Hereditary amyloidosis with primary renal involvement
ORPHA:85450· ICD-10 E85.0
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.
- Prevalence
- Unknown
- Inheritance
- Autosomal dominant
- Age of onset
- All ages