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Published on: August 6, 2018
Growth in treated classical galactosemia patients
Bianca Panis1, Willem-Jan M Gerver, M Estela Rubio-Gozalbo
1Department of Pediatrics, University Hospital Maastricht, 6202 AZ, Maastricht, The Netherlands.
European Journal of Pediatrics
|October 7, 2006
Summary
Children with classical galactosemia experience normal prenatal growth but affected postnatal growth. Final height is often below target, potentially influenced by growth factors and hormonal levels, especially in girls.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Growth and Development
Background:
- Classical galactosemia is a metabolic disorder requiring dietary management.
- Growth abnormalities, including decreased height and weight, have been anecdotally reported in treated children.
- Comprehensive growth studies in this population are limited.
Purpose of the Study:
- To investigate prenatal and postnatal growth patterns in children and adolescents with classical galactosemia.
- To identify factors influencing growth, such as growth velocities, IGF-I, IGFBP-3, and enzyme activity.
- To compare achieved growth with target height.
Main Methods:
- Retrospective analysis of growth data (length, weight, head circumference) from 40 children with classical galactosemia.
- Calculation of prenatal growth parameters and postnatal growth velocities.
- Linear regression analysis correlating growth parameters with biochemical markers (IGF-I, IGFBP-3, galactose-1-phosphate-uridyltransferase activity) and dietary intake.
Main Results:
- Prenatal growth (length, weight, head circumference) was within normal limits.
- Postnatal height and weight growth velocities were variable, with girls showing a trend towards slower growth.
- Mean height corrected for target height was below target, particularly in girls. Height growth velocity correlated with IGF-I, IGFBP-3, and corrected height Z-scores.
Conclusions:
- Prenatal growth in classical galactosemia is typically normal, but postnatal growth is frequently affected.
- Most patients do not reach their target height, though some may achieve it if growth extends beyond 18 years.
- Reduced levels of IGF-I and IGFBP-3, and potentially suboptimal hormonal therapy in girls, may contribute to impaired growth.
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