el-Sobky Mohammed A, Hanna Atef A Zaky, Basha Naguib E, Tarraf Yehia N, Said May H
Department of Pediatric Orthopedics, Faculty of Medicine, Cairo University, Cairo, Egypt.
J Pediatr Orthop B. 2006 May;15(3):222-8. doi: 10.1097/01.bpb.0000192058.98484.5b.
The aim of this study was to evaluate the efficacy of pamidronate in the management of osteogenesis imperfecta patients. This study was carried out in two groups. The first was treated only surgically whereas the second was treated by a combined approach, medical and surgical. Forty patients, divided into two groups, were surgically treated in order to correct bony deformities secondary to osteogenesis imperfecta. Group 1: twenty patients were operated at an average age of 6.5 years. Nine were type I, five type III and six type IV. Group 2: this group consisted of 20 patients to whom intermittent intravenous pamidronate were given at regular intervals for an average of 2 years postoperatively. The average age at surgery was 8.5 years. Four patients were type I, six type III, eight type IV, one type V and the remaining one type VII. The results were assessed according to a scoring system suggested and used by the authors since 1999. Group 1: we had three good, nine fair and eight poor results. Group 2: we had 11 excellent, four good and five fair results. The Bone mineral dens (BMD) increased by an average of 35.2% (22.7-112%), and the rate of refracture decreased. Best results in the management of patients can be obtained through the combined approach (surgical and medical treatment). We now advise preoperative and postoperative pamidronate for these patients.
本研究的目的是评估帕米膦酸在成骨不全患者治疗中的疗效。本研究分为两组进行。第一组仅接受手术治疗，而第二组采用药物和手术相结合的综合方法治疗。40例患者分为两组，接受手术治疗以纠正成骨不全继发的骨骼畸形。第1组：20例患者平均6.5岁时接受手术。其中9例为I型，5例为III型，6例为IV型。第2组：该组由20例患者组成，术后平均2年定期接受间歇性静脉注射帕米膦酸治疗。手术时的平均年龄为8.5岁。4例为I型，6例为III型，8例为IV型，1例为V型，其余1例为VII型。根据作者自1999年以来建议并使用的评分系统评估结果。第1组：结果为3例良好，9例中等，8例差。第2组：结果为11例优秀，4例良好，5例中等。骨密度平均增加35.2%（22.7%-112%），再骨折率降低。通过综合方法（手术和药物治疗）可获得患者治疗的最佳效果。我们现在建议对这些患者在术前和术后使用帕米膦酸。
