Does Allopurinol Affect the Progression of Familial Juvenile Gouty Nephropathy?

Abstract

Familial juvenile gouty nephropathy (FJGN, McKusick 16200) first described by Duncan and Dixon (1), is an autosomal dominant condition with high penetrance and characteristic features. It affects a sub-group of young people who develop early progressive renal dysfunction. A common hallmark is hyperuricaemia disproportionate to age, sex and degree of renal failure. The nature of the biochemical defect remains unknown and there is debate as to whether the abnormal tubular handling of urate causes the damage (2, 3), or if the treatment for the hyperuricaemia affects the evolution of the disease (2, 4). Histologically, a non specific interstitial nephritis (NSIN) is usually seen without evident deposits of sodium urate (1, 2, 3).