Complications of lipodystrophy syndromes


Akinci G., ÇELİK M., AKINCI B.

PRESSE MEDICALE, vol.50, no.3, 2021 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Review
  • Volume: 50 Issue: 3
  • Publication Date: 2021
  • Doi Number: 10.1016/j.lpm.2021.104085
  • Journal Name: PRESSE MEDICALE
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, PASCAL, L'Année philologique, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
  • Keywords: Lipodystrophy, Leptin, Insulin resistance, Organ complications, CONGENITAL GENERALIZED LIPODYSTROPHY, ACQUIRED PARTIAL LIPODYSTROPHY, FAMILIAL PARTIAL LIPODYSTROPHY, CLINICAL-FEATURES, ADIPOSE-TISSUE, INCREASED RISK, DUNNIGAN-TYPE, LEPTIN, MUTATION, DISEASE
  • Dokuz Eylül University Affiliated: Yes

Abstract

Lipodystrophy syndromes are rare complex multisystem disorders caused by generalized or partial lack of adipose tissue. Adipose tissue dysfunction in lipodystrophy is associated with leptin deficiency. Lipodystrophy leads to severe metabolic problems. These abnormalities include, but are not limited to, insulin-resistant diabetes, severe hypertriglyceridemia, and lipid accumulation in ectopic organs such as the liver, and are associated with end-organ complications. Metabolic abnormalities can be present at the time of diagnosis or may develop over time as the disease progresses. In addition to metabolic abnormalities, subtype-specific presentations due to underlying molecular etiology in genetic forms and autoimmunity in acquired forms contribute to severe morbidity in lipodystrophy. (c) 2021 Elsevier Masson SAS. All rights reserved.