Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja A rare, hereditary amyloidosis with primary renal involvement characterized by renal interstitial and medullary deposition of amyloid, low plasma levels of ApoA-1 and slow disease progression. Main clinical signs and symptoms are hypertension, proteinuria, hematuria and edema due to chronic renal insufficiency leading to end stage renal disease. Hepatosplenomegaly, progressive cardiomyopathy and involvement of skin, testes and adrenals (hypergonadotropic hypogonadism) have also been reported. Disease data Klasyfikacja Clinical subtype Synonimy Apolipoprotein A-I amyloidosis Amyloidoza związana z apolipoproteiną A - wariant I Dziedziczna amyloidoza nerek spowodowana wariantem I apolipoproteiny A Dziedziczna nefropatia amyloidowa spowodowana wariantem I apolipoproteiny A Rodzinna amyloidoza nerek spowodowana wariantem I apolipoproteiny A Rodzinna nefropatia amyloidowa spowodowana wariantem I apolipoproteiny A Familial amyloid nephropathy due to apolipoprotein A-I variant Familial renal amyloidosis due to apolipoprotein A-I variant Hereditary amyloid nephropathy due to apolipoprotein A-I variant Hereditary renal amyloidosis due to apolipoprotein A-I variant Kod ORPHA 93560 Kod OMIM - Kod ICD10 E85.0 Kod ICD11 - *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