familial visceral amyloidosis

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familial visceral amyloidosis,

a rare autosomal-dominant type of amyloidosis characterized by nephropathy, arterial hypertension, hepatosplenomegaly, albuminuria, hematuria, and pitting edema. Affected patients usually die within 10 years of onset of clinical manifestations. Also called Ostertag or Ostertag type a.

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