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Park, Lee, Song, Lee, Kim, Chung, Lee, and Kim: Growth Hormone Treatment in Prader-Willi Syndrome
Original Article
Journal of Korean Society of Endocrinology

Journal of Korean Society of Endocrinology 2006; 21(1): 40-46.

Published online: 20 February 2006

DOI: https://doi.org/10.3803/jkes.2006.21.1.40

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 Kim1

Department of Endocrinology and Metabolism, Ajou University School of Medicine, Suwon, Korea.

1Department of Clinical Genetics, Ajou University School of Medicine, Suwon, Korea.

Received 11 June 2004       Accepted 16 June 2005

Copyright © 2006 Korean Endocrine Society

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®) (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

Figures and Tables

Fig. 1
Growth chart of boys (Case 1, 2) with growth hormone treatment.
jkse-21-40-g001
Fig. 2
Growth chart of boys (Case 3, 4) with growth hormone treatment.
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Fig. 3
Growth chart of girls (Case 5, 6) with growth hormone treatment.
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Table 1
Genetic Studies for Diagnosis of Prader-Willi Syndrome
jkse-21-40-i001
Table 2
Serum Insulin-Like Growth Factor 1 Levels
jkse-21-40-i002

IGF-I, insulin-like growth factor 1.

Normal range: provided by DSL kit.

Table 3
Growth Hormone Stimulation by L-Dopa Test
jkse-21-40-i003
Table 4
Growth Hormone Stimulation by Insulin-Induced Hypoglycemia
jkse-21-40-i004
Table 5
Bone Ages in Simple X-rays
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Table 6
Height Velocity before and after Growth Hormone Treatment
jkse-21-40-i006

*, P < 0.01 between before and after growth hormone treatment.

Table 7
No Evidence of Adverse Effects on Glucose Metabolism after Growth Hormone Treatment
jkse-21-40-i007

No significant difference between before and after growth hormone treatment.

References

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