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

Cardiac Manifestations of Congenital Generalized Lipodystrophy.

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


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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