familial visceral amyloidosis
- familial visceral amyloidosis
- a group of rare, autosomal dominant systemic amyloidoses, not associated with transthyretin, caused by mutations in any of a large number of genes and characterized by deposition of amyloid in the viscera without neuropathy. It is manifested by nephropathy, arterial hypertension, hepatosplenomegaly, albuminuria, hematuria, and pitting edema. Affected patients usually die within ten years of onset of clinical manifestations. Called also Ostertag or Ostertag type a.
Medical dictionary.
2011.
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