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Introduction- FEV is considered the gold standard spirometric measure for the assessment and management of cystic fibrosis lung disease. Recent evidence suggests that tests at lower lung volumes may be more sensitive. Objectives- To assess how many other spirometric tests are abnormal in the presence of a normal FEV (≥80%) and which spirometric tests are most sensitive in detecting airway obstruction. Methods -This was a retrospective analysis of 3,169 spirometry tests on 184 patients with cystic fibrosis aged 6 - 57 years. Tests were acceptable if they met ATS criteria. Results- Tests with normal FEV , FEF showed obstruction in 58% of tests, FEF in 31% and FEV /FVC ratio in 72%. Overall 75% of tests had an abnormality. FEF , FEF , FEF , and FEV /FVC ratio were all significantly more sensitive in identifying obstruction than FEV . The FEV /FVC ratio was the most sensitive of all tests in identifying obstruction except in adults (> 20 years), in whom FEF at 90%, was more sensitive than the ratio at 85%. FEF was also at 85% sensitive. Even though the FEV /FVC ratio was the most sensitive of all tests, in the presence of a normal ratio, 35% other tests were abnormal. Conclusions - Normal FEV , is not indicative of normal spirometry. FEV /FVC is the most sensitive measure of early or mild airway obstruction in children with cystic fibrosis. If only the FEV /FVC ratio was considered, many cases of obstruction would be missed, therefore all spirometric measures should be considered in the clinical evaluation of airway obstruction. This article is protected by copyright. All rights reserved.
This article was published in the following journal.
Name: The clinical respiratory journal
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An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)
A strain of mice widely studied as a model for cystic fibrosis. These mice are generated from embryonic stem cells in which the CFTR (cystic fibrosis transmembrane conductance regulator) gene is inactivated by gene targeting. As a result, all mice have one copy of this altered gene in all their tissues. Mice homozygous for the disrupted gene exhibit many features common to young cystic fibrosis patients, including failure to thrive, meconium ileus, and alteration of mucous and serous glands.
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