Sex differences in severity of metabolic complications in patients with congenital generalized lipodystrophy

CONTEXT: Previous data support a higher prevalence of metabolic complications in females affected with familial partial lipodystrophy than in males. However, whether there are any sex differences in the prevalence and severity of metabolic complications among patients with congenital generalized lipodystrophy (CGL) remains unclear. OBJECTIVES: To compare prevalence of metabolic complications and metabolic parameters between males and females with CGL. DESIGN: A retrospective, cross-sectional comparison. SETTINGS: Patients from two tertiary referral centers from Northeastern Brazil - Universidade Federal do Rio Grande do Norte, Natal, and Universidade Federal do Ceará, Fortaleza. PATIENTS: A total of 44 females (age 2-51 years) and 28 males (age 5-46 years) with CGL. Twenty-six (7 M, 19 F) had genotypically confirmed CGL type 1, 43 (20 M, 23 F) had CGL type 2, and 3 patients were not genotyped. MAIN OUTCOME MEASURES: Prevalence of diabetes mellitus and hypertriglyceridemia, and metabolic variables. RESULTS: Females with CGL, as compared to males, had significantly increased prevalence of diabetes (82% vs. 54%; p<0.01) and hypertriglyceridemia (91% vs. 71%; p=0.05); and had higher median fasting serum triglycerides levels (383 vs. 221 mg/dL; p=0.01), but not statistically significantly higher median hemoglobin A1c (7.9% vs. 6.5%; p=0.37) and fasting serum glucose levels (138 vs. 89 mg/dL; p=0.06). CONCLUSIONS: Compared to males, females with CGL have increased prevalence of metabolic complications, such as diabetes and hypertriglyceridemia, suggesting need for close monitoring of metabolic parameters, and early and aggressive intervention among them.