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Амілоїдоз AApoAIV

ORPHA:439232· ICD-10 E85.8· AApoAIV amyloidosis

Визначення(English summary)

A rare nonhereditary systemic amyloidosis characterized by slowly progressive renal dysfunction, increased serum creatinine, mostly normal urine analysis with no significant proteinuria and associated heart disease. Cardiac involvement presents as hypertrophic obstructive cardiomyopathy, left ventricular outflow tract obstruction, coronary artery disease and conduction system abnormalities. Histology reveals medullar amyloid deposits, renal tubular atrophy, interstitial fibrosis, and glomerular sclerosis.

Поширеність
<1 / 1 000 000
Успадкування
Not applicable
Вік початку
Adult