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Growth of infants with osteogenesis imperfecta treated with bisphosphonate
Kosei Hasegawa1, Masaru Inoue, Yoshiki Seino
1Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Osaka Koseinenkin Hospital, Osaka, Japan.
Insights
Cyclic pamidronate therapy in infants with osteogenesis imperfecta (OI) preserved vertebral bone density and improved morphometry. Early prevention of long bone fractures is crucial for optimal growth in children with OI.
Area of Science:
- Pediatric Endocrinology
- Orthopedics
- Genetics
Background:
- Osteogenesis imperfecta (OI) is a genetic disorder causing bone fragility and growth issues.
- Bisphosphonate therapy, including cyclic pamidronate, shows promise for fracture prevention in OI.
- Growth patterns in infants with OI undergoing bisphosphonate treatment require further investigation.
Purpose of the Study:
- To evaluate the growth and skeletal outcomes of infants with OI receiving cyclic pamidronate therapy.
- To assess the impact of bisphosphonate therapy on vertebral morphometry and fracture incidence in young OI patients.
Main Methods:
- Longitudinal monitoring of height and weight in OI infants during 18 months of cyclic pamidronate therapy.
- Radiographic analysis of vertebral morphometry and concavity index to detect fractures.
- Correlation of height z-score changes with femur fracture rates.
Main Results:
- Cyclic pamidronate therapy increased lumbar bone mineral density and reduced vertebral compression fractures.
- Infants experiencing a decrease in height z-score had a higher incidence of femur fractures.
- Vertebral morphometry was preserved during the 18-month treatment period.
Conclusions:
- Bisphosphonates effectively maintain vertebral bone structure in infants with OI.
- Preventing long bone fractures, particularly femur fractures, in infancy is critical for mitigating short stature.
- Early therapeutic interventions should prioritize long bone fracture prevention before ambulation in OI infants.
Background:
Osteogenesis imperfecta (OI) is a heritable bone disease characterized by bone brittleness and various degrees of growth disorder. Cyclic pamidronate therapy is reportedly useful to prevent bone fracture in OI and in infants with OI, but, it remains unclear how infants with OI grow during bisphosphonate therapy.
Methods:
Height and weight measurements of OI infants treated with cyclic pamidronate therapy were taken before and every 6 months during therapy until 18 months. Vertebral morphometry and the concavity index were analyzed using X-ray films taken simultaneously.
Results:
Among OI patients, those in the group for which the height z-score decreased tended to have more femur fractures than those of the group for which the height z-score increased. Morphometry of the lumbar spine showed that compression fractures occurred less during cyclic pamidronate therapy, by which the lumbar bone mineral density increased.
Conclusions:
Bisphosphonate preserved vertebral morphometry during 18 months after starting therapy in infants. Prevention of femur fracture during the infantile period might help prevent short stature; therapeutic strategies during infancy must better emphasize prevention of long bone fracture before the beginning of gait.
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