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Disease-specific growth charts for Korean infants with Prader-Willi syndrome
Jieun Lee1, Tsuyoshi Isojima, Mi Sun Chang
1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Insights
Growth charts specific to Prader-Willi syndrome (PWS) are essential for accurately assessing infants. These new charts help monitor growth patterns and nutritional status in young children with PWS.
Area of Science:
- Pediatrics
- Genetics
- Endocrinology
Background:
- Prader-Willi syndrome (PWS) is characterized by distinct growth patterns, including short stature and obesity.
- Standard growth charts are inadequate for evaluating the growth of children with PWS due to their unique physiology.
- Accurate growth assessment is crucial for managing PWS and guiding treatment decisions.
Purpose of the Study:
- To develop disease-specific growth charts for height and weight in non-growth hormone-treated Korean infants with PWS.
- To provide a reliable tool for evaluating and managing growth in infants diagnosed with PWS.
- To establish reference data for monitoring growth trajectories in this population.
Main Methods:
- A retrospective review of medical records from 122 genetically confirmed Korean infants with PWS.
- Collection of height and weight data prior to growth hormone (GH) treatment.
- Generation of disease-specific growth charts using the LMS smoothing procedure to calculate centiles (3rd to 97th).
Main Results:
- Disease-specific growth charts for height and weight were successfully generated for Korean infants with PWS (0-36 months).
- The charts provide reference centiles (3rd, 10th, 25th, 50th, 75th, 90th, 97th) for growth assessment.
- These charts enable accurate evaluation of growth status in infants with PWS.
Conclusions:
- The developed disease-specific growth charts are vital for the clinical evaluation of Korean infants with PWS.
- These charts facilitate monitoring of growth patterns, nutritional status, and response to potential GH treatment.
- Utilizing these specialized charts ensures appropriate management and care for children with Prader-Willi syndrome.
Abstract:
Patients with Prader-Willi syndrome (PWS) present with short stature and obesity. The growth pattern of children with PWS is different from that of the healthy population. Therefore, it is not appropriate to use normal growth charts to evaluate the growth status of children with PWS. We aimed to develop disease-specific growth charts for height and weight for nongrowth hormone-treated Korean infants with PWS aged between 0 and 36 months and to use these growth charts for the evaluation and management of infants with PWS. We conducted a retrospective review of the medical records of 122 infants with genetically confirmed PWS. Data on the patients' height and weight measurements before they underwent growth hormone treatment were recorded. Disease-specific growth charts were generated and the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th centiles were calculated using the LMS (refers to λ, μ, and σ, respectively) smoothing procedure for height and weight. The disease-specific growth charts for Korean infants with PWS can be used when examining infants with PWS and when evaluating their growth at later stages for comparison purposes. They are also useful for monitoring growth patterns, nutritional assessments, and recording responses to growth hormone treatment.
