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Roderick F. A. de Lind van Wijngaarden, Luuk W. L. de Klerk, Dederieke
A. M. Festen, Hugo J. Duivenvoorden, Barto J. Otten, and Anita C. S.
Hokken-Koelega
J. Clin. Endocrinol. Metab., Apr 2009; 94: 1274 - 1280.
The purpose of this trial was to study the effects of growth hormone (GH)
treatment on the onset of scoliosis and curve progression in children with
Prader-Willi syndrome (PWS). Prader-Willi syndrome is a congenital disease that
involves obesity, decreased muscle tone, decreased mental capacity, and sex
glands that produce little or no hormones. There is a 30–80% prevalence of
scoliosis in children with PWS, depending on age. Scoliosis is generally
considered a contraindication for GH treatment.
This multicenter,
randomized, controlled study included 91 children with PWS (infants, prepubertal
and pubertal children). . Infants and prepubertal children were randomized into
a group that received treatment with growth hormone (1.0 mg/m2 · d) and a
control group for 1 and 2 yr, respectively. Pubertal children were randomized to
receive GH at doses of 1.0 mg/m2 · d or 1.5 mg/m2 · d. Yearly, x-rays of the
spine were taken, and height, weight, truncal lean body mass, and IGF-I were
measured. The study measured the onset of scoliosis and scoliotic curve
progression in all of the participants.
Growth hormone treated children
had similar onset of scoliosis and curve progression as randomized controls. GH
treatment, IGF-I standard deviation score (SDS), and catch-up growth had no
adverse effect on the onset of scoliosis or curve progression. Height SDS,
truncal lean body mass, and IGF-I SDS were significantly higher in GH-treated
children than in randomized controls. At baseline, a higher IGF-I SDS was
associated with a lower severity of scoliosis.
The researchers concluded
that scoliosis should no longer be considered a contraindication for GH
treatment in children with PWS.
