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Efficacy of low dose schedule pamidronate infusion in children with osteogenesis imperfecta
1Department of Pediatrics, Unidad Integrada Hospital Sant Joan de Déu-Hospital Clínic, Universidad de Barcelona, Spain. 7129cps@comb.es
Background:
Osteogenesis imperfecta (OI) is a rare condition in which bones are abnormally brittle with frequent fractures. A variety of therapeutic agents has been used with low efficacy. In this study, we present three patients treated for 4 years with i.v. pamidronate.
Patients And Methods:
Three prepubertal patients, aged 9 (M), 9 (F) and 11 (F) years old, with OI, were treated with 30-60 mg i.v. pamidronate every 6 months over four years. Determinations were made of plasma 1,25-dihydroxycholecalciferol, 25-hydroxycholecalciferol, insulin-like growth factor-I (IGF-I) and its transport protein (IGFBP3), osteocalcin, total alkaline phosphatase and its osseous fraction, and parathormone (PTH) at baseline and after every pamidronate infusion, Densitometry and X-ray of the vertebral column were performed at the same intervals.
Results:
Significant reductions of number of bone fractures and pain were observed in all patients, despite lack of any modification in biochemical parameters. Lumbar X-ray and densitometry showed a striking improvement by the end of the treatment period.
Conclusion:
Pamidronate seems to be useful in the treatment of patients with osteogenesis imperfecta.
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