1984 Volume 26 Issue 8 Pages 1045-1054
An unusal familial glomerular disease was found in two siblings. Among the 22 members (11 males, 11 females) of this pedigree, eight were affected by glomerulonephritis and three developed renal failure. In this pedigree, none had auditory or ocular abnormalities. Throuh the clinical course, neither nephrotic syndrome nor hypertention was observed in the two siblings which were examined, however, after 22 months from the discovery of proteinuria, an elder brother needed hemodialysis because of renal failure. The renal histrogical findings concerning these two siblings showed a characteristic lesion of focal glomerulosclerosis and thinning of the glomerular basement membrane. Hyperprolinemia was also observed in these two siblings. This disease was not HLAlinked in the five tested members of this pedigree. The mode of inheritance appeared to be autosomal dominant.
