Gender differences in the prevalence of metabolic complications in familial partial lipodystrophy (Dunnigan variety)

Familial partial lipodystrophy, Dunnigan type (FPLD; Mendelian Inheritance in Man #151660), is an autosomal dominant disorder characterized by loss of sc fat from the extremities and trunk since puberty and predisposition to insulin resistance and its complications. However, for lack of recognition of affected men, previous studies could not ascertain any gender difference s in phenotypic expression. Therefore, anthropometric variables and prevale nce of diabetes mellitus, dyslipidemia, hypertension, and atherosclerotic v ascular disease were compared among 17 postpubertal men and 22 women with F PLD from eight pedigrees. All individuals completed a questionnaire, and fa sting blood was analyzed for glucose, insulin, and lipoprotein concentratio ns. Both affected men and women had similar patterns of fat loss. Compared with the affected men, women had higher prevalence of diabetes (18% and 50% , respectively; P = 0.05) and atherosclerotic vascular disease (12% and 45% , respectively; P = 0.04) and had higher serum triglycerides (median values , 2.27 and 4.25 mmol/L, respectively; P = 0.02) and lower high-density lipo protein cholesterol concentrations (age-adjusted means, 0.94 and 0.70 mmol/ L, respectively; P = 0.04). The prevalence of hypertension and fasting seru m insulin concentrations were similar. In conclusion, women with FPLD are m ore severely affected with metabolic complications of insulin resistance th an men. These observations raise the possibility that women with generalize d and regional obesity may also have more severe metabolic sequelae of insu lin resistance.