A Case Report: Congenital Lipodystrophy

Abstract

Congenital lipodystrophy is a rare autosomal recessive disorder and in all subjects, there is a near-total absence of adipose tissue resulting in aberrant storage of dietary and endogenous fat in metabolically important tissues such as muscle and liver. Patients with lipodystrophy often have some of the disturbances such as dyslipidemia with high levels of triglycerides circulating in the bloodstream (hypertriglyceridemia) and decreased high-density lipoprotein (HDL), dysglycemia and insulin resistance similar to metabolic syndrome. In patients with congenital lipodystrophy low-fat dietary treatment with supplemental medium chain triglycerides is recommended. Appropriate growth and development along with decrease in triglyceride levels can be achieved by the dietary treatment. In this case report we discussed via a case experience, nutritional strategies in congenital lipodystrophy.