Moorefield W G, Miller G R
J Bone Joint Surg Am. 1980 Jan;62(1):113-9.
To determine the effects of osteogenesis imperfecta and its treatment on adult life, thirty-one patients with osteogenesis imperfecta were examined at an average of nineteen years postoperatively. Seventy-two per cent of the operations that had been performed on these patients in childhood consisted of multiple osteotomies with intramedullary fixation to reduce fracture frequency and prevent bowing of the lower limbs. Only eight patients had remained non-ambulatory, seven of whom had severe long-bone deformity. Based on the study of these patients, we suggest that the severity of diaphyseal tapering and of disease are related; that scoliosis is frequent in adults with osteogenesis imperfecta; that the improvement gained at operation is maintained and enhances ambulation; and that as adults, these patients are generally very productive and socially adaptable individuals.
为确定成骨不全及其治疗对成年生活的影响，我们对31例成骨不全患者进行了检查，平均术后19年。这些患者童年时期接受的手术中，72%为多次截骨并髓内固定，以减少骨折频率并防止下肢弯曲。只有8例患者仍无法行走，其中7例有严重的长骨畸形。基于对这些患者的研究，我们认为骨干变细的严重程度与疾病严重程度相关；成骨不全的成年人中脊柱侧弯很常见；手术取得的改善得以维持并促进了行走能力；而且作为成年人，这些患者总体上是非常有生产力且适应社会的个体。
