Cardiac examination in children with Laron syndrome undergoing mecasermin therapy.

Journal of Pediatric Endocrinology & Metabolism : JPEM
Nurdan ErolAyse Yıldırım

Abstract

Laron syndrome (LS), which can be defined as primary growth hormone resistance or insensitivity, is a rare genetic disease inherited by an autosomal recessive trait. Although it is undistinguishable from growth hormone deficiency, LS has high levels of growth hormone, but insulin-like growth factor (IGF-1) cannot be synthesized. Mecasermin treatment is the only option for the patients who suffer from LS. This study aims to research cardiac findings of children with LS, who receive treatment with mecasermin. The study enrolled five children four males and one female, 4 M/1 F with LS, two of whom were siblings with a mean age of 6.3±2.1 years, a body weight of 13.36±4.74 kg, a height of 88±8.7 cm, and a body mass index (BMI) of 16.47±3.35. Their demographic data were obtained from their family and files. The children received mecasermin via subcutaneous injection at 0.04-0.12 μg/kg doses twice per day. The duration of mecasermin treatment was 8-53 months. All of them were examined clinically by electrocardiogram and echocardiogram. Their cardiac examinations were normal, except for one case, who had systolic murmur at cardiac auscultation. Arrhythmia was not observed on their electrocardiograms. The echocardiograms did not show a...Continue Reading

References

Apr 12, 2001·The Journal of Clinical Endocrinology and Metabolism·P F BackeljauwUNKNOWN GHIS Collaborative Group. Growth hormone insensitivity syndrome
Mar 6, 2004·The Journal of Clinical Endocrinology and Metabolism·Zvi Laron
Dec 29, 2006·The Journal of Clinical Endocrinology and Metabolism·Steven D ChernausekUNKNOWN GH Insensitivity Syndrome Collaborative Group
Oct 11, 2007·European Journal of Endocrinology·Ron G Rosenfeld
Jan 2, 2009·Growth Hormone & IGF Research : Official Journal of the Growth Hormone Research Society and the International IGF Research Society·Mickey ScheinowitzZvi Laron
Jul 25, 2009·BioDrugs : Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy·Stephen F Kemp
Nov 3, 2009·The Journal of Clinical Endocrinology and Metabolism·L Kurt MidyettUNKNOWN MS301 Study Group
Mar 28, 2012·The Journals of Gerontology. Series A, Biological Sciences and Medical Sciences·Zoltan Ungvari, Anna Csiszar
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Apr 1, 2015·Hormone Research in Pædiatrics·Peter BangUNKNOWN EU IGFD Registry Study Group

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