Difference between revisions of "Sandbox:Sahar"

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{{familytree/start |summary=Sample 1}}
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{{familytree | | | | | E04 | | | | |E04=[[Infection]]/[[Inflammation]]}}
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{{familytree | | | | | |!| | | | }}
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{{familytree | | | | | E05 | | | | |E05=Increased production of [[IL-1]]/[[IL-6]]/[[TNF-α]]}}
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{{familytree | | | | | |!| | | | }}
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{{familytree | | | | | C05 | | | | |C05=Upregulation of [[hepatic]] serum amyloid A production}}
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{{familytree | | | | | |!| | | | | | }}
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{{familytree | | | | | C04 | | | | |C04=SAA production uptake by [[macrophages]]}}
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{{familytree | | | | | |!| | | | }}
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{{familytree | | | | | D05 | | | | |D05=C-terminal cleavage of SAA}}
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{{familytree | | | | | |!| | | | }}
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{{familytree | | | | | F01 | | | | |F01=β-sheet configuration of SAA}}
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{{familytree | | | | | |!| | | | }}
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{{familytree | | | | | G05 | | | | |G05=Fibril deposition in [[extracellular space]]}}
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{{familytree | | | | | |!| | | | }}
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{{familytree | | | | | H05 | | | | |H05=Binding of [[glycosaminoglycan]], serum amyloid P, and [[lipid]] components}}
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{{familytree | | | | | |!| | | | }}
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{{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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Revision as of 04:43, 10 November 2019

 
 
 
 
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[1]












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.

  1. Rumjon A, Coats T, Javaid MM (2012). "Review of eprodisate for the treatment of renal disease in AA amyloidosis". Int J Nephrol Renovasc Dis. 5: 37–43. doi:10.2147/IJNRD.S19165. PMC 3304340. PMID 22427728.

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