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Growth Charts for Prader-Willi Syndrome During Growth Hormone Treatment
Merlin G Butler1, Jaehoon Lee2, Devin M Cox3
1University of Kansas Medical Center, Kansas City, KS, USA mbutler4@kumc.edu.
Insights
Standardized growth curves were developed for children with Prader-Willi syndrome (PWS) undergoing growth hormone treatment. These new charts show growth hormone therapy improves height and weight in PWS individuals.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder affecting multiple body systems, often leading to growth abnormalities.
- Growth hormone deficiency is common in PWS, necessitating therapeutic interventions to optimize growth and development.
- Existing growth charts may not accurately reflect growth patterns in PWS individuals receiving growth hormone treatment.
Purpose of the Study:
- To develop syndrome-specific, standardized growth curves for individuals with Prader-Willi syndrome (PWS) aged 0-18 years receiving growth hormone (GH) therapy.
- To provide clinicians with accurate tools for monitoring growth in GH-treated PWS patients.
- To compare growth patterns in GH-treated PWS individuals against non-GH-treated PWS populations.
Main Methods:
- Development of syndrome-specific growth curves using the LMS method for 171 PWS subjects (0-18 years) treated with GH.
- Collection of anthropometric data including weight, height, head circumference, weight/length, and BMI.
- Comparison with normative data and growth charts for non-GH-treated PWS individuals.
Main Results:
- Standardized growth curves for PWS patients treated with growth hormone were established for 7 percentile ranges.
- Growth hormone treatment demonstrated a normalization of stature and significant improvement in weight compared to non-GH-treated PWS individuals.
- The developed charts allow for better tracking of growth trajectories in GH-treated PWS patients.
Conclusions:
- Syndrome-specific growth charts are essential for accurately assessing growth in children with PWS receiving growth hormone therapy.
- Growth hormone treatment significantly improves key growth parameters, including height and weight, in individuals with PWS.
- These standardized growth curves provide valuable clinical guidance for managing GH therapy in PWS.
Abstract:
The purpose of the current study was to develop syndrome-specific standardized growth curves for growth hormone-treated Prader-Willi syndrome (PWS) individuals aged 0 to 18 years. Anthropometric growth-related measures were obtained on 171 subjects with PWS who were treated with growth hormone for at least 40% of their lifespan. They had no history of scoliosis. PWS standardized growth curves were developed for 7 percentile ranges using the LMS method for weight, height, head circumference, weight/length, and BMI along with normative 3rd, 50th, and 97th percentiles plotted using control data from the literature and growth databases. Percentiles were plotted on growth charts for comparison purposes. Growth hormone treatment appears to normalize stature and markedly improves weight in PWS compared with standardized curves for non-growth hormone-treated PWS individuals. Growth chart implications and recommended usage are discussed.
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