OBESITY IN HETEROZYGOUS CARRIERS OF THE GENE FOR THE BARDET-BIEDL SYNDROME

Obesity and renal failure are common manifestations in the autosomal r ecessive Bardet-Biedl (BE) syndrome. Because obesity and hypertension have been reported frequently in non-homozygous relatives of BE patien ts, we hypothesized that BE heterozygotes are predisposed to these con ditions. Clinical information was collected from 34 parents of BE homo zygotes, who are obligate heterozygotes. The proportion of severely ov erweight fathers (26.7%) was significantly higher than that in compara bly aged United States white males (8.9%). We conclude that the BE gen e may predispose male heterozygous carriers to obesity. If BE heterozy gotes are 1% of the general population, we estimate that approximately 2.9% of all severely overweight white males carry a single BE gene. T he BE parents of both sexes were also significantly taller than U.Ss w hite men and women of comparable age. (C) 1995 Wiley-Liss, Inc.