Successful cardiac transplantation in a patient with congenital generalized lipodystrophy

Pediatric Transplantation
Sonia Marcos-AlonsoFrancisco Portela Torrón

Abstract

We report a case of a 12-yr-old boy referred to our unit with congenital generalized lipodystrophy and dilated cardiomyopathy related to a lamin gene mutation. He progressively developed end-stage heart failure and was referred for heart transplant evaluation. The patient's lipid profile, glucose level, and renal function were normal, and vascular retinopathy was ruled out. He underwent orthotopic bicaval HT and had an uneventful recovery. He was discharged home two wk after surgery with good graft function. During follow-up, he developed hyperglycemia and dyslipidemia, which were controlled by increasing leptin dose and starting oral antidiabetic drugs. The patient is currently doing well two yr after transplantation.

References

Sep 1, 1978·Transplantation·R E CasaliC Kjellstrand
Feb 22, 2002·The New England Journal of Medicine·Elif Arioglu OralAbhimanyu Garg
Feb 14, 2004·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·Michael McNallyAllan D Kirk
Dec 21, 2005·Molecular Genetics and Metabolism·Katherine N Jacob, Abhimanyu Garg
Apr 8, 2006·Current Opinion in Cardiology·Nicolas Sylvius, Frédérique Tesson
Mar 22, 2007·Journal of Lipid Research·Robert A HegeleBrian K Rutt
May 10, 2008·Pediatric Transplantation·Clifford ChinStanton B Perry
Oct 31, 2009·The Journal of Clinical Endocrinology and Metabolism·Abhimanyu GargYanick Crow

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Citations

Dec 22, 2017·The Journal of Clinical Endocrinology and Metabolism·Iram HussainAbhimanyu Garg

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