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Beneficial effect of long term intravenous bisphosphonate treatment of osteogenesis imperfecta.

http://www.ncbi.nlm.nih.gov/pubmed/11970931

AIM: To find an effective symptomatic treatment for osteogenesis imperfecta (OI). METHODS: In a prospective observational study disodium pamidronate (APD) was given as monthly intravenous infusions to 28 children and adolescents (aged 0.6-18 years) with severe OI or a milder form of the disease, but with spinal compression fractures. RESULTS: During treatment for 2-9 years, dual energy x ray absorptiometry measurements of the total body and of the lumbar spine showed a gradual increase in bone density. All bone metabolism variables in serum (alkaline phosphatase, osteocalcin, procollagen 1 C-terminal peptide, collagen 1 teleopeptide) and urine (deoxypyridinoline) indicated that there was a decrease in bone turnover. All patients experienced beneficial effects and the younger patients reported a major improvement in wellbeing, pain, and mobility without significant side effects. Vertebral remodelling was also seen. CONCLUSIONS: APD seems to be an efficient symptomatic treatment for children and adolescents with OI.

Pubmed ID: 11970931 RIS Download

Mesh terms: Absorptiometry, Photon | Adolescent | Biological Markers | Bone Density | Bone Remodeling | Child | Child, Preschool | Diphosphonates | Fractures, Spontaneous | Health Status | Humans | Infant | Infusions, Intravenous | Long-Term Care | Osteogenesis Imperfecta | Pain | Prospective Studies | Recurrence | Spinal Cord Compression | Treatment Outcome