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Cardiac Manifestations of Congenital Generalized Lipodystrophy.

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.

SUBMITTER: Sanon VP 

PROVIDER: S-EPMC5070584 | biostudies-other | 2016 Oct

REPOSITORIES: biostudies-other

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Cardiac Manifestations of Congenital Generalized Lipodystrophy.

Sanon Vani P VP   Handelsman Yehuda Y   Pham Son V SV   Chilton Robert R  

Clinical diabetes : a publication of the American Diabetes Association 20161001 4

<b>IN BRIEF</b> 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)  ...[more]