Familial renal amyloidosis. Case reports, literature review and classification.

Three siblings (two brothers and a sister) of Polish origin, presented in late middle age with the features of the nephrotic syndrome and hypertension. Glomerular deposition of amyloid was found in all on renal biopsy. No amyloid was seen on rectal or gingival biopsy. Their mother and a maternal aunt died in middle age with a clincally similar disease. These cases are compared with other forms of hereditary amyloidosis which are briefly reviewed. The hereditary amyloidoses are classified with particular emphasis on neural and renal involvement.