Growth Hormone Treatment in Prader-Willi Syndrome / 대한내분비학회지

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.