Abstract
BackgroundPrader-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.MethodsA 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.ResultOne 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.ConclusionThis 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.
Highlights
Prader-Willi syndrome (PWS) is a multisystem genetic disorder, which has a typical eating behavior and growth pattern
Prader-Willi Syndrome (PWS) is a multisystem genetic disorder presenting with infantile hypotonia, unique eating habits and growth patterns, short stature, typical facial dysmorphism, small hands and feet, intellectual disability, behavioral abnormalities, and hypogonadism
The early diagnosed patients mostly presented with poor feeding and hypotonia
Summary
Prader-Willi syndrome (PWS) is a multisystem genetic disorder, which has a typical eating behavior and growth pattern. 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. Prader-Willi Syndrome (PWS) is a multisystem genetic disorder presenting with infantile hypotonia, unique eating habits and growth patterns, short stature, typical facial dysmorphism, small hands and feet, intellectual disability, behavioral abnormalities, and hypogonadism. Hypotonia and failure to thrive are noted in phase 1a during infancy period. During phase 1b (9-month to 2-year-old), patients can improve weight gain with normal growth velocity. At 2–4 years of age (phase 2a), children with PWS
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