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Young children with Prader-Willi syndrome have unfavorable CV, metabolic risk profile

Issue: October 2009

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LWPES/ESPE 8th Joint Meeting

Sixty-three percent of infants and 73% of prepubertal children with Prader-Willi syndrome have at least one cardiovascular risk factor for metabolic syndrome, according to data from two Dutch studies.

The Dutch Growth Hormone Study (n=85) results indicate that 5% of children with Prader-Willi syndrome have metabolic syndrome, and 40% had at least one component of the syndrome.

The study assessed the CV and metabolic risk profile in children with Prader-Willi syndrome (mean age, 4.9 years) after two years of daily growth hormone (1 mg/m²).

Ninety-five percent of children had an elevated fat percentage. Two years of GH treatment improved fat percentage SDS (P<.0001) and HDL–LDL ratio (P=.04).

Follow-up data from the Dutch Cohort Study (n=55) reveal that the effects of GH treatment were sustained during four years. Children had lower fat percentage SDS (P<.0001) and lean body mass SDS that increased in year one and then remained unchanged. Mean height SDS normalized from –2.27 to –0.24. The researchers reported improved head circumference SDS (0.07) and increased insulin-like growth factor-1 (2.08) and IGF-1/IGFBP-3 ratio (2.32).

GH had no effect on bone maturation, blood pressure, glucose homeostasis or serum lipids, according to data from both trials.

“Based on the results of four years of follow-up in the Dutch Cohort Study showing a favorable effect of GH without adverse effects, long-term continuous GH treatment can be considered an effective, safe therapy for Prader-Willi syndrome children,” Roderick F. A. de Lind van Wijngaarden, MSc, PhD, department of pediatrics, Erasmus University Medical Center Rotterdam/Sophia Children’s Hospital, Netherlands, told Endocrine Today. – by Katie Kalvaitis

For more information:

• De Lind van Wijngaarden RFA. FC10-001. Presented at: LWPES/ESPE 8th Joint Meeting; September 9-12, 2009; New York.

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