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Cystic fibrosis: current concepts.
W V Med J 1993; 89(6):236-40WV

Abstract

Cystic fibrosis (CF), a common fatal genetic disease, is a multisystem disorder whose pathogenesis has recently been linked to defects in CFTR, a newly discovered protein. CFTR is a molecular channel which controls chloride concentration in secretions of the sweat glands and the respiratory, GI, and reproductive tracts. Defective forms of CFTR, arising from various mutations in its gene, are responsible for the inadequate hydration of mucus, pancreatic juice, and other exocrine secretions. The result is dysfunction of the lungs, pancreas, and other involved organs. This article describes the diagnosis, clinical features, and approach to management of CF.

Authors+Show Affiliations

Section of Pulmonary and Critical Care Medicine, West Virginia University School of Medicine, Morgantown.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

7686701

Citation

Abrons, H L.. "Cystic Fibrosis: Current Concepts." The West Virginia Medical Journal, vol. 89, no. 6, 1993, pp. 236-40.
Abrons HL. Cystic fibrosis: current concepts. W V Med J. 1993;89(6):236-40.
Abrons, H. L. (1993). Cystic fibrosis: current concepts. The West Virginia Medical Journal, 89(6), pp. 236-40.
Abrons HL. Cystic Fibrosis: Current Concepts. W V Med J. 1993;89(6):236-40. PubMed PMID: 7686701.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cystic fibrosis: current concepts. A1 - Abrons,H L, PY - 1993/6/1/pubmed PY - 1993/6/1/medline PY - 1993/6/1/entrez SP - 236 EP - 40 JF - The West Virginia medical journal JO - W V Med J VL - 89 IS - 6 N2 - Cystic fibrosis (CF), a common fatal genetic disease, is a multisystem disorder whose pathogenesis has recently been linked to defects in CFTR, a newly discovered protein. CFTR is a molecular channel which controls chloride concentration in secretions of the sweat glands and the respiratory, GI, and reproductive tracts. Defective forms of CFTR, arising from various mutations in its gene, are responsible for the inadequate hydration of mucus, pancreatic juice, and other exocrine secretions. The result is dysfunction of the lungs, pancreas, and other involved organs. This article describes the diagnosis, clinical features, and approach to management of CF. SN - 0043-3284 UR - https://www.unboundmedicine.com/medline/citation/7686701/Cystic_fibrosis:_current_concepts_ L2 - http://babysfirsttest.org/newborn-screening/conditions/cystic-fibrosis-cf DB - PRIME DP - Unbound Medicine ER -