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维持呼吸:囊性纤维化的病理生理学、诊断及治疗

Keep them breathing: Cystic fibrosis pathophysiology, diagnosis, and treatment.

作者信息

Brown Sheena D, White Rachel, Tobin Phil

机构信息

Sheena D. Brown is a clinical assistant professor at Mercer University in Atlanta, Ga. Rachel White practices at Precision Bone and Joint in Austin, Tex. Phil Tobin is director and an associate professor in the PA program at Touro University Nevada. The authors have disclosed no potential conflicts of interest, financial or otherwise.

出版信息

JAAPA. 2017 May;30(5):23-27. doi: 10.1097/01.JAA.0000515540.36581.92.

Abstract

Cystic fibrosis (CF) affects more than 30,000 people in the United States and 80,000 people worldwide. This life-threatening genetic disorder causes a buildup of thick, viscous mucus secretions in various organ systems, most commonly the gastrointestinal, pulmonary, and genitourinary systems. This article reviews the clinical manifestations, diagnosis, and monitoring of patients with CF as well as guidelines for management and emerging pharmacologic treatments.

摘要

在美国,囊性纤维化(CF)影响着超过3万人,全球受影响人数达8万人。这种危及生命的遗传性疾病会导致各个器官系统中积聚浓稠、粘性的黏液分泌物,最常见于胃肠、肺部和泌尿生殖系统。本文综述了CF患者的临床表现、诊断和监测,以及管理指南和新兴的药物治疗方法。

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