Lipodystrophy Syndromes - 04/11/16

Doi : 10.1016/j.ecl.2016.06.012

Iram Hussain, MD ^a, Abhimanyu Garg, MD ^b,^⁎
^a Division of Endocrinology, Department of Internal Medicine, UT Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-8537, USA
^b Division of Nutrition and Metabolic Diseases, Department of Internal Medicine, Center for Human Nutrition, UT Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-8537, USA

^∗Corresponding author.

Résumé

Lipodystrophies are heterogeneous disorders characterized by varying degrees of body fat loss and predisposition to insulin resistance and its metabolic complications. They are subclassified depending on degree of fat loss and whether the disorder is genetic or acquired. The two most common genetic varieties include congenital generalized lipodystrophy and familial partial lipodystrophy; the two most common acquired varieties include acquired generalized lipodystrophy and acquired partial lipodystrophy. Highly active antiretroviral therapy–induced lipodystrophy in patients infected with human immunodeficiency virus and drug-induced localized lipodystrophy are common subtypes. The metabolic abnormalities associated with lipodystrophy include insulin resistance, hypertriglyceridemia, and hepatic steatosis. Management focuses on preventing and treating metabolic complications.

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Keywords : Lipodystrophy, Congenital generalized lipodystrophy, Familial partial lipodystrophy, Acquired generalized lipodystrophy, Acquired partial lipodystrophy, Metreleptin

Plan

Key points

Introduction

Genetic lipodystrophies

Congenital Generalized Lipodystrophy

Familial Partial Lipodystrophy

Acquired lipodystrophies

Acquired Generalized Lipodystrophy

Acquired Partial Lipodystrophy (Barraquer-Simons Syndrome)

Highly Active Antiretroviral Therapy–Induced Lipodystrophy in Patients Infected with Human Immunodeficiency Virus

Localized Lipodystrophies

Management

	Grant Support: This work was supported by the National Institutes of Health grant RO1 DK105448, CTSA grants UL1RR024982 and UL1TR001105, and the Southwest Medical Foundation.
	Disclosure Statement: Dr I. Hussain has no disclosures. Dr A. Garg coholds a patent regarding use of leptin for treating human lipoatrophy and the method of determining predisposition to this treatment but receives no financial compensation. He receives research grant support from Aegerion, Pfizer, and Ionis Pharmaceuticals and is a consultant for Aegerion.