Cardiovascular and metabolic risk profile and acylation-stimulating protein levels in children with Prader-Willi syndrome and effects of growth hormone treatment

J Clin Endocrinol Metab. 2010 Apr;95(4):1758-66. doi: 10.1210/jc.2009-0656. Epub 2010 Feb 19.


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/m(2) . 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.

Publication types

  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Absorptiometry, Photon
  • Aging / physiology
  • Anthropometry
  • Blood Pressure / physiology
  • Body Height / physiology
  • Cardiovascular Diseases / blood*
  • Cardiovascular Diseases / epidemiology
  • Cardiovascular Diseases / etiology*
  • Child
  • Child, Preschool
  • Chromosome Deletion
  • Complement C3
  • Dyslipidemias / blood
  • Dyslipidemias / complications
  • Female
  • Homeostasis
  • Human Growth Hormone / therapeutic use*
  • Humans
  • Infant
  • Insulin Resistance / physiology
  • Intercellular Signaling Peptides and Proteins / blood*
  • Male
  • Metabolic Diseases / blood*
  • Metabolic Diseases / epidemiology
  • Metabolic Diseases / etiology*
  • Netherlands
  • Prader-Willi Syndrome / blood*
  • Prader-Willi Syndrome / complications*
  • Prader-Willi Syndrome / epidemiology
  • Recombinant Proteins / therapeutic use
  • Risk Assessment


  • C3 protein, human
  • Complement C3
  • Intercellular Signaling Peptides and Proteins
  • Recombinant Proteins
  • Human Growth Hormone