4.7 Article

Growth Charts for Non-Growth Hormone Treated Prader-Willi Syndrome

Journal

PEDIATRICS
Volume 135, Issue 1, Pages E126-E135

Publisher

AMER ACAD PEDIATRICS
DOI: 10.1542/peds.2014-1711

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Funding

  1. Prader-Willi Syndrome Association (USA)
  2. Angelman, Rett and Prader-Willi Syndromes Consortium, National Institutes of Health Rare Diseases Clinical Research Network [U54 HD06122]
  3. National Institutes of Health (NIH)

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OBJECTIVE: The goal of this study was to generate and report standardized growth curves for weight, height, head circumference, and BMI for non-growth hormone-treated white male and female US subjects with Prader-Willi syndrome (PWS) between 3 and 18 years of age and develop standardized growth charts. METHODS: Anthropometric measures (N = 133) were obtained according to standard methods from 120 non-growth hormone-treated white subjects (63 males and 57 females) with PWS between 3 and 18 years of age. Standardized growth curves were developed for the third, 10th, 25th, 50th, 75th, 90th, and 97th percentiles by using the LMS method for weight, height, head circumference, and BMI for PWS subjects along with the normative third, 50th, and 97th percentiles from national and international growth data. The LMS smoothing procedure summarized the distribution of the anthropometric variables at each age using three parameters: power of the Box-Cox transformation lambda (L), median mu (M) and coefficient of variation delta (S). RESULTS: Weight, height, head circumference, and BMI standardized growth charts representing 7 percentile ranges were developed from 120 non-growth hormone-treated white male and female US subjects with PWS (age range: 3-18 years) and normative third, 50th, and 97th percentiles from national and international data. CONCLUSIONS: We encourage the use of syndrome-specific growth standards to examine and evaluate subjects with PWS when monitoring growth patterns and determining nutritional and obesity status. These variables can be influenced by culture, individual medical care, diet intervention, and physical activity plans.

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