State of progress in treating cystic fibrosis respiratory disease

被引：0
|
作者：
Patrick A Flume
Donald R Van Devanter
机构：
[1] Medical University of South Carolina,
[2] Case Western Reserve University School of Medicine,undefined
来源：
BMC Medicine | / 10卷
关键词：
cystic fibrosis; pathophysiology; treatment;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
Since the discovery of the gene associated with cystic fibrosis (CF), there has been tremendous progress in the care of patients with this disease. New therapies have entered the market and are part of the standard treatment of patients with CF, and have been associated with marked improvement in survival. Now there are even more promising therapies directed at different components of the pathophysiology of this disease. In this review, our current knowledge of the pathophysiology of lung disease in patients with CF is described, along with the current treatment of CF lung disease, and the therapies in development that offer great promise to our patients.
引用
收藏
相关论文
共 50 条
  • [1] State of progress in treating cystic fibrosis respiratory disease
    Flume, Patrick A.
    Van Devanter, Donald R.
    BMC MEDICINE, 2012, 10
  • [2] Developing collaborative works for faster progress on fungal respiratory infections in cystic fibrosis
    Schwarz, Carsten
    Vandeputte, Patrick
    Rougeron, Amandine
    Giraud, Sandrine
    de Bernonville, Thomas Duge
    Duvaux, Ludovic
    Gastebois, Amandine
    Alastruey-Izquierdo, Ana
    Teresa Martin-Gomez, Maria
    Martin Mazuelos, Estrella
    Sole, Amparo
    Cano, Josep
    Peman, Javier
    Quindos, Guillermo
    Botterel, Francoise
    Bougnoux, Marie-Elisabeth
    Chen, Sharon
    Delhaes, Laurence
    Favennec, Loic
    Ranque, Stephane
    Sedlacek, Ludwig
    Steinmann, Joerg
    Vazquez, Jose
    Williams, Craig
    Meyer, Wieland
    Le Gal, Solene
    Nevez, Gilles
    Fleury, Maxime
    Papon, Nicolas
    Symoens, Francoise
    Bouchara, Jean-Philippe
    MEDICAL MYCOLOGY, 2018, 56 : S42 - S59
  • [3] Monitoring Respiratory Disease Severity in Cystic Fibrosis
    Davies, Jane C.
    Alton, Eric W. F. W.
    RESPIRATORY CARE, 2009, 54 (05) : 606 - 615
  • [4] Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis
    Cuyx, Senne
    De Boeck, Kris
    SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE, 2019, 40 (06) : 762 - 774
  • [5] Treating resistant Pseudomonas aeruginosa lung disease in young children with cystic fibrosis
    Lim, Sandy Z. P.
    Fitzgerald, Dominic A.
    PAEDIATRIC RESPIRATORY REVIEWS, 2018, 27 : 33 - 36
  • [6] Progress and challenges in fungal lung disease in cystic fibrosis
    Hong, Gina
    CURRENT OPINION IN PULMONARY MEDICINE, 2022, 28 (06) : 584 - 590
  • [7] Respiratory disease in cystic fibrosis: from pathophysiology to therapy
    Reynaud-Gaubert, M
    REVUE DES MALADIES RESPIRATOIRES, 1999, 16 (04) : 495 - 509
  • [8] An Overview of the Role of Respiratory Physiotherapy in Cystic Fibrosis Disease
    Abdelbasset, Walid Kamal
    INTERNATIONAL JOURNAL OF LIFE SCIENCE AND PHARMA RESEARCH, 2021, : 14 - 18
  • [9] Respiratory bacterial infections in cystic fibrosis
    Ciofu, Oana
    Hansen, Christine R.
    Hoiby, Niels
    CURRENT OPINION IN PULMONARY MEDICINE, 2013, 19 (03) : 251 - 258
  • [10] Progress in Gene and Cell Therapy for Cystic Fibrosis Lung Disease
    Griesenbach, Uta
    Alton, Eric W. F. W.
    CURRENT PHARMACEUTICAL DESIGN, 2012, 18 (05) : 642 - 662
12345