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


OBJECTIVE
The aim was to study the metabolic and cardiovascular risk profile and ASP levels and to investigate the effects of GH treatment.


METHODS
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.


METHODS
Eighty-five children with PWS (mean +/- sd age of 4.9 +/- 3.0 yr) participated in the study.


METHODS
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.
de Lind van Wijngaarden
Roderick F A
RF
Dutch Growth Research Foundation, 3016 AH Rotterdam, The Netherlands. r.delindvanwijngaarden@erasmusmc.nl
Cianflone
Katherine
K
Gao
Y
Y
Leunissen
Ralph W J
RW
Hokken-Koelega
Anita C S
AC
D015502:Absorptiometry, Photon; D000375:Aging / Q000502:physiology; D000886:Anthropometry; D001794:Blood Pressure / Q000502:physiology; D001827:Body Height / Q000502:physiology; D002318:Cardiovascular Diseases / Q000097:blood* / Q000453:epidemiology / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D003176:Complement C3; D050171:Dyslipidemias / Q000097:blood / Q000150:complications; D005260:Female; D006706:Homeostasis; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D007333:Insulin Resistance / Q000502:physiology; D036341:Intercellular Signaling Peptides and Proteins / Q000097:blood*; D008297:Male; D008659:Metabolic Diseases / Q000097:blood* / Q000453:epidemiology / Q000209:etiology*; D009426:Netherlands; D011218:Prader-Willi Syndrome / Q000097:blood* / Q000150:complications* / Q000453:epidemiology; D011994:Recombinant Proteins / Q000627:therapeutic use; D018570:Risk Assessment
C038245:C3 protein, human; D003176:Complement C3; D036341:Intercellular Signaling Peptides and Proteins; D011994:Recombinant Proteins; D019382:Human Growth Hormone