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成年囊性纤维化患者的诊断

Diagnosis of Adult Patients with Cystic Fibrosis.

作者信息

Nick Jerry A, Nichols David P

机构信息

Department of Medicine, National Jewish Health, 1400 Jackson, Denver, CO 80206, USA; Department of Medicine, University of Colorado Denver School of Medicine, 13001 East 17th Pl, Aurora, CO 80045, USA.

Department of Medicine, University of Colorado Denver School of Medicine, 13001 East 17th Pl, Aurora, CO 80045, USA; Department of Pediatrics, National Jewish Health, Denver, CO 80206, USA.

出版信息

Clin Chest Med. 2016 Mar;37(1):47-57. doi: 10.1016/j.ccm.2015.11.006. Epub 2015 Dec 23.

Abstract

The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

摘要

成人囊性纤维化(CF)的诊断频率越来越高。成年期诊断出的CF患者通常表现出呼吸道症状,且常伴有反复或慢性气道感染。在初次就诊时,患者可能看似仅有单一器官的临床表现,但呼吸道存在亚临床受累情况。成年期诊断出的患者对CF中心的治疗反应良好,新出现的囊性纤维化跨膜受体调节疗法有望用于治疗残余功能突变,因此在不明原因支气管扩张的成年患者中进行诊断的重要性日益增加。

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