteroids and other drugs that suppress or quiet the immune system may be used.

High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.

A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.

Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.

Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.

Blood thinners may be required to treat or prevent clot formation.

====Medications to avoid====

{{MedCondContrPI

|MedCond = nephrosis|Oxandrolone}}

==Where to find medical care for Nephrotic syndrome?==
[http://maps.google.com/maps?rlz=1C1_____enUS444US444&q=nephrotic%20syndrome&safe=active&um=1&ie=UTF-8&sa=N&hl=en&tab=wl Directions to Hospitals Treating Nephrotic syndrome]

==Prevention==
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome. It is advised that Nephrotic Syndrome patients should receive the influenza vaccine annually and the pneumococcal vaccine when due.

==What to expect (Outlook/Prognosis)?==
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
==Possible complications==
*[[Atherosclerosis]] and related heart diseases
*[[Renal vein thrombosis]]
*[[Acute kidney failure]]
*[[Chronic kidney disease]]
*Infections, including pneumococcal pneumonia
*[[Malnutrition]]
*Fluid overload, congestive heart failure, pulmonary edema

{{WH}}
{{WS}}

[[Category:Patient information]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

Nephrotic syndrome (patient information)

2017-01-12T20:27:25Z

Olufunmilola: /* What causes Nephrotic syndrome? */

__NOTOC__
'''For the WikiDoc page for this topic, click [[Nephrotic syndrome|here]]'''

{{Nephrotic syndrome (patient information)}}

'''Editors-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]]{{APM}} '''Associate Editor-In-Chief''': {{OO}} [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]

==Overview==

Nephrotic syndrome is a group of signs and symptoms of a kidney filtering system disease (glomerular disease) which is associated with massive loss of protein in urine (called proteinuria), body or leg swelling (called edema), excessive or abnormal blood clotting (hypercoagulability) and high blood fat content (called hyperlipidemia). The massive loss of protein in the urine results in low level of essential proteins (primarily the essential protein called albumin) in blood leading to hypoalbuminemia.

Key Words and their meanings
*Hyper: excess
*Hypo: little or low level
*Albumin: essential amino acid.
*Amino Acids: building blocks for proteins
*Lipids: Fat
*Glomeruli: basic unit of the kidney filtration system

Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

==What are the symptoms of Nephrotic syndrome?==
Swelling is the most common symptom. It may occur:

*In the face and around the eyes.
*In the arms and legs, especially in the feet and ankles
*In the belly area (swollen abdomen)
Other symptoms include:

*Foamy appearance of the urine
*Weight gain (unintentional) from fluid retention
*Poor appetite
*High blood pressure

'''Patients that experience any of the above signs and symptoms should see their primary care physician immediately.'''

==What causes Nephrotic syndrome?==

Causes of Nephrotic Syndrome can be primary (that is; from the kidney glomeruli) or secondary (from an external insult to the kidney).
Primary cause of Nephrotic Syndrome is often referred to as Idiopathic i.e. there are no known causes for the kidney abnormality. Such is the case in Minimal change kidney disease seen in children.
Primary causes of nephrotic syndrome are usually described by the [[histology]], e.g. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). These diseases are considered to be "[[diagnosis of exclusion|diagnoses of exclusion]]," meaning they are diagnosed only after secondary causes have been excluded.

Secondary Nephrotic Syndrome is nephrotic syndrome that is associated with well-known kidney diseases like autoimmune disease like Henoin Scholein purpura, IgA nephropathy, lupus nephritis etc.
Secondary Nephrotic syndrome is also associated with post streptococcal glomerulonephritis, Alport's syndrome. It has also been associated with allergic reactions, drugs like NSAIDs, vaccination like the influenza and pneumococcal vaccines, tumors and viral infections.

A complete list of possible causes of nephrotic syndrome can be found [[Nephrotic syndrome causes|here]].

==Diagnosis==
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:

*Creatine - blood test
*Blood urea nitrogen (BUN)
*Creatinine clearance
*Albumin blood test - may be low
*Urinalysis - reveals large amounts of urine protein
*Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.

Kidney biopsy may be needed.

Tests to rule out various causes may include the following:

*[[Glucose tolerance test]]
*[[Antinuclear antibody]]
*[[Rheumatoid factor]]
*[[Cryoglobulins]]
*Complement levels
*Hepatitis B and C antibodies
*[[VDRL]] serology
*Serum protein electrophoresis
This disease may also alter the results of the following tests:

*Urinary casts
*Protein electrophoresis - urine
*Serum iron

==When to seek urgent medical care?==
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.

Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.

Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

==Treatment options==
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.

Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.

Corticosteroids and other drugs that suppress or quiet the immune system may be used.

High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.

A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.

Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.

Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.

Blood thinners may be required to treat or prevent clot formation.

====Medications to avoid====

{{MedCondContrPI

|MedCond = nephrosis|Oxandrolone}}

==Where to find medical care for Nephrotic syndrome?==
[http://maps.google.com/maps?rlz=1C1_____enUS444US444&q=nephrotic%20syndrome&safe=active&um=1&ie=UTF-8&sa=N&hl=en&tab=wl Directions to Hospitals Treating Nephrotic syndrome]

==Prevention==
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==What to expect (Outlook/Prognosis)?==
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
==Possible complications==
*[[Atherosclerosis]] and related heart diseases
*[[Renal vein thrombosis]]
*[[Acute kidney failure]]
*[[Chronic kidney disease]]
*Infections, including pneumococcal pneumonia
*[[Malnutrition]]
*Fluid overload, congestive heart failure, pulmonary edema

{{WH}}
{{WS}}

[[Category:Patient information]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

Nephrotic syndrome (patient information)

2017-01-12T19:38:47Z

Olufunmilola: /* What causes Nephrotic syndrome? */

__NOTOC__
'''For the WikiDoc page for this topic, click [[Nephrotic syndrome|here]]'''

{{Nephrotic syndrome (patient information)}}

'''Editors-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]]{{APM}} '''Associate Editor-In-Chief''': {{OO}} [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]

==Overview==

Nephrotic syndrome is a group of signs and symptoms of a kidney filtering system disease (glomerular disease) which is associated with massive loss of protein in urine (called proteinuria), body or leg swelling (called edema), excessive or abnormal blood clotting (hypercoagulability) and high blood fat content (called hyperlipidemia). The massive loss of protein in the urine results in low level of essential proteins (primarily the essential protein called albumin) in blood leading to hypoalbuminemia.

Key Words and their meanings
*Hyper: excess
*Hypo: little or low level
*Albumin: essential amino acid.
*Amino Acids: building blocks for proteins
*Lipids: Fat
*Glomeruli: basic unit of the kidney filtration system

Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

==What are the symptoms of Nephrotic syndrome?==
Swelling is the most common symptom. It may occur:

*In the face and around the eyes.
*In the arms and legs, especially in the feet and ankles
*In the belly area (swollen abdomen)
Other symptoms include:

*Foamy appearance of the urine
*Weight gain (unintentional) from fluid retention
*Poor appetite
*High blood pressure

'''Patients that experience any of the above signs and symptoms should see their primary care physician immediately.'''

==What causes Nephrotic syndrome?==

Causes of Nephrotic Syndrome can be primary (that is; from the kidney glomeruli) or secondary (from an external insult to the kidney).
Primary cause of Nephrotic Syndrome is often referred to as Idiopathic i.e. there are no known causes for the kidney abnormality. Such is the case in Minimal change kidney disease seen in children.
Primary causes of nephrotic syndrome are usually described by the [[histology]], e.g. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). These diseases are considered to be "[[diagnosis of exclusion|diagnoses of exclusion]]," meaning they are diagnosed only after secondary causes have been excluded.

A complete list of possible causes of nephrotic syndrome can be found [[Nephrotic syndrome causes|here]].

==Diagnosis==
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:

*Creatine - blood test
*Blood urea nitrogen (BUN)
*Creatinine clearance
*Albumin blood test - may be low
*Urinalysis - reveals large amounts of urine protein
*Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.

Kidney biopsy may be needed.

Tests to rule out various causes may include the following:

*[[Glucose tolerance test]]
*[[Antinuclear antibody]]
*[[Rheumatoid factor]]
*[[Cryoglobulins]]
*Complement levels
*Hepatitis B and C antibodies
*[[VDRL]] serology
*Serum protein electrophoresis
This disease may also alter the results of the following tests:

*Urinary casts
*Protein electrophoresis - urine
*Serum iron

==When to seek urgent medical care?==
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.

Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.

Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

==Treatment options==
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.

Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.

Corticosteroids and other drugs that suppress or quiet the immune system may be used.

High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.

A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.

Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.

Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.

Blood thinners may be required to treat or prevent clot formation.

====Medications to avoid====

{{MedCondContrPI

|MedCond = nephrosis|Oxandrolone}}

==Where to find medical care for Nephrotic syndrome?==
[http://maps.google.com/maps?rlz=1C1_____enUS444US444&q=nephrotic%20syndrome&safe=active&um=1&ie=UTF-8&sa=N&hl=en&tab=wl Directions to Hospitals Treating Nephrotic syndrome]

==Prevention==
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==What to expect (Outlook/Prognosis)?==
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
==Possible complications==
*[[Atherosclerosis]] and related heart diseases
*[[Renal vein thrombosis]]
*[[Acute kidney failure]]
*[[Chronic kidney disease]]
*Infections, including pneumococcal pneumonia
*[[Malnutrition]]
*Fluid overload, congestive heart failure, pulmonary edema

{{WH}}
{{WS}}

[[Category:Patient information]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

Nephrotic syndrome (patient information)

2017-01-12T19:36:26Z

Olufunmilola: /* What are the symptoms of Nephrotic syndrome? */

__NOTOC__
'''For the WikiDoc page for this topic, click [[Nephrotic syndrome|here]]'''

{{Nephrotic syndrome (patient information)}}

'''Editors-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]]{{APM}} '''Associate Editor-In-Chief''': {{OO}} [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]

==Overview==

Nephrotic syndrome is a group of signs and symptoms of a kidney filtering system disease (glomerular disease) which is associated with massive loss of protein in urine (called proteinuria), body or leg swelling (called edema), excessive or abnormal blood clotting (hypercoagulability) and high blood fat content (called hyperlipidemia). The massive loss of protein in the urine results in low level of essential proteins (primarily the essential protein called albumin) in blood leading to hypoalbuminemia.

Key Words and their meanings
*Hyper: excess
*Hypo: little or low level
*Albumin: essential amino acid.
*Amino Acids: building blocks for proteins
*Lipids: Fat
*Glomeruli: basic unit of the kidney filtration system

Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

==What are the symptoms of Nephrotic syndrome?==
Swelling is the most common symptom. It may occur:

*In the face and around the eyes.
*In the arms and legs, especially in the feet and ankles
*In the belly area (swollen abdomen)
Other symptoms include:

*Foamy appearance of the urine
*Weight gain (unintentional) from fluid retention
*Poor appetite
*High blood pressure

'''Patients that experience any of the above signs and symptoms should see their primary care physician immediately.'''

==What causes Nephrotic syndrome?==
Primary causes of nephrotic syndrome are usually described by the [[histology]], e.g. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). These diseases are considered to be "[[diagnosis of exclusion|diagnoses of exclusion]]," meaning they are diagnosed only after secondary causes have been excluded.

A complete list of possible causes of nephrotic syndrome can be found [[Nephrotic syndrome causes|here]].

==Diagnosis==
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:

*Creatine - blood test
*Blood urea nitrogen (BUN)
*Creatinine clearance
*Albumin blood test - may be low
*Urinalysis - reveals large amounts of urine protein
*Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.

Kidney biopsy may be needed.

Tests to rule out various causes may include the following:

*[[Glucose tolerance test]]
*[[Antinuclear antibody]]
*[[Rheumatoid factor]]
*[[Cryoglobulins]]
*Complement levels
*Hepatitis B and C antibodies
*[[VDRL]] serology
*Serum protein electrophoresis
This disease may also alter the results of the following tests:

*Urinary casts
*Protein electrophoresis - urine
*Serum iron

==When to seek urgent medical care?==
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.

Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.

Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

==Treatment options==
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.

Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.

Corticosteroids and other drugs that suppress or quiet the immune system may be used.

High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.

A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.

Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.

Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.

Blood thinners may be required to treat or prevent clot formation.

====Medications to avoid====

{{MedCondContrPI

|MedCond = nephrosis|Oxandrolone}}

==Where to find medical care for Nephrotic syndrome?==
[http://maps.google.com/maps?rlz=1C1_____enUS444US444&q=nephrotic%20syndrome&safe=active&um=1&ie=UTF-8&sa=N&hl=en&tab=wl Directions to Hospitals Treating Nephrotic syndrome]

==Prevention==
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==What to expect (Outlook/Prognosis)?==
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
==Possible complications==
*[[Atherosclerosis]] and related heart diseases
*[[Renal vein thrombosis]]
*[[Acute kidney failure]]
*[[Chronic kidney disease]]
*Infections, including pneumococcal pneumonia
*[[Malnutrition]]
*Fluid overload, congestive heart failure, pulmonary edema

{{WH}}
{{WS}}

[[Category:Patient information]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

Nephrotic syndrome (patient information)

2017-01-12T19:34:57Z

Olufunmilola: /* Overview */

__NOTOC__
'''For the WikiDoc page for this topic, click [[Nephrotic syndrome|here]]'''

{{Nephrotic syndrome (patient information)}}

'''Editors-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]]{{APM}} '''Associate Editor-In-Chief''': {{OO}} [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]

==Overview==

Nephrotic syndrome is a group of signs and symptoms of a kidney filtering system disease (glomerular disease) which is associated with massive loss of protein in urine (called proteinuria), body or leg swelling (called edema), excessive or abnormal blood clotting (hypercoagulability) and high blood fat content (called hyperlipidemia). The massive loss of protein in the urine results in low level of essential proteins (primarily the essential protein called albumin) in blood leading to hypoalbuminemia.

Key Words and their meanings
*Hyper: excess
*Hypo: little or low level
*Albumin: essential amino acid.
*Amino Acids: building blocks for proteins
*Lipids: Fat
*Glomeruli: basic unit of the kidney filtration system

Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

==What are the symptoms of Nephrotic syndrome?==
Swelling is the most common symptom. It may occur:

*In the face and around the eyes.
*In the arms and legs, especially in the feet and ankles
*In the belly area (swollen abdomen)
Other symptoms include:

*Foamy appearance of the urine
*Weight gain (unintentional) from fluid retention
*Poor appetite
*High blood pressure

==What causes Nephrotic syndrome?==
Primary causes of nephrotic syndrome are usually described by the [[histology]], e.g. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). These diseases are considered to be "[[diagnosis of exclusion|diagnoses of exclusion]]," meaning they are diagnosed only after secondary causes have been excluded.

A complete list of possible causes of nephrotic syndrome can be found [[Nephrotic syndrome causes|here]].

==Diagnosis==
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:

*Creatine - blood test
*Blood urea nitrogen (BUN)
*Creatinine clearance
*Albumin blood test - may be low
*Urinalysis - reveals large amounts of urine protein
*Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.

Kidney biopsy may be needed.

Tests to rule out various causes may include the following:

*[[Glucose tolerance test]]
*[[Antinuclear antibody]]
*[[Rheumatoid factor]]
*[[Cryoglobulins]]
*Complement levels
*Hepatitis B and C antibodies
*[[VDRL]] serology
*Serum protein electrophoresis
This disease may also alter the results of the following tests:

*Urinary casts
*Protein electrophoresis - urine
*Serum iron

==When to seek urgent medical care?==
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.

Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.

Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

==Treatment options==
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.

Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.

Corticosteroids and other drugs that suppress or quiet the immune system may be used.

High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.

A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.

Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.

Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.

Blood thinners may be required to treat or prevent clot formation.

====Medications to avoid====

{{MedCondContrPI

|MedCond = nephrosis|Oxandrolone}}

==Where to find medical care for Nephrotic syndrome?==
[http://maps.google.com/maps?rlz=1C1_____enUS444US444&q=nephrotic%20syndrome&safe=active&um=1&ie=UTF-8&sa=N&hl=en&tab=wl Directions to Hospitals Treating Nephrotic syndrome]

==Prevention==
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==What to expect (Outlook/Prognosis)?==
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
==Possible complications==
*[[Atherosclerosis]] and related heart diseases
*[[Renal vein thrombosis]]
*[[Acute kidney failure]]
*[[Chronic kidney disease]]
*Infections, including pneumococcal pneumonia
*[[Malnutrition]]
*Fluid overload, congestive heart failure, pulmonary edema

{{WH}}
{{WS}}

[[Category:Patient information]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

Nephrotic syndrome (patient information)

2017-01-12T19:31:35Z

Olufunmilola:

__NOTOC__
'''For the WikiDoc page for this topic, click [[Nephrotic syndrome|here]]'''

{{Nephrotic syndrome (patient information)}}

'''Editors-in-Chief:''' [[C. Michael Gibson, M.S., M.D.]]{{APM}} '''Associate Editor-In-Chief''': {{OO}} [[User:Ujjwal Rastogi|Ujjwal Rastogi, MBBS]] [mailto:urastogi@perfuse.org]

==Overview==
Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.
==What are the symptoms of Nephrotic syndrome?==
Swelling is the most common symptom. It may occur:

*In the face and around the eyes.
*In the arms and legs, especially in the feet and ankles
*In the belly area (swollen abdomen)
Other symptoms include:

*Foamy appearance of the urine
*Weight gain (unintentional) from fluid retention
*Poor appetite
*High blood pressure

==What causes Nephrotic syndrome?==
Primary causes of nephrotic syndrome are usually described by the [[histology]], e.g. [[minimal change disease]] (MCD), [[focal segmental glomerulosclerosis]] (FSGS) and [[membranous nephropathy]] (MN). These diseases are considered to be "[[diagnosis of exclusion|diagnoses of exclusion]]," meaning they are diagnosed only after secondary causes have been excluded.

A complete list of possible causes of nephrotic syndrome can be found [[Nephrotic syndrome causes|here]].

==Diagnosis==
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:

*Creatine - blood test
*Blood urea nitrogen (BUN)
*Creatinine clearance
*Albumin blood test - may be low
*Urinalysis - reveals large amounts of urine protein
*Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.

Kidney biopsy may be needed.

Tests to rule out various causes may include the following:

*[[Glucose tolerance test]]
*[[Antinuclear antibody]]
*[[Rheumatoid factor]]
*[[Cryoglobulins]]
*Complement levels
*Hepatitis B and C antibodies
*[[VDRL]] serology
*Serum protein electrophoresis
This disease may also alter the results of the following tests:

*Urinary casts
*Protein electrophoresis - urine
*Serum iron

==When to seek urgent medical care?==
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.

Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.

Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.

==Treatment options==
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.

Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.

Corticosteroids and other drugs that suppress or quiet the immune system may be used.

High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.

A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.

Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.

Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.

Blood thinners may be required to treat or prevent clot formation.

====Medications to avoid====

{{MedCondContrPI

|MedCond = nephrosis|Oxandrolone}}

==Where to find medical care for Nephrotic syndrome?==
[http://maps.google.com/maps?rlz=1C1_____enUS444US444&q=nephrotic%20syndrome&safe=active&um=1&ie=UTF-8&sa=N&hl=en&tab=wl Directions to Hospitals Treating Nephrotic syndrome]

==Prevention==
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome.

==What to expect (Outlook/Prognosis)?==
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
==Possible complications==
*[[Atherosclerosis]] and related heart diseases
*[[Renal vein thrombosis]]
*[[Acute kidney failure]]
*[[Chronic kidney disease]]
*Infections, including pneumococcal pneumonia
*[[Malnutrition]]
*Fluid overload, congestive heart failure, pulmonary edema

{{WH}}
{{WS}}

[[Category:Patient information]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]
[[Category:Pediatrics]]
[[Category:Disease]]
[[Category:Syndromes]]
[[Category:Primary care]]

IgA nephropathy causes

2017-01-12T19:06:17Z

Olufunmilola:

__NOTOC__
{{IgA nephropathy}}
{{CMG}}{{APM}} {{AE}} {{OO}}

==Overview==

==Causes==
===Primary IgA Nephropathy===
The cause of primary IgA nephropathy is unknown. Additionally there are no known infectious or environmental associated factors. However, IgA nephropathy is associated with some genetic mutations and familial clustering as a postulated cause of primary IgAN <ref name="pmid10792601">{{cite journal| author=Hsu SI, Ramirez SB, Winn MP, Bonventre JV, Owen WF| title=Evidence for genetic factors in the development and progression of IgA nephropathy. | journal=Kidney Int | year= 2000 | volume= 57 | issue= 5 | pages= 1818-35 | pmid=10792601 | doi=10.1046/j.1523-1755.2000.00032.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10792601 }} </ref>. These genetic associations are poorly studied and are largely inconclusive. There are ongoing studies in this field.
Primary IgA nephropathy is caused by a genetic predisposition in patients who typically present immediately following a respiratory or gastrointestinal tract infections. The environmental exposure to these infectious agents may thus facilitate the subsequent pathogenesis of the disease.

===Secondary IgA Nephropathy===
The most common etiologies associated with glomerular IgA deposits and thus secondary IgA nephropathy include:
* Liver cirrhosis<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 |volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc=|url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>

* Celiac disease<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 |volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc=|url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>

* HIV infection<ref name="pmid22895519">{{cite journal| author=Radhakrishnan J, Cattran DC| title=The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient. | journal=Kidney Int | year= 2012 |volume= 82 | issue= 8 | pages= 840-56 | pmid=22895519 | doi=10.1038/ki.2012.280 | pmc=|url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22895519 }} </ref>

==References==
{{reflist|2}}
{{WH}}
{{WS}}

[[Category:Nephrology]]
[[Category:Genetic disorders]]

[[Category:Needs content]]

Diabetic nephropathy overview

2017-01-09T17:17:32Z

Olufunmilola: /* Overview */

__NOTOC__
{{Diabetic nephropathy}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Diabetic kidney disease (Diabetic Nephropathy) is the most common cause of chronic kidney disease and end stage renal disease (ESRD) in the United States <ref name="pmid27389078">{{cite journal| author=John S| title=Complication in diabetic nephropathy. | journal=Diabetes Metab Syndr | year= 2016 | volume= 10 | issue= 4 | pages= 247-249 | pmid=27389078 | doi=10.1016/j.dsx.2016.06.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27389078 }} </ref> . Due to the ongoing world wide increase in the incidence of Diabetes Mellitus, Diabetic Nephropathy (DN) is increasingly a major cause of ESRD disease world wide <ref name="pmid25249672">{{cite journal| author=Tuttle KR, Bakris GL, Bilous RW, Chiang JL, de Boer IH, Goldstein-Fuchs J et al.| title=Diabetic kidney disease: a report from an ADA Consensus Conference. | journal=Diabetes Care | year= 2014 | volume= 37 | issue= 10 | pages= 2864-83 | pmid=25249672 | doi=10.2337/dc14-1296 | pmc=4170131 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25249672 }} </ref>.

Diabetic Nephropathy affects male and female patients equally. The incidence of DN in African-Americans (blacks), Native Americans and people of Mexican origins is greater than in white Americans <ref name="pmid25957005">{{cite journal| author=Baudy A, Batuman V| title=Non-diabetic renal disease in diabetic patients: How to identify? When to biopsy? | journal=J Diabetes Complications | year= 2015 | volume= 29 | issue= 5 | pages= 613-4 | pmid=25957005 | doi=10.1016/j.jdiacomp.2015.04.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25957005 }} </ref>.
Currently, the main goal in the treatment of diabetic nephropathy is to slow the progression of chronic kidney disease. This is achieved by excellent control of hyperglycemia, dyslipidemia, and blood pressure. Antiproteinuric therapy through Renin-Angiotensin-Aldosterone System Inhibitors is considered to be a major pillar of the treatment <ref name="pmid26569322">{{cite journal| author=Lozano-Maneiro L, Puente-García A| title=Renin-Angiotensin-Aldosterone System Blockade in Diabetic Nephropathy. Present Evidences. | journal=J Clin Med | year= 2015 | volume= 4 | issue= 11 | pages= 1908-37 | pmid=26569322 | doi=10.3390/jcm4111908 | pmc=4663476 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26569322 }} </ref>. Renin-Angiotensin-Aldosterone System inhibition it thought to be beneficial in the early stages of diabetic nephropathy through decreasing proteinuria and progression <ref name="pmid8416309">{{cite journal| author=Kasiske BL, Kalil RS, Ma JZ, Liao M, Keane WF| title=Effect of antihypertensive therapy on the kidney in patients with diabetes: a meta-regression analysis. | journal=Ann Intern Med | year= 1993 | volume= 118 | issue= 2 | pages= 129-38 | pmid=8416309 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8416309 }} </ref>. Therefore, early diagnosis and institution of prompt treatment is very important in the management of Diabetes Nephropathy. Also, the role of diabetes prevention becomes paramount patients at high risk (e.g. metabolic syndrome, impaired glucose tolerance).

Diabetic nephropathy (DN) is characterized by the presence of proteinuria or decreased renal function in patients with diabetes mellitus<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref> however, diabetic nephropathy can also present in form of non-proteinuric decline in GFR. Nonetheless, proteinuria remains the hallmark of diagnosis for diabetic nephropathy, despite emerging trends suggestive of non proteinuric diabetic nephropathy <ref name="pmid16801579">{{cite journal| author=MacIsaac RJ, Panagiotopoulos S, McNeil KJ, Smith TJ, Tsalamandris C, Hao H et al.| title=Is nonalbuminuric renal insufficiency in type 2 diabetes related to an increase in intrarenal vascular disease? | journal=Diabetes Care | year= 2006 | volume= 29 | issue= 7 | pages= 1560-6 | pmid=16801579 | doi=10.2337/dc05-1788 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16801579 }} </ref>.
===Early Diabetic Nephropathy===
The range of proteinuria in early DN is shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Males: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 30-300 mg/g
*Females: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 20-200 mg/g
===Overt Diabetic Nephropathy===
Overt DN is defined according to the presence of proteinuria or according to renal function.
The following ranges in overt DN are shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Proteinuria > 500 mg/24 hrs or albuminuria > 300 mg/24 hrs.
*Estimated glomerular filtration rate (eGFR) < 60 ml/min/1.73m2

==Historical Perspective==

==Classification==

==Pathophysiology==
Diabetic nephropathy is a serious complication in patients with long standing Type 1 or Type 2 Diabetes Mellitus. It usually occurs in about 10 to 15years following the onset of diabetes mellitus. Poor glycemic control, dyslipidemia, smoking, and environmental and genetic factors play important roles in the development of diabetic nephropathy.

==Causes==

==Differentiating Diabetic nephropathy from other Diseases==
Also called Kimmelstiel-Wilson syndrome, or Nodular diabetic glomerulosclerosis and inter-capillary glomerulonephritis;

==Epidemiology and Demographics==

==Risk Factors==

==Screening==

==Natural History, Complications and Prognosis==

==Diagnosis==

===History and Symptoms===

===Physical Examination===

===Laboratory Findings===
Microalbuminuria, as defined by an urinary albumin-to-creatinine ratio of >30mg/g is an early diagnostic clue to diabetic nephropathy. Some patients may go on to develop high-grade nephrotic range proteinuria, while others may develop diabetic nephropathy without any measurable albuminuria.

===Other Diagnostic Studies===

==Treatment==

===Medical Therapy===

===Primary Prevention===

===Secondary Prevention===

===Cost-Effectiveness of Therapy===

===Future or Investigational Therapies===

==Case Studies==

===Case #1===

==References==

{{Reflist|2}}

{{WH}}
{{WS}}

[[Category:Needs content]]
[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

Diabetic nephropathy overview

2017-01-09T17:11:44Z

Olufunmilola: /* Overview */

__NOTOC__
{{Diabetic nephropathy}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Diabetic kidney disease (Diabetic Nephropathy) is the most common cause of chronic kidney disease and end stage renal disease (ESRD) in the United States <ref name="pmid27389078">{{cite journal| author=John S| title=Complication in diabetic nephropathy. | journal=Diabetes Metab Syndr | year= 2016 | volume= 10 | issue= 4 | pages= 247-249 | pmid=27389078 | doi=10.1016/j.dsx.2016.06.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27389078 }} </ref> . Due to the ongoing world wide increase in the incidence of Diabetes Mellitus, Diabetic Nephropathy (DN) is increasingly a major cause of ESRD disease world wide <ref name="pmid25249672">{{cite journal| author=Tuttle KR, Bakris GL, Bilous RW, Chiang JL, de Boer IH, Goldstein-Fuchs J et al.| title=Diabetic kidney disease: a report from an ADA Consensus Conference. | journal=Diabetes Care | year= 2014 | volume= 37 | issue= 10 | pages= 2864-83 | pmid=25249672 | doi=10.2337/dc14-1296 | pmc=4170131 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25249672 }} </ref>.

Diabetic Nephropathy affects male and female patients equally. The incidence of DN in African-Americans (blacks), Native Americans and people of Mexican origins is greater than in white Americans <ref name="pmid25957005">{{cite journal| author=Baudy A, Batuman V| title=Non-diabetic renal disease in diabetic patients: How to identify? When to biopsy? | journal=J Diabetes Complications | year= 2015 | volume= 29 | issue= 5 | pages= 613-4 | pmid=25957005 | doi=10.1016/j.jdiacomp.2015.04.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25957005 }} </ref>.
Currently, the main goal in the treatment of diabetic nephropathy is to slow the progression of chronic kidney disease. This is achieved by excellent control of hyperglycemia, dyslipidemia, and blood pressure. Antiproteinuric therapy through Renin-Angiotensin-Aldosterone System Inhibitors is considered to be a major pillar of the treatment <ref name="pmid26569322">{{cite journal| author=Lozano-Maneiro L, Puente-García A| title=Renin-Angiotensin-Aldosterone System Blockade in Diabetic Nephropathy. Present Evidences. | journal=J Clin Med | year= 2015 | volume= 4 | issue= 11 | pages= 1908-37 | pmid=26569322 | doi=10.3390/jcm4111908 | pmc=4663476 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26569322 }} </ref>. Renin-Angiotensin-Aldosterone System inhibition it thought to be beneficial in the early stages of diabetic nephropathy through decreasing proteinuria and progression <ref name="pmid8416309">{{cite journal| author=Kasiske BL, Kalil RS, Ma JZ, Liao M, Keane WF| title=Effect of antihypertensive therapy on the kidney in patients with diabetes: a meta-regression analysis. | journal=Ann Intern Med | year= 1993 | volume= 118 | issue= 2 | pages= 129-38 | pmid=8416309 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8416309 }} </ref>. Therefore, early diagnosis and institution of prompt treatment is very important in the management of Diabetes Nephropathy. Also, the role of diabetes prevention becomes paramount patients at high risk (e.g. metabolic syndrome, impaired glucose tolerance).

Diabetic nephropathy (DN) is characterized by the presence of proteinuria or decreased renal function in patients with diabetes mellitus.<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref> However, diabetic nephropathy can also present in form of non-proteinuric decline in GFR, and although proteinuria remains the hallmark of diagnosis for diabetic nephropathy, there are emerging trends suggestive of non proteinuric diabetic nephropathy <ref name="pmid16801579">{{cite journal| author=MacIsaac RJ, Panagiotopoulos S, McNeil KJ, Smith TJ, Tsalamandris C, Hao H et al.| title=Is nonalbuminuric renal insufficiency in type 2 diabetes related to an increase in intrarenal vascular disease? | journal=Diabetes Care | year= 2006 | volume= 29 | issue= 7 | pages= 1560-6 | pmid=16801579 | doi=10.2337/dc05-1788 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16801579 }} </ref>.
===Early Diabetic Nephropathy===
The range of proteinuria in early DN is shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Males: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 30-300 mg/g
*Females: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 20-200 mg/g
===Overt Diabetic Nephropathy===
Overt DN is defined according to the presence of proteinuria or according to renal function.
The following ranges in overt DN are shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Proteinuria > 500 mg/24 hrs or albuminuria > 300 mg/24 hrs.
*Estimated glomerular filtration rate (eGFR) < 60 ml/min/1.73m2

==Historical Perspective==

==Classification==

==Pathophysiology==
Diabetic nephropathy is a serious complication in patients with long standing Type 1 or Type 2 Diabetes Mellitus. It usually occurs in about 10 to 15years following the onset of diabetes mellitus. Poor glycemic control, dyslipidemia, smoking, and environmental and genetic factors play important roles in the development of diabetic nephropathy.

==Causes==

==Differentiating Diabetic nephropathy from other Diseases==
Also called Kimmelstiel-Wilson syndrome, or Nodular diabetic glomerulosclerosis and inter-capillary glomerulonephritis;

==Epidemiology and Demographics==

==Risk Factors==

==Screening==

==Natural History, Complications and Prognosis==

==Diagnosis==

===History and Symptoms===

===Physical Examination===

===Laboratory Findings===
Microalbuminuria, as defined by an urinary albumin-to-creatinine ratio of >30mg/g is an early diagnostic clue to diabetic nephropathy. Some patients may go on to develop high-grade nephrotic range proteinuria, while others may develop diabetic nephropathy without any measurable albuminuria.

===Other Diagnostic Studies===

==Treatment==

===Medical Therapy===

===Primary Prevention===

===Secondary Prevention===

===Cost-Effectiveness of Therapy===

===Future or Investigational Therapies===

==Case Studies==

===Case #1===

==References==

{{Reflist|2}}

{{WH}}
{{WS}}

[[Category:Needs content]]
[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

Diabetic nephropathy overview

2017-01-09T17:07:23Z

Olufunmilola: /* Overview */

__NOTOC__
{{Diabetic nephropathy}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Diabetic kidney disease (Diabetic Nephropathy) is the most common cause of chronic kidney disease and end stage renal disease (ESRD) in the United States <ref name="pmid27389078">{{cite journal| author=John S| title=Complication in diabetic nephropathy. | journal=Diabetes Metab Syndr | year= 2016 | volume= 10 | issue= 4 | pages= 247-249 | pmid=27389078 | doi=10.1016/j.dsx.2016.06.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27389078 }} </ref> . Due to the ongoing world wide increase in the incidence of Diabetes Mellitus, Diabetic Nephropathy (DN) is increasingly a major cause of ESRD disease world wide <ref name="pmid25249672">{{cite journal| author=Tuttle KR, Bakris GL, Bilous RW, Chiang JL, de Boer IH, Goldstein-Fuchs J et al.| title=Diabetic kidney disease: a report from an ADA Consensus Conference. | journal=Diabetes Care | year= 2014 | volume= 37 | issue= 10 | pages= 2864-83 | pmid=25249672 | doi=10.2337/dc14-1296 | pmc=4170131 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25249672 }} </ref>.

Diabetic Nephropathy affects male and female patients equally. The incidence of DN in African-Americans (blacks), Native Americans and people of Mexican origins is greater than in white Americans <ref name="pmid25957005">{{cite journal| author=Baudy A, Batuman V| title=Non-diabetic renal disease in diabetic patients: How to identify? When to biopsy? | journal=J Diabetes Complications | year= 2015 | volume= 29 | issue= 5 | pages= 613-4 | pmid=25957005 | doi=10.1016/j.jdiacomp.2015.04.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25957005 }} </ref>.
Currently, the main goal in the treatment of diabetic nephropathy is to slow the progression of chronic kidney disease. This is achieved by excellent control of hyperglycemia, dyslipidemia, and blood pressure. Antiproteinuric therapy through Renin-Angiotensin-Aldosterone System Inhibitors is considered to be a major pillar of the treatment <ref name="pmid26569322">{{cite journal| author=Lozano-Maneiro L, Puente-García A| title=Renin-Angiotensin-Aldosterone System Blockade in Diabetic Nephropathy. Present Evidences. | journal=J Clin Med | year= 2015 | volume= 4 | issue= 11 | pages= 1908-37 | pmid=26569322 | doi=10.3390/jcm4111908 | pmc=4663476 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26569322 }} </ref>. Renin-Angiotensin-Aldosterone System inhibition it thought to be beneficial in the early stages of diabetic nephropathy through decreasing proteinuria and progression <ref name="pmid8416309">{{cite journal| author=Kasiske BL, Kalil RS, Ma JZ, Liao M, Keane WF| title=Effect of antihypertensive therapy on the kidney in patients with diabetes: a meta-regression analysis. | journal=Ann Intern Med | year= 1993 | volume= 118 | issue= 2 | pages= 129-38 | pmid=8416309 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8416309 }} </ref>. Therefore, early diagnosis and institution of prompt treatment is very important in the management of Diabetes Nephropathy. Also, the role of diabetes prevention becomes paramount patients at high risk (e.g. metabolic syndrome, impaired glucose tolerance).

Diabetic nephropathy (DN) is characterized by the presence of proteinuria or decreased renal function in patients with diabetes mellitus.<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref> However, diabetic nephropathy can also present in form of non-proteinuric decline in GFR, and proteinuria is not a criteria for diabetic nephropathy <ref name="pmid16801579">{{cite journal| author=MacIsaac RJ, Panagiotopoulos S, McNeil KJ, Smith TJ, Tsalamandris C, Hao H et al.| title=Is nonalbuminuric renal insufficiency in type 2 diabetes related to an increase in intrarenal vascular disease? | journal=Diabetes Care | year= 2006 | volume= 29 | issue= 7 | pages= 1560-6 | pmid=16801579 | doi=10.2337/dc05-1788 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16801579 }} </ref>.
===Early Diabetic Nephropathy===
The range of proteinuria in early DN is shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Males: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 30-300 mg/g
*Females: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 20-200 mg/g
===Overt Diabetic Nephropathy===
Overt DN is defined according to the presence of proteinuria or according to renal function.
The following ranges in overt DN are shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Proteinuria > 500 mg/24 hrs or albuminuria > 300 mg/24 hrs.
*Estimated glomerular filtration rate (eGFR) < 60 ml/min/1.73m2

==Historical Perspective==

==Classification==

==Pathophysiology==
Diabetic nephropathy is a serious complication in patients with long standing Type 1 or Type 2 Diabetes Mellitus. It usually occurs in about 10 to 15years following the onset of diabetes mellitus. Poor glycemic control, dyslipidemia, smoking, and environmental and genetic factors play important roles in the development of diabetic nephropathy.

==Causes==

==Differentiating Diabetic nephropathy from other Diseases==
Also called Kimmelstiel-Wilson syndrome, or Nodular diabetic glomerulosclerosis and inter-capillary glomerulonephritis;

==Epidemiology and Demographics==

==Risk Factors==

==Screening==

==Natural History, Complications and Prognosis==

==Diagnosis==

===History and Symptoms===

===Physical Examination===

===Laboratory Findings===
Microalbuminuria, as defined by an urinary albumin-to-creatinine ratio of >30mg/g is an early diagnostic clue to diabetic nephropathy. Some patients may go on to develop high-grade nephrotic range proteinuria, while others may develop diabetic nephropathy without any measurable albuminuria.

===Other Diagnostic Studies===

==Treatment==

===Medical Therapy===

===Primary Prevention===

===Secondary Prevention===

===Cost-Effectiveness of Therapy===

===Future or Investigational Therapies===

==Case Studies==

===Case #1===

==References==

{{Reflist|2}}

{{WH}}
{{WS}}

[[Category:Needs content]]
[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

Diabetic nephropathy overview

2017-01-09T15:56:47Z

Olufunmilola: /* Overview */

__NOTOC__
{{Diabetic nephropathy}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Diabetic kidney disease (Diabetic Nephropathy) is the most common cause of chronic kidney disease and end stage renal disease (ESRD) in the United States <ref name="pmid27389078">{{cite journal| author=John S| title=Complication in diabetic nephropathy. | journal=Diabetes Metab Syndr | year= 2016 | volume= 10 | issue= 4 | pages= 247-249 | pmid=27389078 | doi=10.1016/j.dsx.2016.06.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27389078 }} </ref> . Due to the ongoing world wide increase in the incidence of Diabetes Mellitus, Diabetic Nephropathy (DN) is increasingly a major cause of ESRD disease world wide <ref name="pmid25249672">{{cite journal| author=Tuttle KR, Bakris GL, Bilous RW, Chiang JL, de Boer IH, Goldstein-Fuchs J et al.| title=Diabetic kidney disease: a report from an ADA Consensus Conference. | journal=Diabetes Care | year= 2014 | volume= 37 | issue= 10 | pages= 2864-83 | pmid=25249672 | doi=10.2337/dc14-1296 | pmc=4170131 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25249672 }} </ref>.

Diabetic Nephropathy affects male and female patients equally. The incidence of DN in African-Americans (blacks), Native Americans and people of Mexican origins is greater than in white Americans <ref name="pmid25957005">{{cite journal| author=Baudy A, Batuman V| title=Non-diabetic renal disease in diabetic patients: How to identify? When to biopsy? | journal=J Diabetes Complications | year= 2015 | volume= 29 | issue= 5 | pages= 613-4 | pmid=25957005 | doi=10.1016/j.jdiacomp.2015.04.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25957005 }} </ref>.
Currently, the main goal in the treatment of diabetic nephropathy is to slow the progression of chronic kidney disease. This is achieved by excellent control of hyperglycemia, dyslipidemia, and blood pressure. Antiproteinuric therapy through Renin-Angiotensin-Aldosterone System Inhibitors is considered to be a major pillar of the treatment <ref name="pmid26569322">{{cite journal| author=Lozano-Maneiro L, Puente-García A| title=Renin-Angiotensin-Aldosterone System Blockade in Diabetic Nephropathy. Present Evidences. | journal=J Clin Med | year= 2015 | volume= 4 | issue= 11 | pages= 1908-37 | pmid=26569322 | doi=10.3390/jcm4111908 | pmc=4663476 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26569322 }} </ref>. Renin-Angiotensin-Aldosterone System inhibition it thought to be beneficial in the early stages of diabetic nephropathy through decreasing proteinuria and progression <ref name="pmid8416309">{{cite journal| author=Kasiske BL, Kalil RS, Ma JZ, Liao M, Keane WF| title=Effect of antihypertensive therapy on the kidney in patients with diabetes: a meta-regression analysis. | journal=Ann Intern Med | year= 1993 | volume= 118 | issue= 2 | pages= 129-38 | pmid=8416309 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8416309 }} </ref>. Therefore, early diagnosis and institution of prompt treatment is very important in the management of Diabetes Nephropathy. Also, the role of diabetes prevention becomes paramount patients at high risk (e.g. metabolic syndrome, impaired glucose tolerance).

Diabetic nephropathy (DN) is characterized by the presence of proteinuria or decreased renal function in patients with diabetes mellitus.<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref> However, diabetic nephropathy can also present in form of non-proteinuric decline in GFR, and proteinuria is not a criteria for diabetic nephropathy (reference).
===Early Diabetic Nephropathy===
The range of proteinuria in early DN is shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Males: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 30-300 mg/g
*Females: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 20-200 mg/g
===Overt Diabetic Nephropathy===
Overt DN is defined according to the presence of proteinuria or according to renal function.
The following ranges in overt DN are shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Proteinuria > 500 mg/24 hrs or albuminuria > 300 mg/24 hrs.
*Estimated glomerular filtration rate (eGFR) < 60 ml/min/1.73m2

==Historical Perspective==

==Classification==

==Pathophysiology==
Diabetic nephropathy is a serious complication in patients with long standing Type 1 or Type 2 Diabetes Mellitus. It usually occurs in about 10 to 15years following the onset of diabetes mellitus. Poor glycemic control, dyslipidemia, smoking, and environmental and genetic factors play important roles in the development of diabetic nephropathy.

==Causes==

==Differentiating Diabetic nephropathy from other Diseases==
Also called Kimmelstiel-Wilson syndrome, or Nodular diabetic glomerulosclerosis and inter-capillary glomerulonephritis;

==Epidemiology and Demographics==

==Risk Factors==

==Screening==

==Natural History, Complications and Prognosis==

==Diagnosis==

===History and Symptoms===

===Physical Examination===

===Laboratory Findings===
Microalbuminuria, as defined by an urinary albumin-to-creatinine ratio of >30mg/g is an early diagnostic clue to diabetic nephropathy. Some patients may go on to develop high-grade nephrotic range proteinuria, while others may develop diabetic nephropathy without any measurable albuminuria.

===Other Diagnostic Studies===

==Treatment==

===Medical Therapy===

===Primary Prevention===

===Secondary Prevention===

===Cost-Effectiveness of Therapy===

===Future or Investigational Therapies===

==Case Studies==

===Case #1===

==References==

{{Reflist|2}}

{{WH}}
{{WS}}

[[Category:Needs content]]
[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

Diabetic nephropathy overview

2017-01-09T15:40:41Z

Olufunmilola: /* Overview */

__NOTOC__
{{Diabetic nephropathy}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Diabetic kidney disease (Diabetic Nephropathy) is the most common cause of chronic kidney disease and end stage renal disease (ESRD) in the United States <ref name="pmid27389078">{{cite journal| author=John S| title=Complication in diabetic nephropathy. | journal=Diabetes Metab Syndr | year= 2016 | volume= 10 | issue= 4 | pages= 247-249 | pmid=27389078 | doi=10.1016/j.dsx.2016.06.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27389078 }} </ref> . Due to the ongoing world wide increase in the incidence of Diabetes Mellitus, Diabetic Nephropathy (DN) is increasingly a major cause of ESRD disease world wide <ref name="pmid25249672">{{cite journal| author=Tuttle KR, Bakris GL, Bilous RW, Chiang JL, de Boer IH, Goldstein-Fuchs J et al.| title=Diabetic kidney disease: a report from an ADA Consensus Conference. | journal=Diabetes Care | year= 2014 | volume= 37 | issue= 10 | pages= 2864-83 | pmid=25249672 | doi=10.2337/dc14-1296 | pmc=4170131 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25249672 }} </ref>.

Diabetic Nephropathy affects male and female patients equally. The incidence of DN in African-Americans (blacks), Native Americans and people of Mexican origins is greater than in white Americans <ref name="pmid25957005">{{cite journal| author=Baudy A, Batuman V| title=Non-diabetic renal disease in diabetic patients: How to identify? When to biopsy? | journal=J Diabetes Complications | year= 2015 | volume= 29 | issue= 5 | pages= 613-4 | pmid=25957005 | doi=10.1016/j.jdiacomp.2015.04.015 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25957005 }} </ref>.
Currently, the main goal in the treatment of diabetic nephropathy is to slow the progression of chronic kidney disease. This is achieved by excellent control of hyperglycemia, dyslipidemia, and blood pressure. Antiproteinuric therapy through Renin-Angiotensin-Aldosterone System Inhibitors is considered to be a major pillar of the treatment <ref name="pmid26569322">{{cite journal| author=Lozano-Maneiro L, Puente-García A| title=Renin-Angiotensin-Aldosterone System Blockade in Diabetic Nephropathy. Present Evidences. | journal=J Clin Med | year= 2015 | volume= 4 | issue= 11 | pages= 1908-37 | pmid=26569322 | doi=10.3390/jcm4111908 | pmc=4663476 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26569322 }} </ref>. Renin-Angiotensin-Aldosterone System inhibition it thought to be beneficial in the early stages of diabetic nephropathy through decreasing proteinuria and progression (reference). Therefore, early diagnosis and institution of prompt treatment is very important in the management of Diabetes Nephropathy. Also, the role of diabetes prevention becomes paramount patients at high risk (e.g. metabolic syndrome, impaired glucose tolerance).

Diabetic nephropathy (DN) is characterized by the presence of proteinuria or decreased renal function in patients with diabetes mellitus.<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref> However, diabetic nephropathy can also present in form of non-proteinuric decline in GFR, and proteinuria is not a criteria for diabetic nephropathy (reference).
===Early Diabetic Nephropathy===
The range of proteinuria in early DN is shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Males: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 30-300 mg/g
*Females: Microalbuminuria in the range of 30-300 mg/24 hrs or a spot urinary albumin/creatinine ratio of 20-200 mg/g
===Overt Diabetic Nephropathy===
Overt DN is defined according to the presence of proteinuria or according to renal function.
The following ranges in overt DN are shown below<ref name="pmid6738599">{{cite journal| author=Mogensen CE, Christensen CK| title=Predicting diabetic nephropathy in insulin-dependent patients. | journal=N Engl J Med | year= 1984 | volume= 311 | issue= 2 | pages= 89-93 | pmid=6738599 | doi=10.1056/NEJM198407123110204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6738599 }} </ref><ref name="pmid6690964">{{cite journal| author=Mogensen CE| title=Microalbuminuria predicts clinical proteinuria and early mortality in maturity-onset diabetes. | journal=N Engl J Med | year= 1984 | volume= 310 | issue= 6 | pages= 356-60 | pmid=6690964 | doi=10.1056/NEJM198402093100605 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6690964 }} </ref><ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref>:
*Proteinuria > 500 mg/24 hrs or albuminuria > 300 mg/24 hrs.
*Estimated glomerular filtration rate (eGFR) < 60 ml/min/1.73m2

==Historical Perspective==

==Classification==

==Pathophysiology==
Diabetic nephropathy is a serious complication in patients with long standing Type 1 or Type 2 Diabetes Mellitus. It usually occurs in about 10 to 15years following the onset of diabetes mellitus. Poor glycemic control, dyslipidemia, smoking, and environmental and genetic factors play important roles in the development of diabetic nephropathy.

==Causes==

==Differentiating Diabetic nephropathy from other Diseases==
Also called Kimmelstiel-Wilson syndrome, or Nodular diabetic glomerulosclerosis and inter-capillary glomerulonephritis;

==Epidemiology and Demographics==

==Risk Factors==

==Screening==

==Natural History, Complications and Prognosis==

==Diagnosis==

===History and Symptoms===

===Physical Examination===

===Laboratory Findings===
Microalbuminuria, as defined by an urinary albumin-to-creatinine ratio of >30mg/g is an early diagnostic clue to diabetic nephropathy. Some patients may go on to develop high-grade nephrotic range proteinuria, while others may develop diabetic nephropathy without any measurable albuminuria.

===Other Diagnostic Studies===

==Treatment==

===Medical Therapy===

===Primary Prevention===

===Secondary Prevention===

===Cost-Effectiveness of Therapy===

===Future or Investigational Therapies===

==Case Studies==

===Case #1===

==References==

{{Reflist|2}}

{{WH}}
{{WS}}

[[Category:Needs content]]
[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

Focal segmental glomerulosclerosis medical therapy

2016-12-20T20:45:07Z

Olufunmilola:

__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Treatment of Idiopathic or primary FSGS is very difficult. Treatment should be tailored towards relieving symptoms and preventing disease progression. The use of loop diuretics to manage the edema and corticosteroids to suppress immune function has been successful in some.
However, secondary causes of FSGS are treated by treating the underlying causes. Highly Active Anti-Retroviral Therapy can be used in HIV patients with FSGS. Immunosuppressant like prednisone can be used in immune mediated FSGS. Loop diuretics like furosemide can be used to control the edema and proteinuria.
FSGS patients who progress to End Stage Kidney disease would need lifelong dialysis.

Randomized clinical trials have only addressed patients with primary FSGS and have nephrotic-range proteinuria. For those patients, the use of corticosteroids and immunosuppressive therapy followed by conservative therapy with ACE-I or ARBs is recommended, according to the 2012 KDIGO guidelines.<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref> For patients with relapse, guidelines for relapsing minimal change disease are recommended for patients with FSGS. Finally, patients with resistance to steroids are recommended to use cyclosporine, or a combination of mycophenolate mofetil and high dose dexamethasone for patients who cannot tolerate cyclosporine.

==Medical Therapy==

The use of medical therapy in focal segmental glomerulosclerosis (FSGS) is based on the Kidney Disease - Improve Global Outcomes (KDIGO) guidelines in 2012.<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>

===Initial Treatment<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>===
The use of both corticosteroids and immunosuppressive therapy is recommended for initial treatment in patients with idiopathic FSGS and who have nephrotic syndrome. For patients with secondary FSGS or non-nephrotic range proteinuria, there are currently no reliable clinical trials to support the use of steroids.<ref name="pmid18065791">{{cite journal| author=Gupta K, Iskandar SS, Daeihagh P, Ratliff HL, Bleyer AJ| title=Distribution of pathologic findings in individuals with nephrotic proteinuria according to serum albumin. | journal=Nephrol Dial Transplant | year= 2008 | volume= 23 | issue= 5 | pages= 1595-9 | pmid=18065791 | doi=10.1093/ndt/gfm833 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18065791 }} </ref>
====Corticosteroids====
Either prednisone or prednisolone may be used. Both have equivalent dosage and duration.
*''Dose'': Single dose of 1 mg/kg/d (maximum 80 mg/d) or alternate-day dose of 2 mg/kg (maximum 120 mg).
*''Duration'': Minimum 4 weeks - maximum 16 weeks, as tolerated, or until complete remission is achieved.

Steroids should be tapered slowly over a period of 6 months after achieving complete remission, ie. reduction in dose by 10 mg per 2 weeks down to 0.15 mg/kg/d, then every 2-4 weeks by 2.5 mg. Patients with uncontrolled diabetes mellitus, psychiatric conditions, and severe osteoporosis have a relative contraindication to the use of steroids and may benefit more from calcineurin inhibitors (CNI).

In patients with tip lesions, good renal function, and low degrees of proteinuria, the time for initiation of corticosteroids is controversial, and may occur following the use of renin-angiotensin-system (RAS) blockers, such as ACE-inhibitors or ARBs, to assess for possible spontaneous remission.

====Calcineurin Inhibitors (CNI)<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>====
For patients with relative contraindication to steroids, such as patients with uncontrolled diabetes mellitus, psychiatric conditions, or severe osteoporosis, the use of CNI might be more helpful.

===Conservative Treatment<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>===
Renin-angiotensin-system (RAS) blockade is also necessary for patients with proteinuria, including those with FSGS. The initiation of RAS blockade may take place following initial corticosteroid therapy in patients with nephrotic syndrome to assess for remission on steroids, which is especially implicated in patients with severe nephrotic syndrome.

===Treatment for Relapse<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>===
Treatment for relapse is based upon the guidelines for the management of relapse of minimal change disease in adults.

===Treatment for Steroid-Resistant FSGS===
====Cyclosporine<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>====
*''Dose'': 3-5 mg/kg/d in divided doses (initial target: 125-175 ng/ml)
*''Duration'': At least 4-6 months. Cyclosporine therapy is to be continued for at least 12 months only if there is partial or complete remission with use. For patients who have not achieved any remission by 6 months, discontinuing cyclosporine is recommended.

Slow tapering of cyclosporine should be achieved following the initial duration of use. Tapering should be a reduction in dose by 25% every 2 months. For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil and high-dose dexamethasone is recommended.

====Tacrolimus and Prednisone====
=====Tacrolimus=====
*''Dose'': 0.1-0.2 mg/kg/d in two divided doses (initial target 5-10 ng/ml)
If remission is achieved, follow cyclosporine recommendations.

=====Prednisone=====
*''Dose'': 0.15 mg/kg/d
*''Duration'': 4-6 months
Prednisone is to be tapered off over 4-8 weeks.

====Other====
For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil (MMF) and high-dose dexamethasone is recommended.<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>

==References==
{{Reflist|2}}

[[Category:Disease]]
[[Category:Kidney diseases]]
[[Category: Channelopathy]]
[[Category:Needs content]]
{{WH}}
{{WS}}

Focal segmental glomerulosclerosis medical therapy

2016-12-20T20:39:43Z

Olufunmilola:

__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Randomized clinical trials have only addressed patients with primary FSGS and have nephrotic-range proteinuria. For those patients, the use of corticosteroids and immunosuppressive therapy followed by conservative therapy with ACE-I or ARBs is recommended, according to the 2012 KDIGO guidelines.<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref> For patients with relapse, guidelines for relapsing minimal change disease are recommended for patients with FSGS. Finally, patients with resistance to steroids are recommended to use cyclosporine, or a combination of mycophenolate mofetil and high dose dexamethasone for patients who cannot tolerate cyclosporine.

==Medical Therapy==

The use of medical therapy in focal segmental glomerulosclerosis (FSGS) is based on the Kidney Disease - Improve Global Outcomes (KDIGO) guidelines in 2012.<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>

===Initial Treatment<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>===
The use of both corticosteroids and immunosuppressive therapy is recommended for initial treatment in patients with idiopathic FSGS and who have nephrotic syndrome. For patients with secondary FSGS or non-nephrotic range proteinuria, there are currently no reliable clinical trials to support the use of steroids.<ref name="pmid18065791">{{cite journal| author=Gupta K, Iskandar SS, Daeihagh P, Ratliff HL, Bleyer AJ| title=Distribution of pathologic findings in individuals with nephrotic proteinuria according to serum albumin. | journal=Nephrol Dial Transplant | year= 2008 | volume= 23 | issue= 5 | pages= 1595-9 | pmid=18065791 | doi=10.1093/ndt/gfm833 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18065791 }} </ref>
====Corticosteroids====
Either prednisone or prednisolone may be used. Both have equivalent dosage and duration.
*''Dose'': Single dose of 1 mg/kg/d (maximum 80 mg/d) or alternate-day dose of 2 mg/kg (maximum 120 mg).
*''Duration'': Minimum 4 weeks - maximum 16 weeks, as tolerated, or until complete remission is achieved.

Steroids should be tapered slowly over a period of 6 months after achieving complete remission, ie. reduction in dose by 10 mg per 2 weeks down to 0.15 mg/kg/d, then every 2-4 weeks by 2.5 mg. Patients with uncontrolled diabetes mellitus, psychiatric conditions, and severe osteoporosis have a relative contraindication to the use of steroids and may benefit more from calcineurin inhibitors (CNI).

In patients with tip lesions, good renal function, and low degrees of proteinuria, the time for initiation of corticosteroids is controversial, and may occur following the use of renin-angiotensin-system (RAS) blockers, such as ACE-inhibitors or ARBs, to assess for possible spontaneous remission.

====Calcineurin Inhibitors (CNI)<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>====
For patients with relative contraindication to steroids, such as patients with uncontrolled diabetes mellitus, psychiatric conditions, or severe osteoporosis, the use of CNI might be more helpful.

===Conservative Treatment<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>===
Renin-angiotensin-system (RAS) blockade is also necessary for patients with proteinuria, including those with FSGS. The initiation of RAS blockade may take place following initial corticosteroid therapy in patients with nephrotic syndrome to assess for remission on steroids, which is especially implicated in patients with severe nephrotic syndrome.

===Treatment for Relapse<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>===
Treatment for relapse is based upon the guidelines for the management of relapse of minimal change disease in adults.

===Treatment for Steroid-Resistant FSGS===
====Cyclosporine<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>====
*''Dose'': 3-5 mg/kg/d in divided doses (initial target: 125-175 ng/ml)
*''Duration'': At least 4-6 months. Cyclosporine therapy is to be continued for at least 12 months only if there is partial or complete remission with use. For patients who have not achieved any remission by 6 months, discontinuing cyclosporine is recommended.

Slow tapering of cyclosporine should be achieved following the initial duration of use. Tapering should be a reduction in dose by 25% every 2 months. For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil and high-dose dexamethasone is recommended.

====Tacrolimus and Prednisone====
=====Tacrolimus=====
*''Dose'': 0.1-0.2 mg/kg/d in two divided doses (initial target 5-10 ng/ml)
If remission is achieved, follow cyclosporine recommendations.

=====Prednisone=====
*''Dose'': 0.15 mg/kg/d
*''Duration'': 4-6 months
Prednisone is to be tapered off over 4-8 weeks.

====Other====
For patients who cannot tolerate cyclosporine, the use of a combination of mycophenolate mofetil (MMF) and high-dose dexamethasone is recommended.<ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>

==References==
{{Reflist|2}}

[[Category:Disease]]
[[Category:Kidney diseases]]
[[Category: Channelopathy]]
[[Category:Needs content]]
{{WH}}
{{WS}}

Focal segmental glomerulosclerosis natural history, complications and prognosis

2016-12-20T20:36:14Z

Olufunmilola:

__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
Several clinical and pathological factors are associated with poor renal outcome. The most important prognostic factor in FSGS is the patient's response to therapy. Proteinuria is an important predictor of spontaneous remission or progression of FSGS into ESRD. Generally, spontaneous remission is an unlikely event in FSGS; and more than 50% of patients with nephrotic-range proteinuria reach ESRD within 3-8 years. Other important prognostic factors include male gender, black race, high level of serum creatinine and collapsing variant on histopathological analysis.

==Complications==
Renal complications and associated cardiovascular morbidity are the most common and most feared complications of focal segmental glomerulosclerosis (FSGS). Unlike minimal change disease, spontaneous remission is very unlikely in FSGS. The degree of proteinuria correlates with the probability to spontaneous remission and progression of disease into chronic kidney disease and end-stage renal disease (ESRD).<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref>

==Natural History & Prognosis==
The most important poor prognostic factors in focal segmental glomerulosclerosis (FSGS) are<ref name="FSGS">{{cite web |url=http://cdn.intechopen.com/pdfs/22820/InTech-Focal_segmental_glomerulosclerosis.pdf|title= Focal segmental glomerulosclerosis.|last1= Sohal |first1= DS |last2= Prabhakar |first2= SS|date=November 02, 2011|website= Interchopen |publisher= InTech |accessdate=3 December 2013}}</ref>:
*No adequate response to therapy
*Massive or nephrotic-range proteinuria
*Level of serum creatinine > 1.3 mg/dL
*Collapsing variant on morphological appearance
*Presence of tubulointerstitial fibrosis
*Black race

Inadequate response to therapy is considered the most important predictor of progression to ESRD.<ref name="pmid18813290">{{cite journal| author=Deegens JK, Dijkman HB, Borm GF, Steenbergen EJ, van den Berg JG, Weening JJ et al.| title=Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis. | journal=Kidney Int | year= 2008 | volume= 74 | issue= 12 | pages= 1568-76 | pmid=18813290 | doi=10.1038/ki.2008.413 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18813290 }} </ref> According to a 10-year study in 1999, approximately 50% of patients with heavy proteinuria in the nephrotic-range progress to end-stage renal disease (ESRD) within 3-8 years.<ref name="pmid10382985">{{cite journal| author=Korbet SM|title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. |journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 |pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref> In converse, patients with non-nephrotic range proteinuria often have a better prognosis, with a 10-year survival reaching > 80%.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref> While spontaneous remission is highly uncommon in FSGS, occurring in approximately 5-23% of patients, the degree of proteinuria may predict the probability of spontaneous remission.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref><ref name="pmid23871408">{{cite journal| author=Beck L, Bomback AS, Choi MJ, Holzman LB, Langford C, Mariani LH et al.| title=KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. | journal=Am J Kidney Dis | year= 2013 | volume= 62 | issue= 3 | pages= 403-41 | pmid=23871408 | doi=10.1053/j.ajkd.2013.06.002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23871408 }} </ref>

Serum creatinine > 1.3 mg/dL and interstitial fibrosis > 20% on pathological analysis have been associated with worse renal outcomes.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref> The extent of proteinuria, serum creatinine, and extent of fibrosis on pathological observation have been positively correlated.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref>

While collapsing variant, commonly seen in HIV-induced FSGS, is associated with poorer outcomes, tip variant correlates with better outcomes.<ref name="pmid16518352">{{cite journal| author=Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ et al.| title=Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. | journal=Kidney Int | year= 2006 | volume= 69 | issue= 5 | pages= 920-6 | pmid=16518352 | doi=10.1038/sj.ki.5000160 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16518352 }} </ref> According to Korbet and colleagues, the remission of proteinuria is negatively associated with progression to ESRD in FSGS.<ref name="pmid10382985">{{cite journal| author=Korbet SM| title=Clinical picture and outcome of primary focal segmental glomerulosclerosis. | journal=Nephrol Dial Transplant | year= 1999 | volume= 14 Suppl 3 | issue= | pages= 68-73 | pmid=10382985 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10382985 }} </ref>

==References==
{{Reflist|2}}

{{WH}}
{{WS}}

Focal segmental glomerulosclerosis historical perspective

2016-12-20T20:34:59Z

Olufunmilola:

__NOTOC__
{{Focal segmental glomerulosclerosis}}
{{CMG}}{{APM}} {{AE}}{{OO}}
==Overview==
==Historical Perspective==
It is believed that Theodor Fahr, a German pathologist, first was first to describe "progressive lipoid nephrosis" in 1925 and to draw an illustration of focal segmental glomerulosclerosis (FSGS).<ref>{{cite book | last = Fahr |first = T | authorlink = |coauthors = | title = Pathologische anatomie des morbus brightii. In: Fahr T, Gruber GB, Koch M, et al. eds. Harnorgane Männliche Geschlechtsorgane.| publisher = Springer |date = 1925 | location = Vienna| pages = 156-472 | url = | doi = | id = | isbn = }}</ref> At the time, he referred to it as "lipoid nephrosis with degeneration", showing a clear association to minimal change disease. FSGS was then described in 1957 by Dr. Arnold Rich, a pathologist at Johns Hopkins University.<ref name="pmid13426687">{{cite journal| author=RICH AR| title=A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis. | journal=Bull Johns Hopkins Hosp | year= 1957 | volume= 100 | issue= 4 | pages= 173-86 | pmid=13426687 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13426687 }} </ref> His original article "A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis" was originally published in "Bulletin of the Johns Hopkins Hospital".<ref name="pmid13426687">{{cite journal| author=RICH AR| title=A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis. | journal=Bull Johns Hopkins Hosp | year= 1957 | volume= 100 | issue= 4 | pages= 173-86 | pmid=13426687 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13426687 }} </ref> In his original description, he noted the presence of segmental sclerosis in juxtamedullary glomeruli of autopsies of uremic children with nephrosis.<ref name="pmid13426687">{{cite journal| author=RICH AR| title=A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis. | journal=Bull Johns Hopkins Hosp | year= 1957 | volume= 100 | issue= 4 | pages= 173-86 | pmid=13426687 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13426687 }} </ref> In 1970, Jacob Churg and colleagues finally classified FSGS as a unique renal glomerulopathy in the " Pathology of the Nephrotic Syndrome in Children: Report for the International International Study of Kidney Disease in Children" in view of its clinical and pathological features, and its steroid-resistance in comparison to minimal change disease.<ref name="pmid4193942">{{cite journal| author=Churg J, Habib R, White RH| title=Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. | journal=Lancet | year= 1970 | volume= 760 | issue= 1 | pages= 1299-302 | pmid=4193942 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4193942 }} </ref>

==References==
{{Reflist|2}}

{{WH}}
{{WS}}

Polyarteritis nodosa

2016-12-20T15:33:15Z

Olufunmilola:

'''For patient information, click [[Polyarteritis nodosa (patient information)|here]]'''

'''For the heart in Polyarteritis Nodosa click[[The Heart in Polyarteritis Nodosa| here]]'''

{{DiseaseDisorder infobox |
Name = Polyarteritis nodosa |
Image = PAN Grade 6 atherosclerosis.jpg|
Caption = Lung: Arteriosclerosis Grade 6: Micro med mag H&E; an excellent example of pulmonary polyarteritis nodosa. 4 yo male with primary pulmonary hypertension [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] |
}}
{{Polyarteritis nodosa}}

{{CMG}} {{APM}}; {{AE}}{{OO}} {{CZ}}; [[User:Haritha|Haritha Machavarapu, M.B.B.S.]]

{{SK}} PAN; [[Adolph Kussmaul|Kussmaul]] disease; Kussmaul-Meier disease; periarteritis nodosa

==[[Polyarteritis nodosa overview|Overview]]==

==[[Polyarteritis nodosa historical perspective|Historical Perspective]]==

==[[Polyarteritis nodosa classification|Classification]]==

==[[Polyarteritis nodosa pathophysiology|Pathophysiology]]==

==[[Polyarteritis nodosa causes|Causes]]==

==[[Differentiating Polyarteritis nodosa from other diseases|Differentiating Polyarteritis nodosa from other Diseases]]==

==[[Polyarteritis nodosa epidemiology and demographics|Epidemiology and Demographics]]==

==[[Polyarteritis nodosa risk factors|Risk Factors]]==

==[[Polyarteritis nodosa screening|Screening]]==

==[[Polyarteritis nodosa natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

== Diagnosis ==

[[Polyarteritis nodosa diagnostic criteria|Diagnostic Criteria]] | [[Polyarteritis nodosa history and symptoms| History and Symptoms]] | [[Polyarteritis nodosa physical examination | Physical Examination]] | [[Polyarteritis nodosa laboratory findings | Laboratory Findings]] | [[Polyarteritis nodosa electrocardiography | Electrocardiography]] | [[Polyarteritis nodosa chest x ray | Chest X Ray]] | [[Polyarteritis nodosa CT|CT]] | [[Polyarteritis nodosa MRI|MRI]] | [[Polyarteritis nodosa other diagnostic studies|Other Diagnostic Studies]] | [[Polyarteritis nodosa other imaging findings|Other Imaging Findings]]

==Treatment==
[[Polyarteritis nodosa medical therapy|Medical Therapy]] | [[Polyarteritis nodosa surgery|Surgery]] | [[Polyarteritis nodosa primary prevention|Primary Prevention]] | [[Polyarteritis nodosa secondary prevention|Secondary Prevention]] |[[Polyarteritis nodosa cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | |[[Polyarteritis nodosa future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Polyarteritis nodosa case study one|Case #1]]

[[Category:Rheumatology]]
[[Category:Nephrology]]

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

The WikiDoc Living Textbook of Kidney and Urologic Diseases

2016-12-20T15:32:12Z

Olufunmilola:

__NOTOC__
'''Editor-In-Chief Nephrology:''' {{APM}} {{AE}} {{OO}}
----
==[[:Category:Kidney diseases | Click ''Here'' For A Complete Listing Of All Chapters In The Living Textbook Of Kidney And Urologic Disease In Alphabetical Order]]==

== KIDNEY EMBRYOLOGY ==
[[Pronephros]] | [[Mesonephros]] | [[Metanephros]] | [[Ureteric bud]] | [[Nephron]] | [[Collecting duct system|Collecting system]] | [[Vasculature]]

== KIDNEY ANATOMY ==

=== A. Nephron ===
'''i) [[Glomerulus]]:''' [[Podocytes]] | [[Endothelial cells]] | [[Mesangial cells]] | [[Glomerular basement membrane]] | [[Bowman's capsule|Bowman capsule]]

'''ii) [[Juxtaglomerular apparatus|Juxtaglomerular Apparatus]]:''' Juxtaglomerular granular cells | Extraglomerular mesangium | [[Macula densa]]

'''iii) Proximal Tubule:''' Pars convoluta | Pars recta

'''iv) [[Loop of Henle]]:''' [[Descending limb of loop of Henle|Descending loop]] | [[Thick ascending limb of loop of Henle|Thick Ascending Limb]] | [[Thin ascending limb of loop of Henle|Thin Ascending Limb]]

==== v) [[Distal convoluted tubule|Distal Convoluted Tubule]] ====

==== vi) [[Connecting tubule|Connecting Tubule]] ====

=== '''B. Collecting Ducts''' ===
[[Cortical collecting duct|Cortical Collecting Duct]] | [[Outer medullary collecting duct|Outer Medullary Collecting Duct]] | [[Inner medullary collecting duct|Inner Medullary Collecting Duct]]

=== '''C. Interstitium''' ===
[[Medullary interstitium|Medullary Interstitium]] | Cortical Interstitium

=== '''D. Lymphatics''' ===

=== E. Vasculature ===

=== '''F. Innervation''' ===

== RENAL PHYSIOLOGY ==
'''A. [[Renal blood flow|Renal Blood Flow]]'''

'''B. Glomerular Ultrafiltration:''' Determinants of Glomerular Ultrafiltration | Regulation of Glomerular Ultrafiltration

'''C. Blood Pressure Regulation'''

'''D. Markers of Glomerular Function:''' [[Renal function|Renal Clearance]] | [[Inulin|Inulin clearance]] | [[Creatinine clearance]] | [[Cockroft-Gault formula|Cockroft-Gault Formula]] | [[Urea]] | [[Cystatin C]] | Other biomarkers

'''E. Markers of tubular function and Damage''': [[LDH]] | Kim-1

== KIDNEY PATHOLOGY ==

== PROCEDURES ==

==Anatomy and Physiology==
{{Kidney}}
{{Renal physiology}}
{{Reproductive physiology}}

==Embryology==
{{Development of urinary and reproductive systems}}

==Disease States==
{{Nephrology}}

==Congenital Disease States==
{{Congenital malformations of genital organs and urinary system}}

==Pharmacotherapy==
{{Urologicals}}

==Surgery==
{{Transurethral resection of the prostate}}

Diabetic nephropathy

2016-12-20T15:26:48Z

Olufunmilola:

__NOTOC__
{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = Nodular_glomerulosclerosis.jpeg |
Caption = Photomicrography of nodular glomerulosclerosis in Kimmelstein-Wilson syndrome. Source: CDC |
}}

'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''

{{Diabetic nephropathy}}

{{CMG}}{{APM}} {{AE}} {{OO}}

{{SK}}: Kimmelstiel-Wilson disease; diabetic glomerulosclerosis; nephropathy-diabetic; diabetic glomerulonephropathy

==[[Diabetic nephropathy overview|Overview]]==

==[[Diabetic nephropathy historical perspective|Historical Perspective]]==

==[[Diabetic nephropathy classification|Classification]]==

==[[Diabetic nephropathy pathophysiology|Pathophysiology]]==

==[[Diabetic nephropathy causes|Causes]]==

==[[Differentiating Diabetic nephropathy from other diseases|Differentiating Diabetic nephropathy from other Diseases]]==

==[[Diabetic nephropathy epidemiology and demographics|Epidemiology and Demographics]]==

==[[Diabetic nephropathy risk factors|Risk Factors]]==

==[[Diabetic nephropathy screening|Screening]]==

==[[Diabetic nephropathy natural history, complications and prognosis|Natural History, Complications and Prognosis]]==

==Diagnosis==
[[Diabetic nephropathy history and symptoms|History and Symptoms]] | [[Diabetic nephropathy physical examination|Physical Examination]] | [[Diabetic nephropathy laboratory findings|Laboratory Findings]] | [[Diabetic nephropathy electrocardiogram|Electrocardiogram]] | [[Hashiomoto's thyroiditis chest x ray|Chest X Ray]] | [[Diabetic nephropathy CT|CT]] | [[Diabetic nephropathy MRI|MRI]] | [[Diabetic nephropathy echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Diabetic nephropathy other imaging findings|Other Imaging Findings]] | [[Diabetic nephropathy other diagnostic studies|Other Diagnostic Studies]]

==Treatment==

[[Diabetic nephropathy medical therapy|Medical Therapy]] | [[Diabetic nephropathy surgery|Surgery]] | [[Diabetic nephropathy primary prevention|Primary Prevention]] | [[Diabetic nephropathy secondary prevention|Secondary Prevention]] | [[Diabetic nephropathy cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Diabetic nephropathy future or investigational therapies|Future or Investigational Therapies]]

==Case Studies==
[[Diabetic nephropathy case study one|Case #1]]

==External links==
* [http://www.nlm.nih.gov/medlineplus/ency/article/000494.htm Diabetic nephropathy]. MedlinePlus Medical Encyclopedia. Text from this public domain article was partially used here.

{{Nephrology}}
{{Endocrine pathology}}
{{Diabetes}}

[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

[[de:Diabetische Nephropathie]]
[[es:Nefropatía diabética]]
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[[sr:Дијабетесна нефропатија]]
[[fi:Diabeettinen nefropatia]]
[[sv:Diabetesnefropati]]
[[Category:Mature chapter]]

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Diabetic nephropathy epidemiology and demographics

2016-12-01T21:12:28Z

Olufunmilola: /* Overview */

__NOTOC__
{{Diabetic nephropathy}}
{{CMG}}{{APM}} {{AE}}{{OO}}

==Overview==
The incidence and prevalence of diabetes mellitus have grown significantly throughout the world, due primarily to the increase in the incidence of type 2 diabetes.

In the United States, prevalence of Diabetes Nephropathy had increased from 7.4% to 9.6% within a 10 years period (1988 to 2008), and this trend will likely continue due to the increasing incidence of diabetes in the American populace <ref name="pmid21693741">{{cite journal| author=de Boer IH, Rue TC, Hall YN, Heagerty PJ, Weiss NS, Himmelfarb J| title=Temporal trends in the prevalence of diabetic kidney disease in the United States. | journal=JAMA | year= 2011 | volume= 305 | issue= 24 | pages= 2532-9 | pmid=21693741 | doi=10.1001/jama.2011.861 | pmc=3731378 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21693741 }} </ref>. Studies by de Boer et al showed that DN accounts for 44% of new ESRD cases with 6% attributed to type 1 DM, 38% attributed to type 2 DM, and a projected increase of 3 million cases over the course of 20 years<ref name="pmid21693741">{{cite journal| author=de Boer IH, Rue TC, Hall YN, Heagerty PJ, Weiss NS, Himmelfarb J| title=Temporal trends in the prevalence of diabetic kidney disease in the United States. | journal=JAMA | year= 2011 | volume= 305 | issue= 24 | pages= 2532-9 | pmid=21693741 | doi=10.1001/jama.2011.861 | pmc=3731378 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21693741 }} </ref>. This increased incidence and prevalence of DN is notably greater among African Americans, Asians, and Native Americans than it is among Caucasians. Risk factors for DN include poor glycemic control, hypertension, tobacco use, and early age of onset of Diabetes Mellitus.

Diabetic nephropathy (DN) is the most common etiology of end-stage-renal-disease (ESRD) and most common indication for dialysis in the Western world, accounting for approximately 30-45% of all cases of ESRD. Blacks, Hispanics, and Native Americans are at increased risk of developing DN. Advanced age in type II diabetes and diagnosis at younger age in type I diabetes are significantly associated with DN. There seems to be a familial predisposition to DN.

==Epidemiology and Demographics==
Diabetic nephropathy is the most common etiology of nephropathy and dialysis in the Western world.<ref name="pmid21278716">{{cite journal| author=Gray SP, Cooper ME| title=Diabetic nephropathy in 2010: Alleviating the burden of diabetic nephropathy. | journal=Nat Rev Nephrol | year= 2011 | volume= 7 | issue= 2 | pages= 71-3 | pmid=21278716 | doi=10.1038/nrneph.2010.176 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21278716 }} </ref> It accounts for approximately 30-45% of all causes of end-stage renal disease (ESRD).<ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. | journal=Contrib Nephrol | year= 2011 | volume= 170 | issue= | pages= 1-7 | pmid=21659752 | doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref><ref name="pmid11792755">{{cite journal| author=Kurokawa K, Nangaku M, Saito A, Inagi R, Miyata T| title=Current issues and future perspectives of chronic renal failure. | journal=J Am Soc Nephrol | year= 2002 | volume= 13 Suppl 1 | issue= | pages= S3-6 | pmid=11792755 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11792755 }} </ref> The incidence of albuminuria is currently estimated to be as high as 0.83% annually.<ref name="pmid21659752">{{cite journal| author=Reutens AT, Atkins RC| title=Epidemiology of diabetic nephropathy. |journal=Contrib Nephrol | year= 2011 | volume= 170 |issue= | pages= 1-7 | pmid=21659752 |doi=10.1159/000324934 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21659752 }} </ref> Almost 39% of patients with diabetes mellitus have positive microalbuminuria.<ref name="pmid17118233">{{cite journal| author=Rossing P| title=Diabetic nephropathy: worldwide epidemic and effects of current treatment on natural history. | journal=Curr Diab Rep | year= 2006 | volume= 6 | issue= 6 | pages= 479-83 | pmid=17118233 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17118233 }} </ref> Unfortunately, the worldwide burden of diabetic nephropathy seems to be on the rise, with an incidence rising by 150% per one decade in USA, Europe, and Japan and a prevalence increasing from 6.4% in 2010 and estimated to reach 7.7% in 2030.<ref name="pmid19896746">{{cite journal| author=Shaw JE, Sicree RA, Zimmet PZ| title=Global estimates of the prevalence of diabetes for 2010 and 2030. | journal=Diabetes Res Clin Pract | year= 2010 | volume= 87 | issue= 1 | pages= 4-14 | pmid=19896746 | doi=10.1016/j.diabres.2009.10.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19896746 }} </ref><ref name="pmid7810523">{{cite journal| author=Mallick NP, Jones E, Selwood N| title=The European (European Dialysis and Transplantation Association-European Renal Association) Registry. | journal=Am J Kidney Dis | year= 1995 | volume= 25 | issue= 1 | pages= 176-87 | pmid=7810523 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7810523 }} </ref> Costs due to diabetic nephropathy reach as high as 30-40 billion USD annually in the USA only.<ref name="pmid22177944">{{cite journal| author=Collins AJ, Foley RN, Chavers B, Gilbertson D, Herzog C, Johansen K et al.| title='United States Renal Data System 2011 Annual Data Report: Atlas of chronic kidney disease & end-stage renal disease in the United States. | journal=Am J Kidney Dis | year= 2012 | volume= 59 | issue= 1 Suppl 1 | pages= A7, e1-420 | pmid=22177944 | doi=10.1053/j.ajkd.2011.11.015 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22177944 }} </ref><ref name="pmid11920337">{{cite journal| author=Trivedi HS, Pang MM, Campbell A, Saab P| title=Slowing the progression of chronic renal failure: economic benefits and patients' perspectives. | journal=Am J Kidney Dis | year= 2002 | volume= 39 | issue= 4 | pages= 721-9 | pmid=11920337 | doi=10.1053/ajkd.2002.31990 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11920337 }} </ref>

The burden of diabetic nephropathy from type II diabetes is far more significant than that of type I diabetes.<ref name="pmid11742409">{{cite journal| author=Zimmet P, Alberti KG, Shaw J| title=Global and societal implications of the diabetes epidemic. | journal=Nature | year= 2001 | volume= 414 | issue= 6865 | pages= 782-7 | pmid=11742409 | doi=10.1038/414782a | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11742409 }} </ref> Advanced age in type 2 diabetes and early diagnosis in type 1 diabetes are associated with higher risk of DN.<ref name="pmid9080995">{{cite journal| author=Gall MA, Hougaard P, Borch-Johnsen K, Parving HH| title=Risk factors for development of incipient and overt diabetic nephropathy in patients with non-insulin dependent diabetes mellitus: prospective, observational study. | journal=BMJ | year= 1997 | volume= 314 | issue= 7083 | pages= 783-8 | pmid=9080995 | doi= | pmc=PMC2126209 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9080995 }} </ref><ref name="pmid10332675">{{cite journal| author=Klein R, Klein BE, Moss SE, Cruickshanks KJ, Brazy PC| title=The 10-year incidence of renal insufficiency in people with type 1 diabetes. | journal=Diabetes Care | year= 1999 | volume= 22 | issue= 5 | pages= 743-51 | pmid=10332675 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10332675 }} </ref> Gender does not seem to play a role in the risk of DN. Blacks, Hispanics, and Native Americans are at increased incidence and severity of diabetic nephropathy and are more likely to suffer ESRD, even when adjusting for other associated risk factors, such as hypertension and socioeconomic status.

DN seems to have a familial predisposition. Diabetic siblings of patients with DN have an increased risk of DN up to 3-times as those of patients without DN.<ref name="pmid15586648">{{cite journal| author=Ayodele OE, Alebiosu CO, Salako BL| title=Diabetic nephropathy--a review of the natural history, burden, risk factors and treatment. | journal=J Natl Med Assoc | year= 2004 | volume= 96 | issue= 11 | pages= 1445-54 | pmid=15586648 | doi= | pmc=PMC2568593 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15586648 }} </ref> In one study evaluating familial predisposition of DN in Pima Indian families, the likelihood of developing proteinuria in patients with type 2 diabetes increased significantly when one or both parents have proteinuria.<ref name="pmid2401399">{{cite journal| author=Pettitt DJ, Saad MF, Bennett PH, Nelson RG, Knowler WC| title=Familial predisposition to renal disease in two generations of Pima Indians with type 2 (non-insulin-dependent) diabetes mellitus. | journal=Diabetologia | year= 1990 | volume= 33 | issue= 7 | pages= 438-43 | pmid=2401399 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2401399 }} </ref> Several genes have shown to have an important role in the development of DN:
*DD (homozygous deletion genotype) of the angiotensin converting enzyme (ACE) genotype<ref name="pmid9264004">{{cite journal| author=Jeffers BW, Estacio RO, Raynolds MV, Schrier RW| title=Angiotensin-converting enzyme gene polymorphism in non-insulin dependent diabetes mellitus and its relationship with diabetic nephropathy. | journal=Kidney Int | year= 1997 | volume= 52 | issue= 2 | pages= 473-7 | pmid=9264004 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9264004 }} </ref><ref name="pmid10023638">{{cite journal| author=Kuramoto N, Iizuka T, Ito H, Yagui K, Omura M, Nozaki O et al.| title=Effect of ACE gene on diabetic nephropathy in NIDDM patients with insulin resistance. | journal=Am J Kidney Dis | year= 1999 | volume= 33 | issue= 2 | pages= 276-81 | pmid=10023638 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10023638 }} </ref><ref name="pmid9727374">{{cite journal| author=Kunz R, Bork JP, Fritsche L, Ringel J, Sharma AM| title=Association between the angiotensin-converting enzyme-insertion/deletion polymorphism and diabetic nephropathy: a methodologic appraisal and systematic review. | journal=J Am Soc Nephrol | year= 1998 | volume= 9 | issue= 9 | pages= 1653-63 | pmid=9727374 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9727374 }} </ref>

*Homozygous Z-2 allelle of aldose reductase gene<ref name="pmid9709964">{{cite journal| author=Shah VO, Scavini M, Nikolic J, Sun Y, Vai S, Griffith JK et al.| title=Z-2 microsatellite allele is linked to increased expression of the aldose reductase gene in diabetic nephropathy. | journal=J Clin Endocrinol Metab | year= 1998 | volume= 83 | issue= 8 | pages= 2886-91 | pmid=9709964 | doi=10.1210/jcem.83.8.5028 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9709964 }} </ref>

*Beta subunit of G protein at position 825 allele<ref name="pmid10200987">{{cite journal| author=Blüthner M, Schmidt S, Siffert W, Knigge H, Nawroth P, Ritz E| title=Increased frequency of G-protein beta 3-subunit 825 T allele in dialyzed patients with type 2 diabetes. | journal=Kidney Int | year= 1999 | volume= 55 | issue= 4 | pages= 1247-50 | pmid=10200987 | doi=10.1046/j.1523-1755.1999.00399.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10200987 }} </ref>

==References==

{{Reflist|2}}

[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Nephrology]]

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