FAMILIAL HYPERURICEMIC NEPHROPATHY

This report describes a Polynesian family that had the rare combinatio n of hyperuricemia, precocious gout, hypertension, and renal failure a t an early age, with an autosomal dominant inheritance. One family mem ber had renal biopsy evidence of interstitial urate crystal deposition , a surprisingly uncommon observation in such families, and most had d ecreased fractional excretion of urate, reflecting either decreased se cretion or enhanced postsecretory renal reabsorption of uric acid. One patient has had a successful renal transplant. On the basis of these observations, family members of any such index case should be screened for this disorder. Treatment of affected members might include a uric osuric agent and/or allopurinol early in the course of the disorder. ( C) 1995 by the National Kidney Foundation, Inc.