Consensus statement: Guide to bone health and disease in cystic fibrosis
Consensus statement: Guide to bone health and disease in cystic fibrosis
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DOI：
10.1210/jc.2004-1629
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发表时间：
2005-03-01
影响因子：
5.8
通讯作者：
White, TB
中科院分区：
文献类型：
--
作者：
Aris, RM;Merkel, PA;White, TB
囊性纤维化（CF）是高加索人群中最常见的遗传性疾病，并导致过早的呼吸衰竭。目前在北美和欧洲约有60，000人患有CF，其中40%是成年人。在过去的三十年中，这些患者的寿命从大约2岁增加到32岁。骨疾病已成为CF长期存活者的常见并发症。一些研究发现，50 - 75%的成年人骨密度低，骨折率增加。CF相关骨病的预防和治疗必须解决导致骨骼健康不良的无数风险因素（由于胰腺功能不全导致的脂溶性维生素吸收减少，性激素产生改变，慢性肺部感染伴骨活性细胞因子水平升高，缺乏体力活动和糖皮质激素治疗）。这篇综述是对囊性纤维化中骨健康和疾病指南的浓缩和更新总结：共识会议，该会议是由囊性纤维化基金会于2002年5月召开的一次会议演变而来的，旨在解决囊性纤维化中骨疾病的发病机制，诊断和治疗。这次会议的目的是制定实践指南，优化CF患者的骨骼健康。
Cystic fibrosis (CF) is the most common genetic disease within the Caucasian population and leads to premature respiratory failure. Approximately 60,000 individuals are currently living with CF in North America and Europe, 40% of whom are adults. The lifespan of these patients has increased from approximately 2 to 32 yr of age over the last three decades. Bone disease has emerged as a common complication in long-term survivors of CF. Some studies have observed that 50 - 75% of adults have low bone density and increased rates of fractures. Prevention and treatment of CF-related bone disease must address the myriad risk factors ( decreased absorption of fat-soluble vitamins due to pancreatic insufficiency, altered sex hormone production, chronic lung infection with increased levels of bone-active cytokines, physical inactivity, and glucocorticoid therapy) for poor bone health. This review is a condensed and updated summary of the Guide to Bone Health and Disease in Cystic Fibrosis: A Consensus Conference, a statement that evolved from a meeting convened by the Cystic Fibrosis Foundation in May 2002 to address the pathogenesis, diagnosis, and treatment of bone disease in CF. The goal of this conference was to develop practice guidelines for optimizing bone health in patients with CF.