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Longitudinal evaluation of growth, puberty, and bone maturation in children with Williams syndrome
C J Partsch1, G Dreyer, A Gosch
1Department of Pediatrics, Christian-Albrechts-University, Kiel, Germany.
Insights
Children with Williams syndrome (WS) exhibit distinct growth patterns, including slower early growth and a premature pubertal spurt. This study defines syndrome-specific growth curves for WS, aiding in understanding their unique development.
Area of Science:
- Pediatric Endocrinology
- Human Genetics
- Growth and Development
Background:
- Williams syndrome (WS) is a genetic disorder associated with characteristic facial features, cardiovascular problems, and developmental delays.
- Growth patterns in children with WS are not well-defined, necessitating syndrome-specific growth charts.
Purpose of the Study:
- To establish syndrome-specific growth rate (GR) curves for children with Williams syndrome.
- To characterize bone maturation and pubertal development patterns in WS.
Main Methods:
- A prospective longitudinal study collected growth data from 244 children with WS between 1990 and 1997.
- Growth rate (GR) values were calculated from collected data for both girls and boys.
Main Results:
- Children with WS showed below-normal mean GR in early childhood.
- A premature pubertal growth spurt was observed in girls (at 9 or 11 years) and boys (at 11-12 years).
- Bone age was delayed in childhood and accelerated during puberty, with final heights differing between sexes.
Conclusions:
- Syndrome-specific GR curves for WS reveal a premature and abbreviated pubertal growth spurt in both sexes.
- The observed growth spurt correlates with bone age acceleration during puberty.
- This longitudinal data provides insights into growth dynamics and progression in children with WS.
Objectives:
To establish syndrome-specific growth curves and growth rate (GR) curves for Williams syndrome (WS) and define the pattern of bone maturation and pubertal development.
Methods:
In a prospective longitudinal study between 1990 and 1997, the growth data of 244 children with WS were collected: 295 values for GR were calculated for 74 girls and 331 values for 89 boys.
Results:
Mean GR of children with WS was below normal by 1 to 2 cm/y in the first few years of life. One group of girls (n = 20) experienced an early pubertal growth spurt at age 9 years (maximal GR, 7.8 +/- 2.1 cm/y; menarcheal age, 10.4 +/- 1.4 years). A second group (n = 5) showed the growth spurt at age 11 years (7.5 +/- 1.1 cm/y; menarcheal age, 12.6 +/- 1.3 years). In boys, peak height velocity (8.7 +/- 2.3 cm/y) occurred at age 11 to 12 years. Bone age was delayed in both sexes during childhood and accelerated markedly during puberty. Final height was 152.4 +/- 5.7 cm in girls (n = 38) and 165.2 +/- 10. 9 cm in boys (n = 43).
Conclusions:
The syndrome-specific GR curves for WS showed a premature and abbreviated pubertal growth spurt in both sexes. This growth spurt was directly related to bone age acceleration during puberty. The data from this longitudinal study provide an overview of both the dynamics of growth and its course in children with WS.
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