Growth hormone secretion and its effect on height in pediatric patients with different genotypes of Prader-Willi syndrome

American Journal of Medical Genetics. Part a
Yuji OtoToshiro Nagai

Abstract

There have been multiple reports regarding the growth hormone (GH) secretion in patients with Prader-Willi syndrome (PWS). However, none have compared GH secretion in children with deletion group to those with maternal uniparental disomy (UPD). We evaluated the GH secretion in pediatric patients with PWS. Seventy-six patients with a deletion (n = 55) or UPD (n = 21) were studied. The secretion of GH by insulin stimulation in the patients with UPD (3.6 ± 2.2 ng/ml) was significantly lower than those with deletions (peak GH level: 11.1 ± 8.6 ng/ml; P = 0.0013). We also compared the response to GH replacement therapy. Yearly improvements in height standard deviation score (SDS) were similar in the two groups (first year SDS: 0.47 ± 0.47, deletion; 0.68 ± 0.26, UPD; P = 0.14).

References

Nov 15, 2000·American Journal of Medical Genetics·T NagaiN Niikawa
Aug 6, 2002·The Journal of Clinical Endocrinology and Metabolism·Charlotte HöybyeMarja Thorén
Apr 26, 2003·Journal of Pediatric Endocrinology & Metabolism : JPEM·Kazuo ObataRyoichi Sakuta
Sep 21, 2006·Clinical Endocrinology·G GrugniUNKNOWN Genetic Obesity Study Group of the Italian Society of Pediatric Endocrinology and Diabetology
Aug 14, 2008·The Journal of Clinical Endocrinology and Metabolism·A P GoldstoneUNKNOWN speakers contributors at the Second Expert Meeting of the Comprehensive Care of Patients with PWS
Mar 10, 2009·American Journal of Medical Genetics. Part a·Graziano GrugniPaolo Marzullo
Nov 5, 2011·American Journal of Medical Genetics. Part a·Sung Won ParkDong-Kyu Jin

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Citations

Oct 29, 2013·Archives of Disease in Childhood·Nicola Bridges
Oct 5, 2013·Growth Hormone & IGF Research : Official Journal of the Growth Hormone Research Society and the International IGF Research Society·E MarosticaA Sartorio
May 23, 2013·American Journal of Medical Genetics. Part a·Merlin G ButlerAnn M Manzardo
Feb 28, 2013·Clinical Endocrinology·Laura AudíCarrascosa Antonio
Apr 27, 2018·American Journal of Medical Genetics. Part a·Yuji OtoToshiro Nagai
Nov 20, 2016·Pediatric Obesity·M J KhanM G Shaikh
Mar 1, 2015·Expert Review of Endocrinology & Metabolism·Angela K Lucas-HeraldM Guftar Shaikh

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