Metabolic studies and autonomic function in children with partial lipodystrophy.

Studies of carbohydrate metabolism in 6 children with partial lipodystrophy (none of whom had evidence of renal disease) showed raised fasting serum insulin concentrations. In 4 of the children hyperinsulinaemia was found after oral glucose. Plasma glucose levels within the normal range in all tests indicated that the hyperinsulinaemia was due to insulin resistance. Plasma growth hormone concentration was normal in all children, and plasma cortisol concentration was normal in 5. Concentrations of plasma nonesterified fatty acid (NEFA), serum triglyceride, and cholesterol in fasting blood were normal. Adipose tissue from nondystrophic areas showed an increased proportion of palmitoleic acid. No abnormality of the autonomic nervous system was found. Treatment of 2 patients with propranolol did not improve their lipodystrophy. The metabolic abnormalities present at an early age of the disease show that partial lipodystrophy should not be regarded only as a cosmetic disorder.