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[Diagnosis and management of cystic fibrosis in children].
Rev Mal Respir 2000; 17(3 Pt 2):725-32RM

Abstract

Cystic fibrosis is a genetic disease occurring more frequently in Caucasians. The cystic fibrosis gene, cloned in 1999, codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Dysfunction of this protein leads to the clinical manifestations of cystic fibrosis, mainly lung disease and exocrine pancreas disorders. The pathophysiology of the respiratory component is quite complex, basically related to major and excessive inflammatory processes and to early microbial colonization. Respiratory physical therapy is a key element to management of the respiratory disorder. Antibiotic treatments should be adapted to the bacterial ecology, mainly using antistaphylococcal and antihaemophilus drugs initially, then directed against Pseudomonas aeruginosa. Other drugs including inhaled antiinflammatory drugs are currently in the evaluation stage. In addition, nutritional care and correction of pancreas insufficiency are necessary. The diagnosis of this disease must be made early although systematic neonatal screening is not proposed. Early diagnosis is necessary for improved care and prognosis. Currently, median survival is 29 years. This survival time should probably improve with better understanding of the pathophysiological mechanisms and new therapeutic perspectives.

Authors+Show Affiliations

Service de Pneumologie Pédiatrique, Hôpital Armand Trousseau, Paris, France.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

fre

PubMed ID

11076382

Citation

Tamalet, A, et al. "[Diagnosis and Management of Cystic Fibrosis in Children]." Revue Des Maladies Respiratoires, vol. 17, no. 3 Pt 2, 2000, pp. 725-32.
Tamalet A, Fauroux B, Clement A. [Diagnosis and management of cystic fibrosis in children]. Rev Mal Respir. 2000;17(3 Pt 2):725-32.
Tamalet, A., Fauroux, B., & Clement, A. (2000). [Diagnosis and management of cystic fibrosis in children]. Revue Des Maladies Respiratoires, 17(3 Pt 2), pp. 725-32.
Tamalet A, Fauroux B, Clement A. [Diagnosis and Management of Cystic Fibrosis in Children]. Rev Mal Respir. 2000;17(3 Pt 2):725-32. PubMed PMID: 11076382.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Diagnosis and management of cystic fibrosis in children]. AU - Tamalet,A, AU - Fauroux,B, AU - Clement,A, PY - 2000/11/15/pubmed PY - 2001/2/28/medline PY - 2000/11/15/entrez SP - 725 EP - 32 JF - Revue des maladies respiratoires JO - Rev Mal Respir VL - 17 IS - 3 Pt 2 N2 - Cystic fibrosis is a genetic disease occurring more frequently in Caucasians. The cystic fibrosis gene, cloned in 1999, codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Dysfunction of this protein leads to the clinical manifestations of cystic fibrosis, mainly lung disease and exocrine pancreas disorders. The pathophysiology of the respiratory component is quite complex, basically related to major and excessive inflammatory processes and to early microbial colonization. Respiratory physical therapy is a key element to management of the respiratory disorder. Antibiotic treatments should be adapted to the bacterial ecology, mainly using antistaphylococcal and antihaemophilus drugs initially, then directed against Pseudomonas aeruginosa. Other drugs including inhaled antiinflammatory drugs are currently in the evaluation stage. In addition, nutritional care and correction of pancreas insufficiency are necessary. The diagnosis of this disease must be made early although systematic neonatal screening is not proposed. Early diagnosis is necessary for improved care and prognosis. Currently, median survival is 29 years. This survival time should probably improve with better understanding of the pathophysiological mechanisms and new therapeutic perspectives. SN - 0761-8425 UR - https://www.unboundmedicine.com/medline/citation/11076382/[Diagnosis_and_management_of_cystic_fibrosis_in_children]_ L2 - http://RD3FS2PT9J.search.serialssolutions.com/openurl?url_ver=Z39.88-2004&res_dat=xri:pqm&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&genre=article&issn=0761-8425&eissn=1776-2588&volume=17&issue=3&spage=725&date=2000 DB - PRIME DP - Unbound Medicine ER -