Related Experiment Videos
Linear growth response to exogenous growth hormone in Prader-Willi syndrome
P D Lee1, D M Wilson, L Rountree
1Department of Pediatrics, Children's Hospital, Denver, Colorado 80218-1088.
Insights
Prader-Willi syndrome often causes short stature due to growth hormone (GH) issues. GH therapy improved linear growth and somatomedin-C levels in children with this condition.
Area of Science:
- Pediatric Endocrinology
- Genetics
- Growth Disorders
Background:
- Prader-Willi syndrome is characterized by linear growth retardation and short stature.
- A potential deficiency in growth hormone (GH) secretion is implicated in this abnormal growth pattern.
Observation:
- Two children with Prader-Willi syndrome, previously reported, were observed.
- Both cases exhibited slow linear growth, normal stimulated GH levels, and low somatomedin-C levels before treatment.
Findings:
- Growth hormone (GH) treatment significantly increased linear growth rates in both patients.
- Somatomedin-C levels also showed significant increases following GH therapy.
- Oxandrolone therapy demonstrated an additive effect on linear growth rate in one patient.
Implications:
- The findings support the hypothesis of a neuro-secretory GH deficiency in Prader-Willi syndrome.
- Further research is warranted to investigate the role of GH secretion in Prader-Willi syndrome.
- These results may inform future therapeutic strategies for growth impairment in Prader-Willi syndrome.
Abstract:
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.