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Bone density, fractures and the associated factors in iranian children and adolescent with Osteogenesis Imperfecta
Pooran Mohsenzade1, Anis Amirhakimi1, Naser Honar2
1Shiraz University of Medical Sciences, Shiraz, Iran.
Insights
Vitamin D deficiency is common in children with Osteogenesis Imperfecta (OI) in southern Iran. Early bisphosphonate treatment may reduce fractures, but long-term use might be detrimental.
Area of Science:
- Pediatrics
- Endocrinology
- Orthopedics
Background:
- Osteogenesis Imperfecta (OI) is a common childhood bone fragility disorder.
- Assessing bone mineral density (BMD) and Vitamin D levels in OI patients is crucial for management.
Purpose of the Study:
- To evaluate BMD and Vitamin D status in children with OI in southern Iran.
- To identify factors influencing fracture episodes in OI patients.
Main Methods:
- A case-control study involving 23 children with OI and 23 healthy controls.
- Bone Mineral Density (BMD) measured using Dual-energy X-ray absorptiometry (DXA).
- Data collected on demographics, biochemical parameters, puberty, sun exposure, and physical activity.
Main Results:
- Vitamin D deficiency was prevalent in 43.4% of OI patients.
- 56% of OI patients exhibited low bone mass for their age in the lumbar area.
- Fracture episodes were influenced by Pamidronate initiation timing, number of courses, puberty, and sun exposure.
Conclusions:
- Vitamin D deficiency is widespread in Iranian children with OI.
- Low bone mass is common in OI patients despite bisphosphonate therapy.
- Early Pamidronate initiation and sufficient courses correlate with fewer fractures, while treatment exceeding 3 years may increase fracture risk.
Backround:
Osteogenesis imperfecta(OI) is a frequent bone fragility disorder in children. The purpose of this study was to assess the BMD and Vitamin D level in children with OI in southern Iran.
Method:
This case-control study was conducted on 23 children, clinically diagnosed as osteogenesis imperfecta and 23 age- and gender-matched healthy controls. Demographic and anthropometric data, biochemical parameters, puberty, sun exposure and physical activity were assessed. Bone mineral density (BMD) was measured by Dual-energy X-ray absorptiometry (DXA). Data analysis was done by SPSS22.
Results:
Forty-three point four percent of OI patients and fifty-six point five percent of control group had vitamin D deficiency (P = 0.376). Thirteen OI patients (56%) had low bone mass for chronological age in lumbar area (P < 0.001). Fracture episodes during treatment was significantly influenced by time of Pamidronate start, courses of Pamidronate injection, puberty and sun exposure (P values = 0.015, 0.030, 0.044 and 0.032, respectively). Fracture episodes during treatment had significantly increased in patients who had received Pamidronate more than 3 years compared with those received less than 3 years(P values = 0.047).
Conclusions:
This study showed that vitamin D deficiency is prevalent amongst OI children in southern Iran. More than half of the OI children had low bone mass for chronological age in lumbar area, despite receiving bisphosphonate therapy. The present results revealed that early initiation of Pamidronate and number of Pamidronate courses are associated with lower fracture rate. However, treatment period more than 3 years can have adverse effect on fracture rates.
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