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'''Diabetes mellitus Main page'''
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Patient Information
: [[Diabetes mellitus type 1 (patient information)|Type 1]]
: [[Diabetes mellitus type 2 (patient information)|Type 2]]
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[[Diabetes mellitus#Overview|Overview]]
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[[Diabetes mellitus#Classification|Classification]]
: [[Diabetes mellitus type 1]]
: [[Diabetes mellitus type 2]]
: [[Gestational diabetes]]
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[[Diabetes mellitus#Differential diagnosis|Differential Diagnosis]]
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[[Diabetes mellitus#Complications|Complications]]
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[[Diabetes mellitus#Screening|Screening]]
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[[Diabetes mellitus#Diagnosis|Diagnosis]]
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[[Diabetes mellitus#Prevention|Prevention]]
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| {{#ev:youtube|https://https://www.youtube.com/watch?v=zucxZw069kw|350}}
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__NOTOC__
__NOTOC__
{{Congenital adrenal hyperplasia}}


{{CMG}}; {{AE}}{{MJ}}
{{CMG}}
{{Glomerulonephritis}}
 
==Pathophysiology==
===Microscopic Pathology===
 
[http://www.peir.net Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
 
<div align="left">
<gallery heights="175" widths="175">
image:Acute GN 1.jpg|Glomerulonephritis: Micro H&E med mag; an excellent example of AGN with many neutrophils
image:Acute GN 2.jpg|Acute Glomerulonephritis: Micro H&E high mag; an  excellent example of acute exudative glomerulonephritis.
</gallery>
</div>
 
<br>
 
===Glomerulonephritis Videos===
====Rapidly progressive glomerulonephritis====
 
{{#ev:youtube|CqSyj4cVZPE}}
 
 
====Chronic glomerulonephritis====
 
{{#ev:youtube|eA1vYarRAWo}}
 
===Images===
 
[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]
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 1.jpg|This is a low-power photomicrograph of a saggital section of end stage chronic glomerulonephritis (GN). Note the marked thinning of the cortex (arrow).
Image:Glomerulonephritis case 2.jpg|This is a higher-power photomicrograph of hyalinized glomeruli (arrows) and glomeruli with thick basement membranes.
</gallery>
</div>
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 3.jpg|This is a higher-power photomicrograph of hyalinized glomeruli (1) and glomeruli with thickened basement membranes (2).
Image:Glomerulonephritis case 4.jpg|This is a photomicrograph of interstitial and vascular lesions in end stage renal disease.
</gallery>
</div>
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 5.jpg|This is an immunofluorescent photomicrograph of granular membranous immunofluorescence (immune complex disease). The antibody used for these studies was specific for IgG.
Image:Glomerulonephritis case 6.jpg|This is an electron micrograph of subepithelial granular electron dense deposits (arrows) which correspond to the granular immunofluorescence seen in the previous image.
</gallery>
</div>


{{SK}} Congenital adrenal hyperplasia, CAH, Adrenal hyperplasia
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 7.jpg|This is a photomicrograph of a glomerulus from another case with acute poststreptococcal glomerulonephritis. In this case the immune complex glomerular disease is ongoing with necrosis and accumulation of neutrophils in the glomerulus.
Image:Glomerulonephritis case 8.jpg|This immunofluorescent photomicrograph of a glomerulus from a case of acute poststreptococcal glomerulonephritis shows a granular immunofluorescence pattern consistent with immune complex disease. The primary antibody used for this staining was specific for IgG; however antibodies for complement would show a similar pattern.
</gallery>
</div>


==Overview==
<div align="left">
Congenital adrenal hyperplasia (CAH) refers to any of several autosomal recessive conditions resulting from biochemical paths of the steroidogenesis of cortisol from cholesterol by the adrenal glands. Most of these conditions involve greater or lesser production of sex steroids and can alter development of primary or secondary sex characteristics in affected infants, children, and adults. Only a small minority of people with CAH can be said to have an intersex condition, but this attracted American public attention in the late 1990s and many accounts of varying accuracy have appeared in the popular media. Approximately 95% of cases of CAH are due to 21-hydroxylase deficiency.  
<gallery heights="175" widths="175">
Prenatal diagnosis can be made in both of these disorders by chorionic villous sampling, but this can only be done at 8-10 weeks. In order to prevent the deleterious effect of excess androgens on genital (and brain!) development, therapy must be started earlier. This is most often considered if there is an affected sibling. Treatment is dexamethasone, which is not degraded by the placenta, but is associated with significant maternal weight gain, hypertension, and edema.  
Image:Glomerulonephritis case 9.jpg|This electron micrograph demonstrates scattered subepithelial dense deposits (arrows) and a polymorphonuclear leukocyte in the lumen.  
Image:Glomerulonephritis case 10.jpg|For comparison this is an immunofluorescent photomicrograph of a glomerulus from a patient with Goodpasture's syndrome. The linear (arrows) immunofluorescence is characteristic of Goodpasture's syndrome.  
</gallery>
</div>
 
===Images:===
 
*[http://www.pathologyatlas.ro/Crescentic%20Glomerulonephritis.html Crescentic GN]
 
*[http://www.pathologyatlas.ro/Chronic%20Glomerulonephritis1.html Chronic GN]


==Historical Perspective==
==References==
* Congenital adrenal hyperplasia first time seen in 1865 by Luigi De Crecchio, an Italian [[pathologist]], in a man at autopsy, who had large [[adrenal glands]] and female [[internal organs]].
{{Reflist|2}}


* Important aspects of discovering [[adrenal]] hormones:<ref name="pmid25635623">{{cite journal |vauthors=Delle Piane L, Rinaudo PF, Miller WL |title=150 years of congenital adrenal hyperplasia: translation and commentary of De Crecchio's classic paper from 1865 |journal=Endocrinology |volume=156 |issue=4 |pages=1210–7 |year=2015 |pmid=25635623 |doi=10.1210/en.2014-1879 |url=}}</ref><ref name="ISBN:978-0323297387">{{cite book | last = Melmed | first = Shlomo | title = Williams textbook of endocrinology | publisher = Elsevier | location = Philadelphia, PA | year = 2016 | isbn = 978-0323297387 }}=</ref><ref name="pmid18118071">{{cite journal |vauthors=HENCH PS, KENDALL EC |title=The effect of a hormone of the adrenal cortex (17-hydroxy-11-dehydrocorticosterone; compound E) and of pituitary adrenocorticotropic hormone on rheumatoid arthritis |journal=Proc Staff Meet Mayo Clin |volume=24 |issue=8 |pages=181–97 |year=1949 |pmid=18118071 |doi= |url=}}</ref><ref name="pmid4288776">{{cite journal |vauthors=Biglieri EG, Herron MA, Brust N |title=17-hydroxylation deficiency in man |journal=J. Clin. Invest. |volume=45 |issue=12 |pages=1946–54 |year=1966 |pmid=4288776 |pmc=292880 |doi=10.1172/JCI105499 |url=}}</ref>
[[Category:Disease]]
** In 1563, Eustachius described the [[Adrenal|adrenals]] and then published by Lancisi in 1714.
[[Category:Organ disorders]]
** In 1849, [[Thomas Addison]], found on a bronzed appearance associated with the [[adrenal glands]] called [[melasma]] suprarenale while searching for the cause of [[pernicious anemia]].
[[Category:Inflammations]]
** In 1855, [[Thomas Addison]] defined the clinical features and [[autopsy]] findings in 11 cases of diseases of the [[suprarenal]] capsules, and half of them were [[tuberculous]] in origin.
[[Category:Kidney diseases]]
** In 1856, In [[adrenalectomy]] experiments, [[Brown-Sequard syndrome|Brown-Séquard]] found that the [[adrenal glands]] are nessesary for life.
** In 1896, [[William Osler]] prepared an oral glycerin extract derived from pig [[Adrenal|adrenals]] and showed that it had clinical benefit in patients with [[Addison disease]].
** In 1905, Bulloch and Sequeira described patients with [[congenital adrenal hyperplasia]].
** In 1936, Selye described the concept of stress and its effect on [[pituitary]]-[[adrenal]] function.
** In 1937-1952, Kendall and Reichstein, defined the isolation and structural characterization of [[Adrenocortical hormone|adrenocortical hormones]].
** In 1943, Li and colleagues isolated [[adrenocorticotropic hormone]] from sheep [[pituitary]].
** In 1950, Hench, Kendall, and Reichstein shared the [[Nobel Prize in Medicine]] for describing the [[anti-inflammatory]] effects of [[cortisone]] in patients with [[rheumatoid arthritis]]
** In 1956, Conn described [[primary aldosteronism]].
** In 1981, Vale defined characterization and synthesis of [[corticotropin-releasing hormone]].
** From 1980-present called the molecular era; highlights in this section are:
*** Cloning and functional characterization of [[Steroid hormone receptor|steroid hormone receptors]] discovered.
*** [[Steroidogenic]] [[enzymes]] described.
*** [[Adrenal]] [[transcription factors]] were reported.
*** [[Molecular]] basis for human adrenal diseases described.


==Pathophisiology==
[[Category:Needs overview]]


{{WH}}
{{WS}}
--------------------------------------------------
===Common Causes===
*[[Churg-strauss syndrome]]
*[[Cryoglobulinaemia]]
*[[Diabetes mellitus type 2]]
*[[Dibasic aminoaciduria type 2]]
*[[Endocarditis]]
*[[Glycogenosis type 1a]]
*[[Henoch-schönlein purpura ]]
*[[Hepatitis b]]
*[[Hereditary onycho-osteodysplasia]]
*[[Hypersensitivity vasculitis]]
*[[Iga nephropathy]]
*[[Lepromatous leprosy]]
*[[Mixed essential cryoglobulinaemia]]
*[[Myeloma]]
*[[Paraneoplastic syndrome]]
*[[Polyarteritis nodosa]]
*[[Radiotherapy]]
*[[Schimke immunoosseous dysplasia]]
*[[Secondary syphilis]]
*[[Serum sickness]]
*[[Sickle cell disease]]
*[[Systemic lupus erythematosus]]
*[[Vasculitis]]
*[[Wegener's granulomatosis]]
*[[Wiskott-aldrich syndrome]]




[[image:Adrenal Steroids.png|600px]]
--------------------------------------


__NOTOC__


==Gross Pathology==
{{Glomerulonephritis}}
Gross pathology findings in patients with congenital adrenal hyperplasia are:<ref name="radio">Congenital adrenal hyperplasia. Dr Henry Knipe and Dr M Venkatesh . Radiopaedia.org 2015.http://radiopaedia.org/articles/congenital-adrenal-hyperplasia</ref><ref name="pmid25372578">{{cite journal |vauthors=Teixeira SR, Elias PC, Andrade MT, Melo AF, Elias Junior J |title=The role of imaging in congenital adrenal hyperplasia |journal=Arq Bras Endocrinol Metabol |volume=58 |issue=7 |pages=701–8 |year=2014 |pmid=25372578 |doi= |url=}}</ref>
{{CMG}}; {{AE}}{{HK}}
*Enlarged adrenal glands
*Wrinkled surface adrenal glands
*Cerebriform pattern adrenal glands (pathognomonic sign)
*Normal ultrasound appearances may also be seen
*Testicular masses may be identified representing adrenal rest tissue


==Microscopic Pathology==
==Overview==
In congenital adrenal hyperplasia, hyperplastic cells are usually but not always smaller, with cytoplasm that can be vacuolated also often more basophilic. Rare mitotic figures may be present, but the hyperplastic cells typically lack features of cellular atypia.<ref name="urlAdrenal Gland - Hyperplasia - Nonneoplastic Lesion Atlas">{{cite web |url=https://ntp.niehs.nih.gov/nnl/endocrine/adrenal/hyperpl/index.htm |title=Adrenal Gland - Hyperplasia - Nonneoplastic Lesion Atlas |format= |work= |accessdate=}}</ref>
Glomerulonephritis may be proliferative or non-proliferative and may be associated with [[Nephrotic syndrome|nephrotic]] or [[Nephritic syndrome|nephritic]] features. The various types of glomerulonephritides should be differentiated from each other based on associations, presence of [[pitting edema]], hemeturia, [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital edema, [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]] and [[Electron microscopy|electron microscopic]] features.  
{|
|
[[Image:Cah mic.jpg|thumb|200px|frame|Adrenal gland, Cortex - Hyperplasia in a female rat from a chronic study. There is a hyperplastic lesion (H) in which cortical cells are increased in number but are smaller in size than adjacent normal cortical cells (NC)]]<ref name="urlAdrenal Gland - Hyperplasia - Nonneoplastic Lesion Atlas">{{cite web |url=https://ntp.niehs.nih.gov/nnl/endocrine/adrenal/hyperpl/index.htm |title=Adrenal Gland - Hyperplasia - Nonneoplastic Lesion Atlas |format= |work= |accessdate=}}</ref>
|
[[Image:Cah.jpg|thumb|250px|frame|Adrenal gland, Cortex - Hyperplasia in a male rat from a chronic study. There are two adjacent foci of hyperplasia (H) in the zona fasciculata.]]<ref name="urlAdrenal Gland - Hyperplasia - Nonneoplastic Lesion Atlas">{{cite web |url=https://ntp.niehs.nih.gov/nnl/endocrine/adrenal/hyperpl/index.htm |title=Adrenal Gland - Hyperplasia - Nonneoplastic Lesion Atlas |format= |work= |accessdate=}}</ref>
|}
==Classification==
Congenital adrenal hyperplasia is classified into seven types based on the genetic causes that lead to hyperplasia and hormonal imbalance.


{| align="center" class="wikitable" style="border: 0px; font-size: 90%; margin: 3px;"
==Differential Diagnosis==
! align="center" style="background:#DCDCDC;" rowspan="2" colspan="2" |Disease
The following table differentiates between various types of glomerulonephritides:
! align="center" style="background:#DCDCDC;" colspan="2" |History and symptoms
{| class="wikitable"
! align="center" style="background:#DCDCDC;" colspan="3" |Laboratory findings
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Glomerulonephritis
! align="center" style="background:#DCDCDC;" |Defective gene
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Sub-entity
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Causes and associations
! colspan="7" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History and Symtoms
! colspan="9" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
|-
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |ANCA
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Anti-glomerular basement membrane antibody (Anti-GBM antibody)
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Immune complex formation
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
|-
!Blood pressure
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
!Genitalia
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
!Increased
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia (pre-dominantly microscopic)
!Decreased
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
!K levels
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemoptysis
!
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Peri-orbital edema
|-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" rowspan="2" |[[21-hydroxylase deficiency]]
| rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Non-proliferative
|Classic type
!Minimal change disease
|
|
* Low in salt-wasting
* Idiopathic
 
* Protein tyrosine phosphatase receptor type O (glomerular epithelial protein 1- GLEPP1)
* Normal in non-salt-wasting
|
|
* Female: ambiguous
* Young children
 
* Recent infection and immunization
* Male: normal or scrotal pigmentation and large phallus 
* Atopy
* Hodgkin lymphoma
* Thrombosis (due to urinary loss of antithrombin-III)
|
|
* [[Deoxycorticosterone]]
+
* 11-Deoxy-[[cortisol]]
* [[17-Hydroxyprogesterone|17-hydroxyprogesterone]], mild elevation
|
|
* [[Cortisol]]
-
* [[Corticosterone]]
* [[Aldosterone]]
|
|
* High in salt wasting type
-
* Normal in non salt wasting
|
|
* CYP21A1 and CYP21A2 gene
-
|-
|Non-classic type
|
|
* Normal
+/-
|
|
* Female: virilization after puberty
-
* Male: normal appearance
|
|
* [[17-Hydroxyprogesterone|17-hydroxyprogesterone]]
+
* Exaggerated [[Androstenedione]], [[DHEA]], and [[17-Hydroxyprogesterone|17-hydroxyprogesterone]]
response to [[ACTH]]
|
|
+
|
|
* Normal
-
|
|
* CYP21A1 and CYP21A2 gene
-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" colspan="2" |[[17 alpha-hydroxylase deficiency|17-α hydroxylase deficiency]]
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Hypertension
|
|
* Female: normal
-
* Male: ambiguous
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Deoxycorticosterone]]
* [[Corticosterone]]
* [[Progesterone]]
|
|
* [[Cortisol]]
-
* [[Aldosterone]]
|
|
* Low
* Normal
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* CYP17A1
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" colspan="2" |[[11β-hydroxylase deficiency|11-β hydroxylase deficiency]]
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Hypertension
|
|
* Female: ambiguous
* Fusion of podocytes
 
* Male: normal or scrotal pigmentation and large phallus
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* [[Deoxycorticosterone]]
* 11-Deoxy-[[cortisol]]
* [[17-Hydroxyprogesterone|17-hydroxyprogesterone]], mild elevation
|
|
* [[Cortisol]]
-
* [[Corticosterone]]
|-
* [[Aldosterone]]
!Focal segmental glomerulosclerosis
|
|
* Low
* Idiopathic
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* HIV
* CYP11B1
* Heroine use
|-
* Sickle cell disease
| align="center" style="padding: 5px 5px; background: #DCDCDC;" colspan="2" |3β-Hydroxysteroid Dehydrogenase
* Interferon
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Severe obesity
* Mixed cryoglobunemia (Hepatitis C)
|
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Adults
* [[Dehydroepiandrosterone]]
|<nowiki>+</nowiki>
* [[17-hydroxypregnenolone]]
|<nowiki>-</nowiki>
* [[Pregnenolone]]
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
| +
| +
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
|
* [[Cortisol]]
* Focal (some glomeruli) and segmental (only part of glomerulus)
* [[Aldosterone]]
|
|
* High
* Effacement of podocytes
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
|<nowiki>-</nowiki>
|-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" colspan="2" |Cytochrome P450-oxidoreductase (POR) deficiency (ORD)
!Membranous glomerulonephritis
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
|
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Idiopathic
* Hepatitis B and C
* Solid tumors
* Systemic lupus erythmatosus
* Drugs (NSAIDS, penclliamine, gold, captopril)
|
|
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
| +
| +
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Thick glomerular basement membrance
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" colspan="2" |Congenital lipoid adrenal hyperplasia
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
|
|
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Sub-epithelial immune complex depositis with 'spike and dome' appearance
|<nowiki>-</nowiki>
|-
|-
| align="center" style="padding: 5px 5px; background: #DCDCDC;" colspan="2" |Cholesterol side-chain cleavage enzyme deficiency
| rowspan="7" align="center" style="background:#4479BA; color: #FFFFFF;" + |Proliferative
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
!IgA nephropathy
|
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
* Idiopathic
|
* Viral infections
|
| align="center" style="padding: 5px 5px; background: #F5F5F5;" |
|}
 
==Screening==
According to Endocrine Society Clinical Practice Guideline, screening for 21-hydroxylase deficiency by measuring 17a-hydroxyprogesterone is recommended for all newborns.
*Blood sample on filter paper should be obtained from heel puncture preferably between two and four days after birth.
*Screening programs should be done using a two-tier protocol (initial immunoassay with further evaluation of positive tests by liquid chromatography/tandem mass spectrometry.
*Most affected neonates have concentrations greater than 3500 ng/dL (105 nmol/L).<ref name="pmid2208708">{{cite journal |vauthors=Gonzalez RR, Mäentausta O, Solyom J, Vihko R |title=Direct solid-phase time-resolved fluoroimmunoassay of 17 alpha-hydroxyprogesterone in serum and dried blood spots on filter paper |journal=Clin. Chem. |volume=36 |issue=9 |pages=1667–72 |year=1990 |pmid=2208708 |doi= |url=}}</ref><ref name="pmid20823466">{{cite journal |vauthors=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, Meyer-Bahlburg HF, Miller WL, Montori VM, Oberfield SE, Ritzen M, White PC |title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline |journal=J. Clin. Endocrinol. Metab. |volume=95 |issue=9 |pages=4133–60 |year=2010 |pmid=20823466 |pmc=2936060 |doi=10.1210/jc.2009-2631 |url=}}</ref>
===Genetic counseling===
The Endocrine Society's Clinical Practice Guideline recommends that genetic counseling be provided for individuals who are planning to conceive, and there is a family history of 21-hydroxylase deficiency.<ref name="pmid20823466">{{cite journal |vauthors=Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, Meyer-Bahlburg HF, Miller WL, Montori VM, Oberfield SE, Ritzen M, White PC |title=Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline |journal=J. Clin. Endocrinol. Metab. |volume=95 |issue=9 |pages=4133–60 |year=2010 |pmid=20823466 |pmc=2936060 |doi=10.1210/jc.2009-2631 |url=}}</ref>
 
 
==Differentiating congenital adrenal hyperplasia from other diseases==
[[Congenital adrenal hyperplasia]] must be differentiated from diseases that cause [[ambiguous genitalia]]:<ref name="pmid17875484">{{cite journal |vauthors=Hughes IA, Nihoul-Fékété C, Thomas B, Cohen-Kettenis PT |title=Consequences of the ESPE/LWPES guidelines for diagnosis and treatment of disorders of sex development |journal=Best Pract. Res. Clin. Endocrinol. Metab. |volume=21 |issue=3 |pages=351–65 |year=2007 |pmid=17875484 |doi=10.1016/j.beem.2007.06.003 |url=}}</ref><ref name="pmid10857554">{{cite journal |vauthors=White PC, Speiser PW |title=Congenital adrenal hyperplasia due to 21-hydroxylase deficiency |journal=Endocr. Rev. |volume=21 |issue=3 |pages=245–91 |year=2000 |pmid=10857554 |doi=10.1210/edrv.21.3.0398 |url=}}</ref>
{| class="wikitable"
!Disease name
! colspan="2" |Laboratory tests
!Important clinical findings
|-
!
!Increased
!Decreased
!
|-
|[[21-hydroxylase deficiency|Classic type of 21-hydroxylase deficiency]]
|
|
* [[17-Hydroxyprogesterone|17-hydroxyprogesterone]]
* Young children
* [[Progesterone]]
* History of mucosal infections (e.g. gastroenteritis) and upper respiratory tract infection
* [[Androstenedione]]
* 2-3 days after infection (synpharyngitic)
* [[DHEA]]
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| -
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| +
|
|
* [[Aldosterone]]
* Crescent formation
* [[Corticosterone]] (salt-wasting)
* [[Cortisol]] (simple [[virilizing]])
|
|
* [[Ambiguous genitalia]] in female
* Mesangial proliferation
* [[Virilization]] in female
|<nowiki>-</nowiki>
* Salt-wasting
* [[Hypotension]] and [[hyperkalemia]]
|-
|-
|[[11β-hydroxylase deficiency|11-β hydroxylase deficiency]]
! rowspan="5" |Rapidly progressive glomerulonephritis
|
|
* [[Deoxycorticosterone]]
* Goodpasture syndrome
* 11-Deoxy-[[cortisol]]
* [[17-Hydroxyprogesterone|17-hydroxyprogesterone]], mild elevation
|
|
* [[Cortisol]]
* Young adults
* [[Corticosterone]]
|<nowiki>+/-</nowiki>
* [[Aldosterone]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
|
* [[Ambiguous genitalia]] in female
* Hypercellular and inflamed glomeruli (Crescent formation)
* [[Hypertension]] and [[hypokalemia]]
* [[Virilization]]
|-
|[[17 alpha-hydroxylase deficiency|17-α hydroxylase deficiency]]
|
* [[Deoxycorticosterone]]
* [[Corticosterone]]
* [[Progesterone]]
|
* [[Cortisol]]
* [[Aldosterone]]
|
|
* [[Ambiguous genitalia]] in male
*  Diffuse thickening of the glomerular basement membrane with absence of subepithelial and subendothelial deposits 
* [[Hypertension]]
|<nowiki>+ (Linear)</nowiki>
 
* [[Primary amenorrhea]]
 
* Absence of [[secondary sexual characteristics]]
 
* Minimal [[body hair]]
|-
|-
|3β-Hydroxysteroid Dehydrogenase
|
|
* [[Dehydroepiandrosterone]]
* Post infectious glomerulonephritis
* [[17-hydroxypregnenolone]]
* [[Pregnenolone]]
|
|
* [[Cortisol]]
* Streptococcal skin infections
* [[Aldosterone]]
* Streptococcal pharyngitis
* 2-3 weeks after infection
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
|
* [[Vomiting]], [[volume depletion]], [[hyponatremia]], and [[hyperkalemia]]
* Hypercellular and inflamed glomeruli
* 46-XY infants often show [[undervirilization]], due to a block in [[testosterone]] synthesis
|-
|Gestational [[hyperandrogenism]]
| colspan="2" |
* Maternal serum [[androgen]] concentrations (usually [[testosterone]] and [[androstenedione]]) are high
* If [[virilization]] is caused by exogenous hormone administration, the values may be low because the offending hormone is usually a synthetic [[steroid]] not measured in assays for [[testosterone]] or other [[androgens]]
|
|
* [[Androgen]] excess sign and symptoms in mother
* Sub-epithelial immune complex deposits
* History of [[androgen]] containing [[medication]]  consumption during [[pregnancy]] in mother
| + (Granular)
* [[Virilization]] in a 46,XX individual with normal female internal anatomy
* Causes include maternal [[luteoma]] or theca-[[lutein]] [[cysts]], and [[placental]] [[aromatase]] enzyme deficiency
|}
 
congenital adrenal hyperplasia must be differentiated from diseases that cause [[virilization]] and [[hirsutism]] in female:<ref name="pmid24830586">{{cite journal |vauthors=Hohl A, Ronsoni MF, Oliveira Md |title=Hirsutism: diagnosis and treatment |journal=Arq Bras Endocrinol Metabol |volume=58 |issue=2 |pages=97–107 |year=2014 |pmid=24830586 |doi= |url=}}</ref><ref name="pmid10857554">{{cite journal |vauthors=White PC, Speiser PW |title=Congenital adrenal hyperplasia due to 21-hydroxylase deficiency |journal=Endocr. Rev. |volume=21 |issue=3 |pages=245–91 |year=2000 |pmid=10857554 |doi=10.1210/edrv.21.3.0398 |url=}}</ref><ref name="ISBN:978-0323297387">{{cite book | last = Melmed | first = Shlomo | title = Williams textbook of endocrinology | publisher = Elsevier | location = Philadelphia, PA | year = 2016 | isbn = 978-0323297387 }}=</ref>
 
{| class="wikitable"
!Disease name
!Steroid status
!Other laboratory
!Important clinical findings
|-
|-
|Non-classic type of 21-hydroxylase deficiency
|Increased:
* [[17-Hydroxyprogesterone|17-hydroxyprogesterone]]
* Exaggerated [[Androstenedione]], [[DHEA]], and [[17-Hydroxyprogesterone|17-hydroxyprogesterone]]
response to [[ACTH]]
|
|
* Low [[testosterone]] levels
* Granulomatosis with polyangitis (Wegner's granulomatosis)
|
* No symptoms in infancy and male
 
* [[Virilization]] in females
|-
|[[11β-hydroxylase deficiency|11-β hydroxylase deficiency]]
|Increased:
* DOC
* 11-Deoxy-[[Cortisol]]
Decreased:
* [[Cortisol]]
* [[Corticosterone]]
* [[Aldosterone]]
|
|
* Low [[testosterone]] levels
* Necrotizing granulomas (Nasopharynx, lungs, kidneys)
* [[Conjunctivitis]]
* Ulceration of the [[cornea]]
* [[Episcleritis]]
* Peripheral neuropathy
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+ (C-ANCA)</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
|
* [[Hypertension]] and [[hypokalemia]]
* Hypercellular and inflamed glomeruli (Crescent formation)
* [[Virilization]]
|<nowiki>-  (pauci-immune)</nowiki>
|<nowiki>+/-</nowiki>
|-
|-
|3β-Hydroxysteroid Dehydrogenase
|Increased:
* [[DHEA]]
* [[17-hydroxypregnenolone]]
* [[Pregnenolone]]
Decreased:
* [[Cortisol]]
* [[Aldosterone]]
|
* Low [[testosterone]] levels
|
|
* Salt-wasting [[adrenal crisis]] in infancy
* Churg Strauss syndrome
 
* Mild [[virilization]] of genetically female infants
* [[Undervirilization]] of genetically male infants, making it the only form of [[CAH]] which can cause [[ambiguous genitalia]] in both genetic sexes.
|-
|[[Polycystic ovary syndrome ]]
|
|
* High [[DHEAS]] and [[androstenedione]] levels
* Necrotizing granulomas (Lungs and kidneys)
* Asthma
* Peripheral neuropathy
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
|
* Low [[testosterone]] levels
+ (C-ANCA)
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
|
* [[Polycystic ovaries]] in sonography
* Hypercellular and inflamed glomeruli (Crescent formation)
* [[Obesity]]
|<nowiki>- (pauci-immune)</nowiki>
* [[PCOS]] is the most common cause of [[hirsutism]] in women
|<nowiki>-</nowiki>
* No evidence another diagnosis
|-
|-
|[[Adrenal tumors]]
|
|
* Variable levels depends on [[tumor]] type
* Microscopic polyngitis
|
|
* Low [[testosterone]] level
* Necrotizing vasculitis (no granuloma)
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| +
|
|
* Older age
+ (P-ANCA)
* Rapidly progressive symptoms
|<nowiki>-</nowiki>
|-
|<nowiki>-</nowiki>
|Ovarian [[virilizing]] tumor
|
|
* Variable levels depends on [[tumor]] type
* Hypercellular and inflamed glomeruli (Crescent formation)
|
|<nowiki>- (pauci-immune)</nowiki>
* [[Testosterone]] is high
|<nowiki>-</nowiki>
|
* Older age
* Rapidly progressive symptoms
|-
|-
|[[Cushing's syndrome]]
!Membranoproliferative glomerulonephritis
|
|
* Increase [[cortisol]] & metabolites
* Idiopathic
* Variable other [[steroids]]
* Hepatitis B and C (Type 1)
* C3 nepritic factor (Type2)
|
|
* Variable [[mineralocorticoid]] excess
* Hemeturia
|
* Oliguria
* [[Cushingoid appearance]]
* Periorbital edema
|-
* Hypertension
|[[Hyperprolactinemia]]
|<nowiki>+/-</nowiki>
|
|<nowiki>+</nowiki>
* Normal levels of most of [[steroids]]
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
|
* Increased [[prolactin]]
* Thick glomerular basement membrane (Tram-track appearance)
|
|
* [[Infertility]], [[galactorrhea]]
* Mesangial proliferation and leukocyte infiltration
|<nowiki>+ (Granular)</nowiki>
|}
|}
==References==
==References==
{{reflist|2}}
{{Reflist|2}}
 
{{WH}}
{{WS}}
 
[[Category:Needs content]]
 
[[Category:Disease]]
[[Category:Organ disorders]]
[[Category:Inflammations]]
[[Category:Kidney diseases]]

Latest revision as of 06:42, 28 July 2020

Diabetes mellitus Main page

Patient Information

Type 1
Type 2

Overview

Classification

Diabetes mellitus type 1
Diabetes mellitus type 2
Gestational diabetes

Differential Diagnosis

Complications

Screening

Diagnosis

Prevention


https://https://www.youtube.com/watch?v=zucxZw069kw%7C350}}


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Glomerulonephritis Main page

Glomerulonephritis patient information

Overview

Classification

[[]]
[[]]
[[]]

Pathophysiology

Differential Diagnosis

Screening

Diagnosis

Prevention

Pathophysiology

Microscopic Pathology

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology


Glomerulonephritis Videos

Rapidly progressive glomerulonephritis

{{#ev:youtube|CqSyj4cVZPE}}


Chronic glomerulonephritis

{{#ev:youtube|eA1vYarRAWo}}

Images

Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Images:

References

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Common Causes




Glomerulonephritis Main page

Glomerulonephritis patient information

Overview

Classification

[[]]
[[]]
[[]]

Pathophysiology

Differential Diagnosis

Screening

Diagnosis

Prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [3]

Overview

Glomerulonephritis may be proliferative or non-proliferative and may be associated with nephrotic or nephritic features. The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.

Differential Diagnosis

The following table differentiates between various types of glomerulonephritides:

Glomerulonephritis Sub-entity Causes and associations History and Symtoms Laboratory Findings
Hyperlipidemia and hypercholesterolemia Nephrotic features Nephritic features ANCA Anti-glomerular basement membrane antibody (Anti-GBM antibody) Immune complex formation Light microscope Electron microscope Immunoflourescence pattern
History Pitting edema Hemeturia (pre-dominantly microscopic) Hypertension Hemoptysis Oliguria Peri-orbital edema
Non-proliferative Minimal change disease
  • Idiopathic
  • Protein tyrosine phosphatase receptor type O (glomerular epithelial protein 1- GLEPP1)
  • Young children
  • Recent infection and immunization
  • Atopy
  • Hodgkin lymphoma
  • Thrombosis (due to urinary loss of antithrombin-III)

+

-

-

-

+/-

-

+

+

-

-

-

-

  • Normal
  • Fusion of podocytes

-

Focal segmental glomerulosclerosis
  • Idiopathic
  • HIV
  • Heroine use
  • Sickle cell disease
  • Interferon
  • Severe obesity
  • Mixed cryoglobunemia (Hepatitis C)
  • Adults
+ - - - +/- - + + - - - -
  • Focal (some glomeruli) and segmental (only part of glomerulus)
  • Effacement of podocytes
-
Membranous glomerulonephritis
  • Idiopathic
  • Hepatitis B and C
  • Solid tumors
  • Systemic lupus erythmatosus
  • Drugs (NSAIDS, penclliamine, gold, captopril)
+ - - - +/- - + + - - - +
  • Thick glomerular basement membrance
  • Sub-epithelial immune complex depositis with 'spike and dome' appearance
-
Proliferative IgA nephropathy
  • Idiopathic
  • Viral infections
  • Young children
  • History of mucosal infections (e.g. gastroenteritis) and upper respiratory tract infection
  • 2-3 days after infection (synpharyngitic)
+/- + + - + +/- - - + - - +
  • Crescent formation
  • Mesangial proliferation
-
Rapidly progressive glomerulonephritis
  • Goodpasture syndrome
  • Young adults
+/- + + + + + - - + - + +
  • Hypercellular and inflamed glomeruli (Crescent formation)
  •  Diffuse thickening of the glomerular basement membrane with absence of subepithelial and subendothelial deposits 
+ (Linear)
  • Post infectious glomerulonephritis
  • Streptococcal skin infections
  • Streptococcal pharyngitis
  • 2-3 weeks after infection
+/- + + + + + - - + - - +
  • Hypercellular and inflamed glomeruli
  • Sub-epithelial immune complex deposits
+ (Granular)
  • Granulomatosis with polyangitis (Wegner's granulomatosis)
+/- + + + + + - - + + (C-ANCA) - -
  • Hypercellular and inflamed glomeruli (Crescent formation)
- (pauci-immune) +/-
  • Churg Strauss syndrome
  • Necrotizing granulomas (Lungs and kidneys)
  • Asthma
  • Peripheral neuropathy
+/- + + + + + - - +

+ (C-ANCA)

- -
  • Hypercellular and inflamed glomeruli (Crescent formation)
- (pauci-immune) -
  • Microscopic polyngitis
  • Necrotizing vasculitis (no granuloma)
+/- + + + + + - - +

+ (P-ANCA)

- -
  • Hypercellular and inflamed glomeruli (Crescent formation)
- (pauci-immune) -
Membranoproliferative glomerulonephritis
  • Idiopathic
  • Hepatitis B and C (Type 1)
  • C3 nepritic factor (Type2)
  • Hemeturia
  • Oliguria
  • Periorbital edema
  • Hypertension
+/- + + + + + - + - - - +
  • Thick glomerular basement membrane (Tram-track appearance)
  • Mesangial proliferation and leukocyte infiltration
+ (Granular)

References

Template:WH Template:WS