ICCBH2015 Poster Presentations (1) (201 abstracts)
Increase of bone mineral density in patients with osteogenesis imperfecta treated with pamidronate disodium.
Hamilton Cabral de Menezes Filho , Joyce de Brito Pupo , Paula de Magalhaes Velasco Bastos , Chong Ae Kim , Debora Romeo Bertola , Simone Sakura Ito , Tiago Jeronimo dos Santos , Nuvarte Setian & Durval Damiani
Instituto da Criança – Hospital das Clínicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil.
Objective: This study aimed to evaluate the efficacy of the treatment with pamidronate disodium (PD) with respect to the increase of bone mineral density (BMD) in patients with osteogenesis imperfecta (OI).
Patients and methods: We evaluated nine patients (six boys) with OI (four with type III, four with type IV, one with type I) treated with PD from 4.68 to 7.92 years (mean±S.D.: 6.75±1.38 years). Intravenous PD was administered in a 1 mg/kg single daily dose for three sequential days at 4-month intervals. For each patient BMD was evaluated twice through dual-energy x-ray absorptiometry (DEXA-scan): the first DEXA-scan was performed before or up to 0.72 years after the beginning of the treatment (mean age: 6.69±1.11 years), and the second DEXA-scan was performed 0.94 to 2.8 years after the first one (interval between the two DEXA-scans: 1.86±0.64 years). The values of lumbar spine (L1–L4) BMD z-score in the first DEXA-scan (Z1) and in the second DEXA-scan (Z2) were compared through paired Student’s t-test (P-values<0.05 were significant).
Results: The values of Z1 and Z2 ranged respectively between −2.8 and −7.9 (mean±S.D.: −5.12±1.59) and between −1.76 and −5.1 (mean±SD: −3.44±1.21). The mean of Z2 was significantly higher than the mean of Z1 (P=0.0002). In all patients the values of Z2 were higher than the values of Z1 and the difference Z2–Z1 varied between 1.12 and 3.52 (mean±SD: 1.80±0.76). The increase of lumbar BMD z-score varied between 14 and 53% (mean±SD: 33±12).
Conclusion: The significant improvement of BMD in patients with OI treated with PD shows that this treatment increased bone mineral accretion efficiently.
Disclosure: The authors declared no competing interests.
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