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.