Cystic fibrosis: A changing landscape

被引：2

作者：

Wendekier, Camille ^{[1
]}

Wendekier-Raybuck, Katheryn ^{[1
]}

机构：

[1] St Francis Univ, Loretto, PA 15940 USA

来源：

NURSE PRACTITIONER | 2021年 / 46卷 / 09期

关键词：

CF; CFTR gene; CFTR modulators; cystic fibrosis; cystic fibrosis transmembrane conductance regulator gene;

D O I：

10.1097/01.NPR.0000769756.82495.f3

中图分类号：

R47 [护理学];

学科分类号：

1011 ;

摘要：

Cystic fibrosis is an autosomal recessive genetic disorder that causes a lifetime of debilitating and potentially fatal complications affecting the lungs and other organ systems. Over 1,700 gene mutations that cause this rare disorder have been identified. This article describes the current treatment landscape for adults with CF, including the 2019 FDA approval of a breakthrough triple-drug combination therapy that may significantly improve the quality of life for an estimated 90% of patients with CF.

引用

页码：48 / 55

页数：8

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