Description of the disease * EnglishPolish Pobierz sekcję do PDF 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. Disease data Classification Clinical subtype Synonyms Familial amyloid nephropathy due to fibrinogen A alpha-chain variant Amyloidoza fibrynogenu A łańcuchów alfa Dziedziczna amyloidoza nerek spowodowana wariantem fibrynogenu A łańcuchów alfa Dziedziczna nefropatia amyloidowa spowodowana wariantem fibrynogenu A łańcuchów alfa Rodzinna nefropatia amyloidowa spowodowana wariantem fibrynogenu A łańcuchów alfa 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 ORPHA code 93562 OMIM code - ICD10 code E85.0 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl