Linear growth response to exogenous growth hormone in Prader-Willi syndrome.
Linear growth retardation and adult short stature are usual characteristics of Prader-Willi syndrome. Several lines of evidence suggest that a deficiency in growth hormone (GH) secretion may contribute to this abnormal growth pattern. We have recently reported observations in 4 children with Prader-Willi syndrome treated with GH. This report extends our observations in 2 of these cases. Both cases had abnormally low growth rate, normal stimulated GH levels, and low somatomedin-C levels prior to therapy. GH treatment led to significant increases in linear growth rate and somatomedin-C levels. An additive effect of oxandrolone therapy on linear growth rate was demonstrated in one case. Our results support the possibility of a neuro-secretory GH deficiency in Prader-Willi syndrome and suggest a need for further investigations.
Lee
P D
PD
Department of Pediatrics, Children's Hospital, Denver, Colorado 80218-1088.
Wilson
D M
DM
Rountree
L
L
Hintz
R L
RL
Rosenfeld
R G
RG
D001827:Body Height / Q000187:drug effects*; D002648:Child; D004334:Drug Administration Schedule; D005260:Female; D013006:Growth Hormone / Q000008:administration & dosage* / Q000097:blood; D006801:Humans; D008297:Male; D011218:Prader-Willi Syndrome / Q000503:physiopathology*; D011994:Recombinant Proteins / Q000008:administration & dosage
D011994:Recombinant Proteins; D013006:Growth Hormone