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[成骨不全症:一项多学科挑战]

[Osteogenesis imperfecta : A multidisciplinary challenge].

作者信息

Mindler Gabriel T, Ganger Rudolf, Stauffer Alexandra, Marhofer Peter, Raimann Adalbert

机构信息

Abteilung für Kinderorthopädie, Orthopädisches Spital Speising, Speisinger Str. 109, 1130, Wien, Österreich.

Vienna Bone and Growth Center, Wien, Österreich.

出版信息

Orthopadie (Heidelb). 2022 Jul;51(7):595-606. doi: 10.1007/s00132-022-04260-7. Epub 2022 May 25.

Abstract

Osteogenesis imperfecta (OI) describes a group of rare diseases which are associated with an increased tendency to bone fractures. In addition to the main symptom of fractures, OI is characterized by a variety of pediatric, pediatric orthopedic and anesthesiological challenges. The multidisciplinary expertise is mostly concentrated at specialized centers. The current treatment concepts aim at minimizing the fracture rate, prevention and treatment of deformities, pain reduction and improved mobility. In addition to pharmacotherapy, conservative and surgical measures are also applied. Scheduled interventions on the extremities are one of the most commonly performed operations in children with OI. Various intramedullary nailing techniques are available. This article addresses the important aspects of multidisciplinary care of children with OI concerning the treatment of the lower extremities.

摘要

成骨不全症(OI)是一组罕见疾病,与骨折倾向增加有关。除了骨折这一主要症状外,成骨不全症还具有多种儿科、小儿骨科和麻醉方面的挑战。多学科专业知识大多集中在专门的中心。目前的治疗理念旨在将骨折率降至最低、预防和治疗畸形、减轻疼痛并改善活动能力。除了药物治疗外,还采用保守和手术措施。对四肢进行定期干预是成骨不全症患儿最常进行的手术之一。有多种髓内钉技术可供选择。本文讨论了成骨不全症患儿多学科护理中有关下肢治疗的重要方面。

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