Lipodystrophies are clinically heterogeneous acquired or inheriteddisorders characterized by the selective loss of adipose tissue.Affected patients are predisposed to insulin resistance andits attendant complications, including diabetes mellitus, dyslipidemia,hepatic steatosis, and acanthosis nigricans. Features of polycysticovary syndrome — hirsutism, oligoamenorrhea, and polycysticovaries — may develop in affected women. The mechanismsinvolved in the pathogenesis of various types of lipodystrophyare listed in Table 1.
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Table 1. Clinical Features and Pathogenetic Basis of Various Types of Lipodystrophies.
More than a century after the clinical phenotype was first described,1we are beginning to understand the molecular and cellular . . . [Full Text of this Article]
Acquired Lipodystrophies
Lipodystrophy in Patients with Human Immunodeficiency Virus Infection
Clinical Picture
Pathogenetic Basis
Acquired Partial Lipodystrophy (The Barraquer–Simons Syndrome)
Clinical Picture
Pathogenetic Basis
Acquired Generalized Lipodystrophy
Clinical Picture
Pathogenetic Basis
Localized Lipodystrophies
Inherited Lipodystrophies
Congenital Generalized Lipodystrophy (The Berardinelli–Seip Syndrome)
Clinical Picture
Molecular Basis of Congenital Generalized Lipodystrophy Type 1
Molecular Basis of Congenital Generalized Lipodystrophy Type 2
Other Types of Congenital Generalized Lipodystrophy
Dunnigan Variety of Familial Partial Lipodystrophy
Clinical Picture
Molecular Basis
Familial Partial Lipodystrophy Associated with PPAR Gene Mutations
Other Types of Familial Partial Lipodystrophy
Lipodystrophy Associated with Mandibuloacral Dysplasia
Clinical Picture
Molecular Basis
Other Inherited Lipodystrophies
Mechanisms of Insulin Resistance and Metabolic Complications
Therapeutic Approaches
Improving Cosmetic Appearance
Management of Dyslipidemia
Management of Hyperglycemia
Additional Considerations for HIV-Infected Patients with Lipodystrophy
Other Therapeutic Options
Conclusions
Source Information
From the Division of Nutrition and Metabolic Diseases, Department of Internal Medicine, and the Center for Human Nutrition, University of Texas Southwestern Medical Center, Dallas.
Address reprint requests to Dr. Garg at the Center for Human Nutrition, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX 75390-9052, or at abhimanyu.garg@utsouthwestern.edu.
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Gene Mutations
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