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Hereditary amyloidosis with primary renal involvement

Disease definition

A group of rare renal diseases, characterized by amyloid fibril deposition of apolipoprotein A-I or A-II (AApoAI or AApoAII amyloidosis), lysozyme (ALys amyloidosis) or fibrinogen A-alpha chain (AFib amyloidosis) in one or several organs. Renal involvement leading to chronic renal disease and renal failure is a common sign. Additional manifestations depend on the organ involved and the type of amyloid fibrils deposited.

ORPHA:85450

Classification level: Disorder
  • Synonym(s):
    • Amyloidosis, Ostertag type
    • Familial amyloid nephropathy
    • Familial renal amyloidosis
    • Hereditary amyloid nephropathy
    • Hereditary renal amyloidosis
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: E85.0
  • OMIM: 105200
  • UMLS: -
  • MeSH: -
  • GARD: 8282
  • MedDRA: -

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