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Alagille Syndrome
Growth Hormone Deficiency
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Longer-Term Study Confirms Benefit With Growth Hormone in Prader-Willi Syndrome

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Short-term data suggest a positive effect of growth hormone (GH) on mental and motor development in infants and children with Prader-Willi syndrome (PWS). Recently, a Dutch study confirmed these benefits in a prospective, cohort study examining 3 years of GH treatment.

During the 3-year treatment period, mental and motor developmental were assessed using Bayleys Scales of Infant Development II (BSID-II) and expressed as % of the expected development (100%). The investigators found that mental development increased from 58.1% at baseline to 79.6% and that motor development increased from 41.9% to 78.2% (both P<.01). Lower baseline psychomotor development and younger age at start of GH treatment were associated with a higher increase in mental and motor development (P<.01).

The investigators concluded that GH treatment, particularly when started as young as possible, helped reduce the gap between infants with PWS and their non-PWS, healthy counterparts.

Read more here.

Reference

Donze SH, Damen L, Mahabier EF, Hokken-Koelega ACS. Improved mental and motor development during 3 years of GH treatment in very young children with Prader-Willi syndrome. J Clin Endocrinol Metab. 2018 Aug 2. doi: 10.1210/jc.2018-00687.

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