NEWLY DISCOVERED FAMILIAL JUVENILE GOUTY NEPHROPATHY IN A JAPANESE FAMILY

Our attention was initially called to 2 young Japanese sisters with go ut and renal insufficiency, which led to an investigation of members o f their family with similar conditions. One sister, a 26-year-old woma n who had suffered from polyuria since infancy, suffered from gout and renal insufficiency. Her younger sister also had a history of polyuri a, hyperuricemia, and moderately reduced renal function. Their urinary uric acid levels were reduced but purine enzyme activities in the ery throcytes were normal. A renal biopsy specimen from the younger sister showed severe interstitial fibrosis with tubular atrophy. An investig ation of the family revealed an autosomal dominant transmission patter n. We believe these are new familial cases of juvenile gouty nephropat hy found in a Japanese family.