Hereditary amyloidosis with primary renal involvement

Orpha code: 85450OMIM code: 105200

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.

Disease data
Classification

Disease

Synonyms
Amyloidosis, Ostertag type
Amyloidoza, typ Ostertaga
Dziedziczna amyloidoza nerek
Dziedziczna nefropatia amyloidowa
Rodzinna amyloidoza nerkowa
Rodzinna nefropatia amyloidowa
Familial amyloid nephropathy
Familial renal amyloidosis
Hereditary amyloid nephropathy
Hereditary renal amyloidosis
ORPHA code
85450
OMIM code
105200
ICD10 code
E85.0
ICD11 code
-

No additional description.

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