Thirteen children with cystic fibrosis (CF), aged 1.5 months-15 years,
had 18 episodes of hypochloraemia and metabolic alkalosis over the pe
riod 1983-1991. Five patients were not known to have CF prior to devel
oping these electrolyte disturbances. There were two distinct clinical
presentations: 5 patients had an acute isolated picture of heat exhau
stion while 8 patients (all infants) had a more chronic course associa
ted with failure to thrive. Many episodes were not associated with par
ticularly high environmental temperatures, although most occurred duri
ng the summer and early autumn months. Serum electrolytes should be as
sessed regularly in children with CF, and this diagnosis should be con
sidered in any infant presenting with unexplained hypochloraemic metab
olic alkalosis.