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Relation of Sweat Chloride Concentration to Severity of Lung Disease in Cystic Fibrosis by Pamela B. Davis; Mark D. Schluchter; Michael W. Konstan is a Medicine article available to read on EtoBox.

What is Relation of Sweat Chloride Concentration to Severity of Lung Disease in Cystic Fibrosis about?

In cystic fibrosis (CF), sweat chloride concentration has been proposed as an index of CFTR function for testing systemic drugs designed to activate mutant CFTR. This suggestion arises from the assumption that greater residual CFTR function should lead to a lower sweat chloride concentration, as well as protection against severe lung disease. This logic gives rise to the hypothesis that the lower the sweat chloride concentration, the less severe the lung disease. In order to test this hypothesis, we studied 230 patients homozygous for the DeltaF508 allele, and 34 patients with at least one allele associated with pancreatic sufficiency, born since January 1, 1955, who have pulmonary function data and sweat chloride concentrations recorded in our CF center database, and no culture positive for B. cepacia. We calculated a severity index for pulmonary disease, using an approach which takes into account all available pulmonary function data as well as the patient's current age and survival status. Patients with alleles associated with pancreatic sufficiency had significantly better survival (P = 0.0083), lower sweat chloride concentration (81.4 +/- 23.8 vs. 103.2 +/- 14.2 mEq/l, P < 0.0

Who reads Relation of Sweat Chloride Concentration to Severity of Lung Disease in Cystic Fibrosis?

Author

Pamela B. Davis; Mark D. Schluchter; Michael W. Konstan

Publisher

John Wiley and Sons; Wiley (John Wiley & Sons); John Wiley & Sons Inc.; Wiley (ISSN 8755-6863)

Published

2004

Language

EN

Field

Medicine (Health Sciences)