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AFib amyloidosis

Disease definition

A rare, hereditary amyloidosis with primary renal involvement characterized by fibrinogen A-alpha-chain amyloid deposition predominantly in the kidney glomeruli and clinically presenting with hypertension, uremia, nephrotic syndrome slowly progressing to end-stage renal disease. Extra-renal involvement is possible, due to neurological, cardiac, visceral and vascular amyloid deposition.

ORPHA:93562

Classification level: Subtype of disorder
  • Synonym(s):
    • Familial amyloid nephropathy due to fibrinogen A alpha-chain variant
    • Fibrinogen A alpha-chain amyloidosis
    • Hereditary amyloid nephropathy due to fibrinogen A alpha-chain variant
    • Hereditary renal amyloidosis due to fibrinogen A alpha-chain variant
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: -
  • ICD-10: E85.0
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

Article for general public

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