Cystic Fibrosis Liver Disease
نویسندگان
چکیده
Cystic fibrosis (CF) is the most common fatal autosomal recessive disorder in the white population with a frequency of 1 in 2500 live births. Inherited defects in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in abnormal regulation of salt and water movement across membranes. The overall feature of CF is that secretions are dehydrated due to water deprivation of luminal surfaces. This is true for the sinopulmonary tract, biliary tree and for reproductive tubes (vas deferens and fallopian tubes).
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