Linear growth response to exogenous growth hormone in Prader-Willi syndrome

P. D.K. Lee, D. M. Wilson, L. Rountree, R. L. Hintz, R. G. Rosenfeld

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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 effecft of oxandrolone therapy on linear growth rate was demonstrated in one case. Our results support the possibility of a neurosecretory GH deficiency in Prader-Willi syndrome and suggest a need for further investigations.

Original languageEnglish (US)
Pages (from-to)865-871
Number of pages7
JournalAmerican Journal of Medical Genetics
Volume28
Issue number4
DOIs
StatePublished - Jan 1 1987

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ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

Cite this

Lee, P. D. K., Wilson, D. M., Rountree, L., Hintz, R. L., & Rosenfeld, R. G. (1987). Linear growth response to exogenous growth hormone in Prader-Willi syndrome. American Journal of Medical Genetics, 28(4), 865-871. https://doi.org/10.1002/ajmg.1320280411