title: Intravenous pamidronate therapy in Taiwanese patients with osteogenesis imperfecta.
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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.
authors.forename: Hsiang-Yu
authors.initials: HY
authors.lastname: Lin
authors.forename: Shuan-Pei
authors.initials: SP
authors.lastname: Lin
authors.forename: Chih-Kuang
authors.initials: CK
authors.lastname: Chuang
authors.forename: Ming-Ren
authors.initials: MR
authors.lastname: Chen
authors.forename: Chia-Ying
authors.initials: CY
authors.lastname: Chang
mesh_terms: D000293:Adolescent; D000328:Adult; D015519:Bone Density / Q000187:drug effects; D050071:Bone Density Conservation Agents / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D004164:Diphosphonates / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D010013:Osteogenesis Imperfecta / Q000188:drug therapy*; D000077268:Pamidronate; D012189:Retrospective Studies; D013624:Taiwan
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D000077268:Pamidronate