Successful cardiac transplantation in a patient with congenital generalized lipodystrophy

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, gluco...

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Bibliographic Details
Published in:Pediatric transplantation 2016-03, Vol.20 (2), p.321-324
Main Authors: Marcos‐Alonso, Sonia, Bautista‐Hernandez, Víctor, Portela Torrón, Francisco
Format: Article
Language:English
Subjects:
Administration, Oral
Angiography
Blood Glucose - analysis
Body Mass Index
cardiomyopathy
Cardiomyopathy, Dilated - complications
Cardiomyopathy, Dilated - surgery
Child
Constriction, Pathologic
Dyslipidemias - complications
Heart Failure - surgery
Heart Transplantation
Humans
Hyperglycemia - complications
Hypoglycemic Agents - therapeutic use
Kidney Function Tests
Lamin Type A - genetics
lipodystrophy
Lipodystrophy, Congenital Generalized - complications
Lipodystrophy, Congenital Generalized - surgery
Male
Mutation
pediatric heart transplant
Treatment Outcome
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Summary: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.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.12656