Scheres Luuk J J, van Dijk Fleur S, Harsevoort Arjan J, van Dijk Atty T H, Dommisse Anne Marieke, Janus Guus J M, Franken Anton A M
Expert Center for adults with Osteogenesis Imperfecta, Isala Hospital, Zwolle, The Netherlands.
Department of Clinical Genetics, University Medical Center Groningen, Groningen, The Netherlands.
Bone Rep. 2018 Apr 25;8:168-172. doi: 10.1016/j.bonr.2018.04.009. eCollection 2018 Jun.
An expert center for adults with Osteogenesis Imperfecta (OI) has been founded at the Isala Hospital in Zwolle, the Netherlands to achieve optimal care for adults with OI. Clinical data such as patient history, Dual Energy X-ray Absorptiometry measurements and laboratory findings are collected with patient consent. This study provides an overview of clinical characteristics of the patients who visited the clinic during its first 5 years, a total of 151 patients. In this study, we focus on bisphosphonate use and bone density measurements at time of presentation at the expert center. As such, insight into the natural history of OI in adults will be increased. Analysing the data of a large group of adults with this rare disorder within a national expert center will allow detailed exploration of the course of OI over time.
荷兰兹沃勒的伊萨拉医院设立了一个成骨不全症（OI）成人专家中心，旨在为成骨不全症成人患者提供最佳护理。在患者同意的情况下收集临床数据，如病史、双能X线吸收测定法测量结果和实验室检查结果。本研究概述了该诊所头5年接待的患者的临床特征，共有151名患者。在本研究中，我们重点关注患者在专家中心就诊时双膦酸盐的使用情况和骨密度测量结果。这样一来，对成人OI自然病史的了解将得到加深。在国家专家中心分析一大群患有这种罕见疾病的成年人的数据，将有助于详细探究OI随时间的发展过程。
