Report of Congenital Generalized Lipodystrophy in Children's Medical Center, Tehran, Iran

Abstract

Generalized lipodystrophy is a rare disorder in children characterized by absence of subcutaneous adipose tissue. We evaluated 10 patients with generalized lipodystrophy by skin and liver biopsy. Eight patients with steatohepatitis and cirrhosis were treated with Ursobile (uorsodeoxycholic acid) and vitamin E. In follow-up, serum triglyceride level was decreased.