Benefits of pamidronate in children with osteogenesis imperfecta: an open prospective study - 01/01/05
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Data presented in part at the 8th international conference on osteogenesis imperfecta, Annecy, 2002
Abstract |
Objectives.- To study the efficacy of pamidronate in children with osteogenesis imperfecta (OI).
Patients and methods.- Twenty-nine patients (median age 8.7 years), were given pamidronate in cyclic infusions of 3 days. Patients received 3-13 cycles (median 6), at a dose of 0.5 mg/kg/day in infants (below 2 years of age) and 1 mg/kg/day in children (2 years and older). The interval time between cycles was 2 months in infants and 4 months in children. The median follow-up was 16 months. All patients received daily supplementation of calcium, vitamin D and physical rehabilitation. Assessments were performed at baseline and before each cycle. Fracture rate under treatment was compared to the one in the pre-treatment period.
Results.- Pain decreased after the first infusion cycle (P < 0.0001). The median of fracture incidence decreased from 15 to 0.5 per year in infants and from 2.0 to 1 per year in children (P = 0.04). Alkaline phosphatase decreased by 31.2% and N-telopeptide collagen cross-links decreased by 61.8% (P < 0.001). Bone mineral density (BMD) of the spine increased by a median of 55.4% (P < 0.001). Z-scores increased from a median of -4.7 to -2.6 (P < 0.001). The femoral neck, BMD increased by a median of 16%. The area of the first four lumbar vertebrae increased by a median of 21.5% (P < 0.001). No adverse effect on growth or on fracture healing was observed. Side effects were symptomatic hypocalcemia in one infant, and the transient acute phase reaction.
Conclusion.- Pamidronate increases BMD, decreases bone remodeling markers, pain and fracture rate in infants and children with OI.
Le texte complet de cet article est disponible en PDF.Keywords : Children, Fracture, Osteogenesis imperfecta, Pamidronate
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Vol 72 - N° 4
P. 313-318 - juillet 2005 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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