Renal function in familial amyloidosis with polyneuropathy.

The renal function is assumed to be normal in most patients with familial amyloidosis with polyneuropathy (FAP) type 1. In the present study of 24 patients with FAP type 1, estimation of the urinary concentrating capacity and 51Cr-EDTA clearance demonstrated that the glomerular clearance and the concentrating capacity were imparied in most patients. The concentrating capacity was significantly correlated with the clearance values. Urine electrophoresis indicated a glomerular cause for the proteinuria. It is suggested that the renal dysfunction should be attributed to deposition of amyloid substance in the glomeruli.