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Endocrine dysfunction in Prader-Willi syndrome: a review with special reference to GH

P Burman1, E M Ritzén, A C Lindgren

  • 1Pharmacia Corporation, SE-112 87 Stockholm, Sweden. pia.burman@eu.pnu.com

Endocrine Reviews
|December 12, 2001
PubMed

Insights

Prader-Willi syndrome, a genetic disorder, is linked to growth hormone (GH) deficiency. GH replacement therapy shows promise for improving growth and body composition in affected children.

Area of Science:

  • Endocrinology
  • Genetics
  • Pediatrics

Background:

  • Prader-Willi syndrome (PWS) is a rare genetic disorder affecting 1 in 10,000-16,000 infants.
  • Key features include short stature, hypotonia, developmental delay, behavioral issues, and hyperphagia leading to obesity.
  • PWS is often associated with hypothalamic-pituitary dysfunction, specifically growth hormone (GH) deficiency and hypogonadotropic hypogonadism.

Purpose of the Study:

  • To evaluate the efficacy and benefits of growth hormone (GH) replacement therapy in children with Prader-Willi syndrome.
  • To explore the potential role of sex hormone replacement therapy in managing PWS.

Main Methods:

  • Review of clinical trials evaluating GH replacement therapy in children with PWS.
  • Analysis of existing data on sex hormone replacement therapy in PWS patients.

Main Results:

  • GH treatment demonstrated encouraging results, including improved linear growth and increased lean body mass.
  • GH therapy also showed potential benefits in reducing fat mass, improving respiratory function, and enhancing physical activity.
  • Limited data exist on the efficacy and long-term benefits of sex hormone replacement therapy.

Conclusions:

  • Prader-Willi syndrome is a significant condition characterized by GH deficiency and hypogonadism.
  • Active treatment of these endocrine abnormalities, particularly GH replacement, is likely to benefit affected individuals.
  • Further research is needed to establish the long-term benefits of GH therapy and clarify the role of sex hormone replacement.

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