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.
Cystic Fibrosis Walk
Cystic Fibrosis Walk
Cystic Fibrosis Walk
Cystic Fibrosis Walk
Cystic Fibrosis Walk
Cystic Fibrosis Walk
Cystic Fibrosis Walk
Cystic Fibrosis Walk
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