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

Obesity and renal failure are common manifestations in the autosomal recessive Bardet-Biedl (BE) syndrome. Because obesity and hypertension have been reported frequently in non-homozygous relatives of BE patients, we hypothesized that BE heterozygotes are predisposed to these conditions. Clinical information was collected from 34 parents of BE homozygotes, who are obligate heterozygotes. The proportion of severely overweight fathers (26.7%) was significantly higher than that in comparably aged United States white males (8.9%). We conclude that the BE gene may predispose male heterozygous carriers to obesity. If BE heterozygotes are 1% of the general population, we estimate that approximately 2.9% of all severely overweight white males carry a single BE gene. The BE parents of both sexes were also significantly taller than U.Ss white men and women of comparable age. (C) 1995 Wiley-Liss, Inc.