Cystic Fibrosis Walk
In 1981, Michael Knowles, Richard Boucher and colleagues, from the University of North Carolina, USA, demonstrated an abnormally high potential difference in the nasal mucosa of patients with CF thus providing more direct evidence of epithelial dysfunction (Knowles et al, 1981 below). There was a defect in the ability of chloride to move across CF cells, chloride permeability was not activated by beta-agonists (as it is in non-CF subjects) and there was an excessively rapid absorption of sodium. The abnormality was also present in newborns with CF indicating a primary abnormality rather being secondary to circulating CF factors or other substances.In 1983 Paul Quinton, who himself has CF, showed that the chloride impermeability he had demonstrated in sweat glands was the basis for the raised sweat electrolytes in patients with cystic fibrosis (Quinton, 1983 below). These were the most important advances to date in understanding the basic defect as a membrane electrolyte transport problem since the discovery of the abnormal salt content of the sweat by Paul di Sant’Agnese in 1953.