Increasing life expectancy in cystic fibrosis: Advances and challenges

被引：92
|
作者：
McBennett, Kimberly A. [1 ,2 ]
Davis, Pamela B. [1 ]
Konstan, Michael W. [1 ,2 ]
机构：
[1] Case Western Reserve Univ, Sch Med, Cleveland, OH USA
[2] Rainbow Babies & Childrens Hosp, Cleveland, OH 44106 USA
来源：
PEDIATRIC PULMONOLOGY | 2022年 / 57卷
关键词：
CFTR modulators; cystic fibrosis; lung function; survival; TEZACAFTOR-IVACAFTOR; PSEUDOMONAS-CEPACIA; PULMONARY-FUNCTION; HISPANIC PATIENTS; DIAGNOSIS; OUTCOMES; COLONIZATION; LUMACAFTOR; GUIDELINES; IBUPROFEN;
D O I：
10.1002/ppul.25733
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
Since the first description of cystic fibrosis in 1938, there have been significant advances in both quality of life and longevity for people living with this disease. In this article we describe the milestones of the last 80 years and what we perceive to be the remaining barriers to normalcy for this population.
引用
收藏
页码：S5 / S12
页数：8
相关论文
共 50 条
  • [31] Cystic fibrosis: Epidemiology, clinical manifestations, diagnosis and treatment
    Caceres, Layla Diab
    de Lucas, Ester Zamarron
    MEDICINA CLINICA, 2023, 161 (09): : 389 - 396
  • [32] Nutritional Status in the First 2 Years of Life in Cystic Fibrosis Diagnosed by Newborn Screening
    Munck, Anne
    Boulkedid, Rym
    Weiss, Laurence
    Foucaud, Pierre
    Wizla-Derambure, Nathalie
    Reix, Philippe
    Bremont, Francois
    Derelle, Jocelyne
    Schroedt, Julien
    Alberti, Corinne
    JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2018, 67 (01) : 123 - 130
  • [33] Quality of life in patients with cystic fibrosis
    deJong, W
    Kaptein, AA
    vanderSchans, CP
    Mannes, GPM
    vanAalderen, WMC
    Grevink, RG
    Koeter, GH
    PEDIATRIC PULMONOLOGY, 1997, 23 (02) : 95 - 100
  • [34] New Challenges in the Diagnosis and Management of Cystic Fibrosis
    Levy, Hara
    Farrell, Philip M.
    JOURNAL OF PEDIATRICS, 2015, 166 (06) : 1337 - 1341
  • [35] Managing Fungal Infections in Cystic Fibrosis Patients: Challenges in Clinical Practice
    Magee, Lauren C.
    Louis, Mariam
    Khan, Vaneeza
    Micalo, Lavender
    Chaudary, Nauman
    INFECTION AND DRUG RESISTANCE, 2021, 14 : 1141 - 1153
  • [36] Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non-Cystic Fibrosis Bronchiectasis
    Bienvenu, Thierry
    Sermet-Gaudelus, Isabelle
    Burgel, Pierre-Regis
    Hubert, Dominique
    Crestani, Bruno
    Bassinet, Laurence
    Dusser, Daniel
    Fajac, Isabelle
    AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2010, 181 (10) : 1078 - 1084
  • [37] Factors associated to quality of life in children and adolescents with cystic fibrosis
    Santana, Nelbe Nesi
    Moutinho de Miranda Chaves, Celia Regina
    Goncalves, Christine Pereira
    Gomes Junior, Saint Clair dos Santos
    REVISTA PAULISTA DE PEDIATRIA, 2020, 38 : e2018397
  • [38] Cystic fibrosis newborn screening does not delay the identification of cystic fibrosis in children with negative results
    MacLean, Joanna E.
    Solomon, Melinda
    Corey, Mary
    Selvadurai, Hiran
    JOURNAL OF CYSTIC FIBROSIS, 2011, 10 (05) : 333 - 337
  • [39] Advances in the detection and management of cystic fibrosis related diabetes
    Hameed, Shihab
    Jaffe, Adam
    Verge, Charles F.
    CURRENT OPINION IN PEDIATRICS, 2015, 27 (04) : 525 - 533
  • [40] Results from an online survey of adults with cystic fibrosis: Accessing and using life expectancy information
    Keogh, Ruth H.
    Bilton, Diana
    Cosgriff, Rebecca
    Kavanagh, Dominic
    Rayner, Oliver
    Sedgwick, Philip M.
    PLOS ONE, 2019, 14 (04):
12345