Cystic fibrosis: an inborn error of cellular electrolyte transport?
Abnormal epithelial transport in cystic fibrosis (CF) appears to provide a unifying hypothesis to explain the varying clinical manifestations of CF. The major abnormality is the cell regulation of epithelial Cl- secretion; however, a number of other abnormalities of electrolyte transport has been observed. A description of the normal physiology of secretion and the current state of our knowledge of the abnormalities of epithelial secretion is discussed.
Department of Paediatrics and Child Health, Sydney University, New South Wales, Australia.
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O'Loughlin
D000818:Animals; D001692:Biological Transport; D002118:Calcium / Q000378:metabolism; D002712:Chlorides / Q000378:metabolism; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism*; D004573:Electrolytes / Q000378:metabolism*; D004848:Epithelium / Q000378:metabolism; D006801:Humans; D012964:Sodium / Q000378:metabolism
D002712:Chlorides; D004573:Electrolytes; D012964:Sodium; D002118:Calcium