Growth hormone normalises height, prediction of final height and hand length in children with Prader-Willi syndrome after 4 years of therapy.
BACKGROUND
Based on the reported favourable effects of growth hormone (GH) treatment on growth and body composition in Prader-Labhart-Willi syndrome, we studied age dependency and the long-term effects on growth dynamics to elucidate the assumed hypothalamic GH deficiency.


METHODS
We examined 23 children treated with hGH (24 U/m(2)/week) during a median of 4 (range 1.5-5.5) years; group 1: 10 young underweight (age 0.3-4.1 years), group 2: 8 prepubertal overweight (age 3.7-9.5 years) and group 3: 5 pubertal overweight children (age 9.0-14.6 years).


RESULTS
After 4 years of therapy, height gain amounted to 1.8 SD; height (0.0 SD) and hand length (-0.2 SD) were normalised in the 2 prepubertal groups; in children above 6 years, height prediction approached parental target height. Weight for height rose in group 1 (to 0.64 SD) and decreased in group 2 (to 0.71 SD) to normal levels. Bone maturation of the pubertal children was too advanced to show a clear growth response to GH (height gain 0.42 SD). Even in this group, weight for height was reduced, but remained supernormal.


CONCLUSIONS
Under exogenous GH, growth and body proportions are normalised in prepubertal children. With early institution of treatment, final height prediction reaches the parental target height range after 3 years. Such a growth-promoting effect of exogenous GH has so far only been described in children with GH deficiency.
Eiholzer
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U
Foundation Growth Puberty Adolescence, Zurich, Switzerland.
l'Allemand
D
D
D001786:Blood Glucose / Q000032:analysis; D001827:Body Height; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D006442:Glycated Hemoglobin / Q000032:analysis; D006225:Hand / Q000033:anatomy & histology*; D019382:Human Growth Hormone / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D011218:Prader-Willi Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D013997:Time Factors
D001786:Blood Glucose; D006442:Glycated Hemoglobin; D019382:Human Growth Hormone