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[Physiopathology of cystic fibrosis lung disease].
Rev Mal Respir 1999; 16(3):339-45RM

Abstract

Cystic fibrosis is a common genetic disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Although the link between the mutations of the CFTR gene and the progressive lung disease is not completely understood, noteworthy advances have been made in the understanding of the mechanisms underlying lung injury in CF. The pathophysiologic cascade starts with CFTR dysfunction resulting from mutations of the CFTR gene, and leading to abnormal ion and water transport across airway epithelia. Abnormal airway surface liquid may in turn lead to impairment of mucociliary clearance, airway mucosal anti-microbial defenses, chronic bacterial infection and inflammation. Each of these steps has been elucidated in more detail in the recent years. This is of relevance from a therapeutic viewpoint. New therapeutic interventions include gene therapy, agents that may circumvent the defect in intracellular processing of mutated CFTR, agents that may modulate defects in epithelial ion transport, and anti-inflammatory agents.

Authors+Show Affiliations

Service de Pneumologie, Hôpital Ambroise Paré, Boulogne.

Pub Type(s)

English Abstract
Journal Article
Research Support, Non-U.S. Gov't
Review

Language

fre

PubMed ID

10472642

Citation

Chinet, T. "[Physiopathology of Cystic Fibrosis Lung Disease]." Revue Des Maladies Respiratoires, vol. 16, no. 3, 1999, pp. 339-45.
Chinet T. [Physiopathology of cystic fibrosis lung disease]. Rev Mal Respir. 1999;16(3):339-45.
Chinet, T. (1999). [Physiopathology of cystic fibrosis lung disease]. Revue Des Maladies Respiratoires, 16(3), pp. 339-45.
Chinet T. [Physiopathology of Cystic Fibrosis Lung Disease]. Rev Mal Respir. 1999;16(3):339-45. PubMed PMID: 10472642.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Physiopathology of cystic fibrosis lung disease]. A1 - Chinet,T, PY - 1999/9/3/pubmed PY - 1999/9/3/medline PY - 1999/9/3/entrez SP - 339 EP - 45 JF - Revue des maladies respiratoires JO - Rev Mal Respir VL - 16 IS - 3 N2 - Cystic fibrosis is a common genetic disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Although the link between the mutations of the CFTR gene and the progressive lung disease is not completely understood, noteworthy advances have been made in the understanding of the mechanisms underlying lung injury in CF. The pathophysiologic cascade starts with CFTR dysfunction resulting from mutations of the CFTR gene, and leading to abnormal ion and water transport across airway epithelia. Abnormal airway surface liquid may in turn lead to impairment of mucociliary clearance, airway mucosal anti-microbial defenses, chronic bacterial infection and inflammation. Each of these steps has been elucidated in more detail in the recent years. This is of relevance from a therapeutic viewpoint. New therapeutic interventions include gene therapy, agents that may circumvent the defect in intracellular processing of mutated CFTR, agents that may modulate defects in epithelial ion transport, and anti-inflammatory agents. SN - 0761-8425 UR - https://www.unboundmedicine.com/medline/citation/10472642/[Physiopathology_of_cystic_fibrosis_lung_disease]_ L2 - http://www.em-consulte.com/retrieve/pii/MDOI-RMR-06-1999-16-3-0761-8425-101019-ART92 DB - PRIME DP - Unbound Medicine ER -