Endocrine Abstracts

Objective: Prader-Willi syndrome (PWS) is a complex genetic disorder. Body composition abnormalities, hyperphagia, progressive obesity and diminished growth hormone secretion are common. In a previous one year GH intervention trial in 19 PWS adults, we have shown beneficial effects on body composition. In the present study we sought to re-evaluate the cohort, with special emphasis on long-term effects of GH treatment.

Methods: Fourteen patients, 7 men and 7 women, mean age 32 years, were evaluated. Mean follow-up was 6 years. Nine patients were treated with GH during a mean time of 5 years. Body composition and bone mineral density (BMD) were determined with Dual Energy X-ray absorptiometry (DEXA). Plasma glucose and serum insulin were measured

Results: Only small variations were seen in BMI, except in one woman (not treated with GH) who increased enormously in weight. In the GH treated patients a mean increase in lean body mass of 8 kg (P=0.004) and a mean decrease in body fat of 9% (P=0.017) were demonstrated. BMD was unchanged. Only one non-GH-treated woman developed overt diabetes.

Conclusion: In this cohort data show that five years of GH treatment has sustained favourable effects on body composition without significant side effects.