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.
O'Loughlin
E V
EV
Department of Paediatrics and Child Health, Sydney University, New South Wales, Australia.
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