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Clin Diabetes. 2016 Oct;34(4):181-186.

Cardiac Manifestations of Congenital Generalized Lipodystrophy.

Author information

1
Division of Cardiology, University of Texas Health Science Center at San Antonio and Audie L. Murphy VA Hospital, San Antonio, TX.
2
Metabolic Institute of America, Tarzana, CA.

Abstract

IN BRIEF Congenital lipodystrophy is a rare genetic disorder characterized by a near-complete absence of fat cells, hypoleptinemia leading to a voracious appetite, and marked insulin resistance. This article focuses on the known cardiovascular manifestations of patients with congenital lipodystrophy, including cardiomyopathy, cardiac arrhythmias, and accelerated atherosclerosis arising from a markedly deranged metabolic milieu. Future research that targets leptin deficiency (metreleptin) and apoC3 mRNA (antisense oligonucleotide) could open a window for potential pharmacological treatment of this challenging disorder.

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