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Growth hormone secretion decreases with age in paediatric Prader-Willi syndrome

M Cohen1, J Harrington1, I Narang2

  • 1Division of Endocrinology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Clinical Endocrinology
|December 16, 2014
PubMed

Insights

Growth hormone (GH) deficiency is common in Prader-Willi syndrome (PWS). GH deficiency is less prevalent in infants under 18 months, suggesting careful age-based interpretation of GH stimulation tests is needed for PWS patients.

Area of Science:

  • Pediatric Endocrinology
  • Genetics and Genetic Diseases
  • Metabolic Disorders

Background:

  • Growth hormone (GH) deficiency is a frequent characteristic of Prader-Willi syndrome (PWS).
  • Biochemical confirmation of GH deficiency in PWS is not always consistent.
  • Current criteria for initiating GH treatment in pediatric PWS vary, with some nations requiring documented biochemical GH deficiency.

Purpose of the Study:

  • To investigate age-related patterns in the prevalence of biochemical GH deficiency in infants and children diagnosed with PWS.
  • To evaluate the significance of age in interpreting GH stimulation test results, particularly in the infant population with PWS.

Main Methods:

  • A retrospective analysis of patient charts was performed.
  • Data were collected from children diagnosed with PWS who underwent GH stimulation testing between 2000 and 2012 at a major pediatric hospital.
  • The study reviewed charts of 47 children aged 0.4 to 15.5 years with PWS, assessing biochemical GH status relative to age and body mass index (BMI).

Main Results:

  • Of the 47 patients, 32 (68%) exhibited biochemical GH deficiency.
  • GH deficiency showed a significant correlation with older age (r=0.45, P=0.02) and higher BMI z-scores (r=0.45, P=0.02).
  • Biochemical GH deficiency was notably less prevalent in children under 18 months (27%) compared to older children (81%; P=0.001), and more prevalent in obese patients (88%) versus non-obese patients (58%; P=0.04).

Conclusions:

  • The diagnostic value of GH stimulation tests for assessing GH status in Prader-Willi syndrome patients under 18 months of age is questionable.
  • Results from GH stimulation tests in young PWS patients should be interpreted with careful consideration of the patient's age.
  • Age is a critical factor in the interpretation of GH stimulation test results for pediatric PWS.
Abstract

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