Difference between revisions of "Sandbox:Sahar"

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! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Amyloisosis subtype}}
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! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Protein}}
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! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|Involved organs severity}}
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! style="background: #4479BA; width: 100px;" | {{fontcolor|#FFF|}}
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Primary
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|[[Anemia]]
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|[[MRI]] is the best radiologic tool to differentiate between retroperitoneal masses.
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Secondary
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|✔
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|[[Leukocytosis]], positive inflammatory markers
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |DRA (dialysis related amyloidosis)
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|✔
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|positive [[tumor marker]]
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Senile systemic (cardiac) amyloidosis
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|[[Diabetes mellitus|DM type II]], [[amylase]] and [[lipase]] levels may be slightly elevated
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |Meretoja syndrome
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{{familytree/start |summary=Sample 1}}
 
{{familytree/start |summary=Sample 1}}
 
{{familytree | | | | | E04 | | | | |E04=[[Infection]]/[[Inflammation]]}}
 
{{familytree | | | | | E04 | | | | |E04=[[Infection]]/[[Inflammation]]}}
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{{familytree | | | | | K05 | | | | |K05=Resistant to proteolysis}}
 
{{familytree | | | | | K05 | | | | |K05=Resistant to proteolysis}}
 
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! colspan="2" style="background:#DCDCDC;" align="center" + |The above algorithm is adopted from International Journal of Nephrology and Renovascular Disease<ref>{{cite journal |vauthors=Rumjon A, Coats T, Javaid MM |title=Review of eprodisate for the treatment of renal disease in AA amyloidosis |journal=Int J Nephrol Renovasc Dis |volume=5 |issue= |pages=37–43 |date=2012 |pmid=22427728 |pmc=3304340 |doi=10.2147/IJNRD.S19165 |url=}}</ref>
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! colspan="2" style="background:#DCDCDC;" align="center" + |The above algorithm is adopted from International Journal of Nephrology and Renovascular Disease
 
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<references />

Revision as of 05:29, 10 November 2019

Amyloisosis subtype Protein Involved organs severity
Primary _ Anemia MRI is the best radiologic tool to differentiate between retroperitoneal masses.
Secondary Leukocytosis, positive inflammatory markers
DRA (dialysis related amyloidosis) positive tumor marker
Senile systemic (cardiac) amyloidosis _ DM type II, amylase and lipase levels may be slightly elevated
Meretoja syndrome
 
 
 
 
Infection/Inflammation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Increased production of IL-1/IL-6/TNF-α
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Upregulation of hepatic serum amyloid A production
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
SAA production uptake by macrophages
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
C-terminal cleavage of SAA
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
β-sheet configuration of SAA
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Fibril deposition in extracellular space
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Binding of glycosaminoglycan, serum amyloid P, and lipid components
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Resistant to proteolysis
 
 
 
 
The above algorithm is adopted from International Journal of Nephrology and Renovascular Disease












Associated Conditions
Conditions Examples
Chronic infections
Monogenic periodic fever syndromes
Conditions predisposing to recurrent infections
Neoplasia
Inflammatory Arthritis
Systemic Vasculitis
Others
Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
Nephrotic Syndrome and Renal Failure Secondary (AA) Amyloidosis
  • Biopsy and congo-red staining of the sample
Primary (AL) Amyloidosis


Diabetic Nephropathy
Minimal Change Disease
Focal Segmental Glomerulosclerosis
  • Biopsy:
    • Podocyte foot process effacement
    • Capillary lumen abolished by the segmental increase in matrix
Fabry's Disease
  • Deficient alpha galactosidase A
Light Chain Deposition Disease
  • Biopsy:
    • Non-amyloid granules
Membranous Glomerulonephritis
Fibrillary-Immunotactoid Glomerulopathy
  • Biopsy:
    • Polycloncal IgG deposits
    • Infiltration of glomerular structures by amorphous acellular material (nonbranching fibrils 12-24nm in diameter)
    • Ig heavy-chain and one light-chain subclass
Organ System Involvement Differential Diagnosis Causes Clinical Features Laboratory Findings Gold Standard Test Therapy
Polyneuropathy POEMS syndrome (Demyelinating)
Metabolic Syndrome (Axonal pathology)
Vitamin Deficiencies (Axonal Pathology)



Guillain-Barre Syndrome (Demyelinating)
  • Delayed F waves
  • Clinical diagnostic criteria (progressive weakness of more than two limbs, areflexia, and progression for no more than four weeks)
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (Mixed axonal and demyelinatiing)


  • EFNS/PNS criteria
  • Koski criteria
Multifocal Motor Neuropathy
  • Progressive, asymmetric, distal and upper limb predominant weakness
  • No significant sensory abnormalities
  • Areflexia
  • Clinical criteria (EFNS/PNS):
    • Slowly progressive or step-wise progressive, focal, asymmetric limb weakness; i.e., motor involvement in the motor nerve distribution of at least two nerves for > 1 month.
    • No objective sensory abnormalities except for minor vibration sense abnormalities in the lower limbs


Organ System Involvement Differential Diagnosis Causes Features Laboratory Findings Gold Standard Test Therapy
Organomegaly (Hepatosplenomegaly and Lymphadenopathy) Malaria
Kala-azar


Infective Hepatitis
Chronic Myelogenous Leukemia (CML)
Lymphoma
Primary (AL) Amyloidosis
  • Typical green birefringence under polarized light after Congo red staining (appears in red under normal light)
  • Congo red staining
  • Melphalan-prednisone/dexamethasone
  • Dexamethasone plus Cyclophosphamide-thalidomide
  • Stem cell transplantation
Gaucher's Disease



Example #1

The patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.


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