title: Acquired generalized lipodystrophy associated with autoimmune hepatitis and low serum C4 level.
abstract: Lipodystrophies are a group of diseases characterized by loss of fat tissue and are associated with insulin resistance. A six-year-old girl followed with the diagnosis of autoimmune hepatitis showed a severe loss of fat tissue, hyperinsulinemia, impaired glucose tolerance, hypertriglyceridemia and low serum complement 4 (C4) levels. She had coarse facial features with generalized loss of subcutaneous fat and prominent muscularity. Remarkable acanthosis nigricans was present over the neck, axilla, and umbilicus. Two hours after glucose loading, the glucose tolerance test revealed a glucose level of 258 mg/dL, a HbA1c value of 6.8%, and an insulin level of 642.9 mIU/mL, documenting a state of insulin resistance and type 2 diabetes mellitus. Acquired generalized lipodystrophy was diagnosed and metformin with dietary intervention was initiated. Low serum complement levels proved the autoimmune nature of the process. We conclude that the serum complement levels must be investigated in patients with acquired lipodystrophy, particularly when it is associated with autoimmune hepatitis.
authors.affiliation: Department of Pediatric Endocrinology, Uludağ University, Faculty of Medicine, Bursa, Turkey. erderen@yahoo.com
authors.forename: Erdal
authors.initials: E
authors.lastname: Eren
authors.forename: Tanju Başarır
authors.initials: TB
authors.lastname: Özkan
authors.forename: Esra D Papatya
authors.initials: ED
authors.lastname: Çakır
authors.forename: Halil
authors.initials: H
authors.lastname: Sağlam
authors.forename: Ömer
authors.initials: Ö
authors.lastname: Tarım
mesh_terms: D002648:Child; D003181:Complement C4 / Q000172:deficiency*; D005260:Female; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008060:Lipodystrophy / Q000097:blood / Q000150:complications* / Q000503:physiopathology
chemical_list: D003181:Complement C4