Dropping acid: why is cystic fibrosis mucus abnormal?
نویسندگان
چکیده
منابع مشابه
Mucus, phlegm, and sputum in cystic fibrosis.
Although some clinicians still believe that cystic fibrosis (CF) lung disease is largely due to hypersecretion of very viscous mucus, it has never been demonstrated that there is mucus hypersecretion in CF and it is clear that there is almost no intact mucin (the principal polymeric component of normal mucus) in CF sputum. CF sputum has lower viscosity when compared to asthma or bronchitis sput...
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BACKGROUND Electrochemical skin conductance measurement is an active electrophysiologic method in which incremental low direct voltage is applied on the skin. It generates a current due to reverse iontophoresis which previous studies suggested to be mostly related to chloride anion movements. As sweat chloride movements upon electric stimulation were likely to be impaired in cystic fibrosis (CF...
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The most common lethal genetic disease in the Cauca-sian population is cystic fibrosis (CF). In 1970, the median life expectancy was 8 yr; today, with the concerted effort of scientists and clinicians, the median survival age has risen to 30 yr. About 1 in 23 people carry a single defective copy of the CF gene in the United States population and ف 40,000 people in the US have cystic fibrosis....
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BACKGROUND In cystic fibrosis (CF), genetic mutations in the CF transmembrane conductance regulator (CFTR) gene cause reduced chloride efflux from ciliated airway epithelial cells. This results in a reduction in periciliary liquid (PCL) depth of the airway surface liquid due to associated reduced water efflux. PCL layer dehydration reduces mucociliary clearance (MCC), leading to airway obstruct...
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Perlman, M., Williams, J., Hirsch, M., and Bar-Ziv, J. (1975). Archives of Disease in Childhood, 50, 727. Familial non-cystic fibrosis mucus inspissation of respiratory tract. Two sibs, whose parents are first cousins, have had chronic obstructive airways disease from birth with recurrent otitis media, sinusitis, and mastoiditis. The disease, associated with clinically abnormal mucus, differs f...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2018
ISSN: 0903-1936,1399-3003
DOI: 10.1183/13993003.02057-2018