Growth trajectories of children and adolescents with osteogenesis imperfecta in a reference center in Rio de janeiro,
Luísa Cunha Pereira1, Dayana Rodrigues Farias2, Fernanda De Bastiani3
1National Institute of Women, Children and Adolescents' Health Fernandes Figueira, Department of Nutrition, Oswaldo Cruz Foundation, Rio de Janeiro, RJ, Brazil.
Insights
Growth monitoring for children with Osteogenesis Imperfecta (OI) requires specific charts. This study developed Brazilian OI growth charts, revealing compromised anthropometric measures necessitating tailored nutritional evaluation.
Area of Science:
- Pediatrics
- Genetics
- Nutrition
Background:
- Osteogenesis Imperfecta (OI) is a rare genetic disorder impacting collagen, essential for bone and connective tissue.
- Growth monitoring in children with OI is vital for detecting comorbidities and managing stature deficits.
- Specific growth charts are needed to sensitively track changes and identify issues in OI patients.
Purpose of the Study:
- To evaluate weight, height, and BMI trajectories in Brazilian patients with Osteogenesis Imperfecta (OI).
- To propose specific growth charts tailored for Brazilian children and adolescents with OI.
Main Methods:
- Collected weight and height data from birth to 19 years for 237 Brazilian OI patients.
- Utilized GAMLSS statistical package (R software) to generate gender and OI subtype-stratified growth charts.
- Compared generated charts with WHO standards and analyzed growth-associated factors using linear mixed effects regression.
Main Results:
- Factors influencing growth included gender, OI subtype, pamidronate use, and rod placement.
- Type I OI showed deficits compared to WHO standards; Types III and IV had significantly lower weight and height trajectories after age 2 and throughout the period, respectively.
- Height was more impaired than weight, resulting in BMI charts closer to WHO standards, sometimes exceeding them in severe phenotypes.
Conclusions:
- Children and adolescents with OI exhibit compromised anthropometric measures.
- Nutritional evaluation for OI patients must utilize specific growth charts.
- These developed charts serve as a crucial reference for the Brazilian population, aiding clinical evaluation.
Background:
Osteogenesis Imperfecta (OI) is a rare genetic disorder affecting collagen production, crucial for bone and connective tissue. Monitoring growth in children with OI is essential for detecting comorbidities. Despite common stature deficits, specific growth charts are crucial for sensitively tracking changes and identifying additional issues in this population. The aim of this study is to evaluate weight, height and BMI trajectories in Brazilian patients with Osteogenesis Imperfecta (OI), proposing specific growth charts.
Methods:
Weight and height repeated measures from birth to 19 years old were collected from medical records of all patients with OI treated in a reference center in Rio de Janeiro, Brazil. The GAMLSS statistical package (R software) was used to generate charts of weight-for-age, height-for-age and BMI-for-age, stratified for gender and OI subtype. Charts were compared to the World Health Organization (WHO) standards. The association with other variables was investigated using linear mixed effects regression.
Results:
237 participants were included (type I = 134; type III = 41, type IV = 57; type V = 5). Factors associated with growth were: gender, OI subtype, pamidronate use, and rod placement. In type I, weight and height trajectories followed the pattern of WHO standards, but with a deficit in both genders. The growth of types III and IV was significantly lower compared to type I only after 2 years old for weight and all over the period for height. In general, there is greater impairment of height than weight, so the BMI chart is closer to the WHO standard, sometimes even surpassing it, especially in more severe phenotypes.
Conclusion:
Children and adolescents with OI have compromised anthropometric measures and their nutritional evaluation must be done by specific growth charts. This work is an important step towards facilitating the clinical evaluation of these patients and can be used as reference for Brazilian population.
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