Pediatrics & Neonatology
Volume 49, Issue 5 , Pages 161-165, October 2008

Intravenous Pamidronate Therapy in Taiwanese Patients with Osteogenesis Imperfecta

  • Hsiang-Yu Lin

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan
    • Mackay Medicine, Nursing and Management College, Taipei, Taiwan
    • Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan
  • ,
  • Shuan-Pei Lin

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan
    • Mackay Medicine, Nursing and Management College, Taipei, Taiwan
    • Department of Infant and Child Care, National Taipei College of Nursing, Taipei, Taiwan
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, Mackay Memorial Hospital, 92 Section 2, Chung-Shan North Road, Taipei 10449, Taiwan
  • ,
  • Chih-Kuang Chuang

      Affiliations

    • Department of Medical Research, Mackay Memorial Hospital, Taipei, Taiwan
    • Medical College, Fu-Jen Catholic University, Taipei, Taiwan
  • ,
  • Ming-Ren Chen

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan
    • Mackay Medicine, Nursing and Management College, Taipei, Taiwan
  • ,
  • Chia-Ying Chang

      Affiliations

    • Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan

Received 14 February 2008; received in revised form 16 July 2008; accepted 19 September 2008.

Article Outline

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 (30 mg/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.

Key Words:  bisphosphonates , bone mineral density , fracture , osteogenesis imperfecta , pamidronate

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PII: S1875-9572(09)60002-4

doi:10.1016/S1875-9572(09)60002-4

Pediatrics & Neonatology
Volume 49, Issue 5 , Pages 161-165, October 2008