Growth Hormone Treatment in Prader-Willi Syndrome.

Ji Eun Park; Seung Won Lee; Kyoung Eun Song; Hyoung Suk Lee; Dae Jung Kim; Yoon Sok Chung; Kwan Woo Lee; Hyon Joo Kim

doi:10.3803/jkes.2006.21.1.40

Original Article Growth Hormone Treatment in Prader-Willi Syndrome.: Ji Eun Park, Seung Won Lee, Kyoung Eun Song, Hyoung Suk Lee, Dae Jung Kim, Yoon Sok Chung, Kwan Woo Lee, Hyon Joo Kim; Endocrinology and Metabolism 2006;21(1):40-46
DOI: https://doi.org/10.3803/jkes.2006.21.1.40
Published online: February 1, 2006

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¹Department of Endocrinology and Metabolism, Ajou University School of Medicine, Suwon, Korea.
²Department of Clinical Genetics, Ajou University School of Medicine, Suwon, Korea.

Abstract

BACKGROUND
Prader-Willi syndrome (PWS) is a congenital disorder, which is clinically characterized by a short stature, muscular hypotonia, hypogonadism, mental retardation and hyperphagia, leading to early childhood obesity. Impaired growth hormone (GH) secretion, hypogonadism, and obesity are common in patients with PWS. The purpose of this study was to find the effects of growth hormone treatment in patients with PWS. METHODS: Six patients with PWS confirmed by a genetic study were recruited, and treated with growth hormone(Eutropin(R))(0.8-1 IU/kg/week) divided into five or seven day doses per week for six months. The heights and weights of the subjects were evaluated. GH status were evaluated using the serum insulin-like growth factor (IGF)-I level, the L-dopa test, and insulin-induced hypoglycemia tess. Glucose metabolism was evaluated using the random serum glucose and HbA1c levels. RESULTS: GH was found to be deficient in 2 out of 6 subjects by the insulin test, in 3 out of 6 by the IGF-I level, and in 5 out of in 5 by the L-dopa test. After six months of GH treatment, the height percentile was increased and weight percentile decreased. The serum glucose and HbA1c levels remained unchanged. CONCLUSION: Six months of GH treatment in patients with PWS improved the height and degree of obesity. This study has shown the beneficial effects of GH treatment for patients with PWS, and without significant side effects.

Keywords: Growth hormone;Height;Weight;Prader-Willi syndrome;

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Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study
Su Jin Kim, Joong Bum Cho, Min Jung Kwak, Eun Kyung Kwon, Kyung Hoon Paik, Dong-Kyu Jin
Korean Journal of Pediatrics.2008; 51(7): 742. CrossRef
Phenotype-genotype correlations and the efficacy of growth hormone treatment in Korean children with Prader-Willi syndrome
Keun Wook Bae, Jung Min Ko, Han-Wook Yoo
Korean Journal of Pediatrics.2008; 51(3): 315. CrossRef

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