囊性纤维化相关性骨病。
Cystic fibrosis-related bone disease.
机构信息
aDepartment of Rheumatology bINSERM U1088 'Pathophysiological mechanisms and consequences of cardiovascular calcification: role of cardiovascular and bone remodelling', Avenue René Laennec, University hospital of Amiens, Amiens cPMOI EA 4490, IFR 114, F-59000 Lille dDépartement Universitaire de Rhumatologie, Hôpital Roger-Salengro, CHU Lille, 59037 Lille cedex, France.
出版信息
Curr Opin Pulm Med. 2013 Nov;19(6):681-6. doi: 10.1097/MCP.0b013e3283659e35.
PURPOSE OF REVIEW
This review highlights recently published data on the pathophysiology, guidelines and treatment of cystic fibrosis (CF)-related bone disease.
RECENT FINDINGS
The exact role of the cystic fibrosis transmembrane conductance regulator (CFTR), specifically the ΔF508 allele, has been investigated in F508del-CFTR homozygous mice and the F508del-CFTR mutation may contribute to CF-related bone disease by slowing new bone formation. The European Cystic Fibrosis Society has issued guidelines for bone mineral density assessment, management of low-trauma fractures and bisphosphonate therapy. A systematic review based on meta-analyses reports that oral and intravenous bisphosphonates both improve bone mineral density in CF patients, but no data are available concerning the reduction of low-trauma fractures.
SUMMARY
European Cystic Fibrosis Society guidelines may help physicians to improve the management of CF-related bone disease.
目的综述
本文重点介绍了囊性纤维化（CF）相关骨病的病理生理学、指南和治疗方面的最新研究进展。
最近发现
囊性纤维化跨膜电导调节因子（CFTR），特别是 ΔF508 等位基因的具体作用，已在 F508del-CFTR 纯合子小鼠中进行了研究，并且 F508del-CFTR 突变可能通过减缓新骨形成而导致 CF 相关骨病。欧洲囊性纤维化协会已经发布了骨密度评估、低创伤性骨折管理和双膦酸盐治疗的指南。一项基于荟萃分析的系统评价报告称，口服和静脉用双膦酸盐均可改善 CF 患者的骨密度，但尚无关于减少低创伤性骨折的数据。
总结
欧洲囊性纤维化协会指南可能有助于医生改善 CF 相关骨病的管理。
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