Developmental charts for children with osteogenesis imperfecta, type I (body height, body weight and BMI)
Krzysztof Graff1, Malgorzata Syczewska2
1Department of Rehabilitation, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warszawa, Poland.
Insights
Children with osteogenesis imperfecta type I (OI) are shorter than their peers from birth. Their growth slows after age 8, resulting in impaired adult height. This study provides developmental charts for OI type I children.
Area of Science:
- Pediatrics
- Genetics
- Orthopedics
Background:
- Osteogenesis imperfecta (OI) type I is a common, non-deforming genetic collagen disorder.
- Previous literature suggests normal or slightly reduced stature in OI type I, but lacks supporting data.
- Characterized by blue sclera and vertebral fractures, potentially causing mild scoliosis.
Purpose of the Study:
- To create developmental charts for body height, weight, and BMI in children with OI type I.
- To provide empirical data on the growth patterns of children with OI type I.
Main Methods:
- Utilized anthropometric data from 117 patients (61 boys, 56 girls) with OI type I.
- Pooled 823 measurements into a comprehensive database.
- Employed reverse transformation to address data limitations in specific age and gender groups.
Main Results:
- Children with OI type I are smaller than healthy peers from ages 2-3.
- A slight catch-up growth occurs between ages 4-7.
- Growth velocity decreases after age 8, leading to a median adult height SDS of -2.7.
Conclusions:
- Children with OI type I exhibit reduced stature from early childhood.
- Growth faltering becomes evident from age 8 onwards.
- Final adult height is significantly impaired in individuals with OI type I.
Abstract:
Osteogenesis imperfecta (OI) is a rare genetic disorder of type I collagen. Type I is the most common, which is called a non-deforming type of OI, as in this condition, there are no major bone deformities. This type is characterised by blue sclera and vertebral fractures, leading to mild scoliosis. The body height of these patients is regarded as normal, or only slightly reduced, but there are no data proving this in the literature. The aim of this study is the preparation of the developmental charts of children with OI type I. The anthropometric data of 117 patients with osteogenesis imperfecta were used in this study (61 boys and 56 girls). All measurements were pooled together into one database (823 measurements in total). To overcome the problem of the limited number of data being available in certain age classes and gender groups, the method called reverse transformation was used. The body height of the youngest children, aged 2 and 3 years, is less than that of their healthy peers. Children between 4 and 7 years old catch up slightly, but at later ages, development slows down, and in adults, the median body height shows an SDS of -2.7.
Conclusion:
These results show that children with type I OI are smaller from the beginning than their healthy counterparts, their development slows down from 8 years old, and, ultimately, their body height is impaired. What is Known: • The body height of patients with osteogenesis imperfecta type I is regarded as normal, or only slightly reduced, but in the known literature, there is no measurement data supporting this opinion. What is New: • Children with type I osteogenesis imperfecta are smaller from the beginning than their healthy counterparts, their development slows down from 8 years old and, ultimately, their final body height is impaired. • The developmental charts for the body height, body weight and BMI of children with type I osteogenesis imperfecta are shown.
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