Pediatric pulmonology
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Pediatric pulmonology · Feb 2010
ReviewCystic fibrosis lung disease starts in the small airways: can we treat it more effectively?
The aims of this article are to summarize existing knowledge regarding the pathophysiology of small airways disease in cystic fibrosis (CF), to speculate about additional mechanisms that might play a role, and to consider the available or potential options to treat it. In the first section, we review the evidence provided by pathologic, physiologic, and imaging studies suggesting that obstruction of small airways begins early in life and is progressive. In the second section we discuss how the relationships between CF transmembrane conductance regulator (CFTR), ion transport, the volume of the periciliary liquid layer and airway mucus might lead to defective mucociliary clearance in small airways. ⋯ In the final section, we discuss how established therapies impact small airways disease and new directions that may lead to improvement in the treatment of small airways disease. We conclude that there are many reasons to believe that small airways play an important role in the pathophysiology of (early) CF lung disease. Therapy should be aimed to target the small airways more efficiently, especially with drugs that can correct the basic defect at an early stage of disease.
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Pediatric pulmonology · Feb 2010
A child's asthma quality of life rating does not significantly influence management of their asthma.
Children of parents who perceive their children have increased asthma severity use more medical services and reliever medication. ⋯ Parent's QOL perception, and not the child's, is associated with asthma management. RAP decreased the parent's QOL symptoms assessment and was important in determining the child's asthma management.