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Growth charts for Thai children with Prader-Willi syndrome aged 0-18 years
Nantiya Mongkollarp1, Thipwimol Tim-Aroon2, Chusak Okascharoen3
1Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Insights
This study presents the first growth charts for Thai children with Prader-Willi syndrome (PWS), a genetic disorder. These charts aid in monitoring PWS patient growth and understanding ethnic variations.
Area of Science:
- Genetics
- Pediatrics
- Endocrinology
Background:
- Prader-Willi syndrome (PWS) is a genetic disorder characterized by distinct eating behaviors and growth patterns.
- Infants with PWS typically have low birth weight, followed by hyperphagia later in childhood.
- Existing literature indicates ethnic variations in growth among individuals with PWS.
Purpose of the Study:
- To develop specific growth charts for Thai children diagnosed with Prader-Willi syndrome.
- To provide a tool for monitoring growth in this population.
- To compare growth patterns of Thai PWS patients with other ethnic groups.
Main Methods:
- A retrospective multicenter study involving 113 genetically confirmed PWS patients in Thailand (2000-2017).
- Growth data from non-growth hormone treated patients (ages 0-18) were analyzed.
- Growth charts were constructed using Generalized Least Squares (GLS) methods and smoothed with Fractional Polynomials and Exponential Transformation.
Main Results:
- Developed growth charts for non-growth hormone treated Thai children with PWS (ages 0-18).
- Thai PWS patients exhibited a similar growth pattern to other ethnicities, with some notable differences.
- Mean birth weight was lower than typical newborns; mean adult height was lower than American but taller than Japanese PWS patients.
Conclusions:
- This study introduces the first PWS-specific growth charts for a Southeast Asian population.
- These charts are valuable for enhancing patient care and assessing future growth hormone treatment efficacy.
- Findings highlight ethnic variations in PWS growth, necessitating population-specific monitoring tools.
Background:
Prader-Willi syndrome (PWS) is a multisystem genetic disorder, which has a typical eating behavior and growth pattern. In the infancy period, children with PWS have low body weight followed by hyperphagia in later childhood. Disease-specific growth charts have been recommended for monitoring PWS patients. Previous literature demonstrated growth differences among individuals with PWS of different ethnicity.
Methods:
A retrospective multicenter study was performed in PWS patients from different areas of Thailand included collaboration with the Thai PWS support group during 2000-2017. Baseline characteristics and anthropometric data were reviewed. Both growth hormone and non-growth hormone received patients were included, but the data after receiving GH were excluded before curve construction. Growth charts for Thai PWS compared to the 50th normative centile were constructed using Generalized Least Squares (GLS) methods. Curve smoothing was performed by Fractional Polynomials and Exponential Transformation.
Result:
One hundred and thirteen patients with genetically confirmed PWS (55 males and 58 females) were enrolled. Fifty percent of patients were diagnosed less than 6 months of age. We developed growth charts for non-growth hormone treated Thai children with PWS aged between 0 and 18 years. A growth pattern was similar to other ethnicities while there were some differences. Mean birth weight of PWS patients was less than that of typical newborns. Mean adult height at 18 years of age in Thai children with PWS was lower than that in American children, but taller than Japanese. Mean weight of Thai PWS males at 18 years of age was more than those from other countries.
Conclusion:
This study is the first to document PWS-specific growth charts in Southeast Asian population. These growth charts will be useful in improving the quality of patient care and in evaluating the impact of growth hormone treatment in the future.
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