Context: Reports on the cardiovascular and metabolic risk profile in children with Prader-Willi syndrome (PWS) and the effects of GH treatment are scarce. Acylation-stimulating protein (ASP) stimulates glucose uptake and triglyceride storage in adipose tissue. Objectives: The aim was to study the metabolic and cardiovascular risk profile and ASP levels and to investigate the effects of GH treatment. Design: We conducted a randomized controlled GH trial. Infants and prepubertal children were assigned to receive GH (1 mg/m2·d) or to serve as controls for 12 and 24 months, respectively. Patients: Eighty-five children with PWS (mean ± SD age of 4.9 ± 3.0 yr) participated in the study. Main Outcome Measures: We measured fat percentage (fat%) with dual-energy x-ray absorptiometry, blood pressure, fasting insulin and glucose levels, serum lipids, and ASP levels. Results: Mean ± SD fat% was 28.4 ± 6.2 in infants and 36.9 ± 8.5 in prepubertal children. Fat% SD score (SDS) was above 2 SDS in 95% of prepubertal children. In addition, 63% of infants and 73% of prepubertal children demonstrated at least one cardiovascular risk factor, defined as hypertension or dyslipidemia. The metabolic syndrome was demonstrated in 5% of all children. Mean ± SD baseline ASP was 107 ± 45 nmol/liter (normal < 58 nmol/liter) and correlated with fat mass and TG levels. GH improved fat%SDS and the HDLc/LDLc ratio (P < 0.0001 and P = 0.04). GH had no effect on mean ASP levels in this population. Conclusions: Many children with PWS had dyslipidemia and high ASP levels. GH improved fat% and high-density lipoprotein cholesterol/low-density lipoprotein cholesterol, but not ASP. High ASP levels may prevent complete normalization of fat%SDS during GH treatment but may contribute in keeping glucose and insulin levels within normal range. Copyright

dx.doi.org/10.1210/jc.2009-0656, hdl.handle.net/1765/27659
Journal of Clinical Endocrinology and Metabolism
Free full text at PubMed
Erasmus MC: University Medical Center Rotterdam

de Lind van Wijngaarden, R.F.A, Cianflone, K, Gao, Y, Leunissen, R.W.J, & Hokken-Koelega, A.C.S. (2010). Cardiovascular and metabolic risk profile and acylation-stimulating protein levels in children with Prader-Willi syndrome and effects of growth hormone treatment. Journal of Clinical Endocrinology and Metabolism, 95(4), 1758–1766. doi:10.1210/jc.2009-0656