Literature DB >> 19133566

Intravenous pamidronate therapy in Taiwanese patients with osteogenesis imperfecta.

Hsiang-Yu Lin1, Shuan-Pei Lin, Chih-Kuang Chuang, Ming-Ren Chen, Chia-Ying Chang.   

Abstract

BACKGROUND: Information on the long-term efficacy of intravenous pamidronate therapy in Asian patients with osteogenesis imperfecta (OI) is limited. We report our experience using pamidronate in Taiwanese patients with OI.
METHODS: Twenty-six patients with type I, III, or IV OI (eight males and 18 females; age range at last follow-up, 2.9-39.2 years) who received (or were currently receiving) intravenous pamidronate treatment (30mg/m2/dose, every month) were retrospectively analyzed. Patients were followed for 1.0-7.3 years over the study period from February 2000 to October 2007.
RESULTS: The mean standard deviation score (SDS) for bone mineral density (BMD) had increased significantly from -4.72 to -3.37 (p < 0.005) after 1 year of treatment. In 16 patients evaluated after 4 years and eight after 6 years, the mean SDS continued to improve, to -2.69 (p < 0.001) and -1.54 (p < 0.005), respectively. The fracture rate decreased significantly (from 2.8 +/- 1.1 to 0.6 +/- 0.6, p < 0.001), and nine patients (35%) had no fractures while receiving treatment. The response to pamidronate was significantly better in patients with poorer initial BMD SDS (1 year: r = -0.71, p < 0.01; 4 years: r = -0.81, p < 0.01).
CONCLUSION: This retrospective study suggests that Taiwanese patients with OI can benefit from pamidronate treatment, leading to a reduced incidence of fractures and increased BMD, especially in patients with poor baseline BMD.

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Year:  2008        PMID: 19133566     DOI: 10.1016/S1875-9572(09)60002-4

Source DB:  PubMed          Journal:  Pediatr Neonatol        ISSN: 1875-9572            Impact factor:   2.083


  5 in total

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4.  Surgical Strategy to Decrease the Revision Rate of Fassier-Duval Nailing in the Lower Limbs of Osteogenesis Imperfecta.

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5.  Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses.

Authors:  Hsiang-Yu Lin; Shou-Chuan Shih; Chih-Kuang Chuang; Ming-Ren Chen; Dau-Ming Niu; Shuan-Pei Lin
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  5 in total

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