Familial primary amyloidosis with severe amyloid heart disease

摘要: Abstract Among twelve siblings there were five cases of primary systemic amyloidosis. The anatomic findings are reported, based particularly on two autopsy cases. predominant clinical feature was cardiac involvement, manifesting itself as progressive failure three to six years' duration, and appearing when the patients in their forties. characteristic hemodynamic findings, with dip-plateau tracings, described. According history, other appeared be suffering from same disease. Family studies did not reveal any signs amyloid disease, but age persons examined below onset symptoms overt Serum protein studies, however, revealed some abnormalities obscure nature third generation.