E. Berard et al., CYSTIC-FIBROSIS OF THE PANCREAS REVEALED BY WATER DEPLETION WITH HYPOCHLORONATREMIC ALKALOSIS IN 3 INFANTS AND ONE NEWBORN, Archives de pediatrie, 1(1), 1994, pp. 42-45
Background. - Acute dehydration with hypochloronatremic metabolic alka
losis is a classical complication of cystic fibrosis of the pancreas.
Its progressive development as a revealing manifestation of the diseas
e is rare as is its appearance in newborns. Case n degrees 1. - A 13 m
onth-old girl was admitted because of status epilepticus. She was seve
rely dehydrated (20% weight loss) but had normal diuresis. Investigati
ons showed metabolic alkalosis, hypochloronatremia and hypokalemia. Al
l the manifestations disappeared within 5 days with treatment but thre
e sweat tests were abnormal. Case n degrees 2. - A 7 month-old girl wa
s admitted because she suffered from progressive loss of weight (10%);
she was dehydrated and had metabolic alkalosis plus hypochloronatremi
a, but her diuresis was normal. Two sweat tests were abnormal. Case n
degrees 3.-A 4.5 month-old boy was admitted because he suffered from s
evere (12%) weight loss. His diuresis was normal despite dehydration;
metabolic alkalosis and hypochloronatremia, larrelmia were found. Two
subsequent sweat tests were abnormal. Case n degrees 4. - A 3 day-old
girl was admitted suffering from meconium ileus. Two initial mechanism
analysis for protein were abnormal. She had a cardiorespiratory arres
t on the 13(th) day of life when she had lost 14% of her birth weight;
investigations showed metabolic alkalosis, hyponatremia (83 mEq/l), h
ypochloremia (45 mEq/l); kalemia was 5.9 mEq/l. Peritoneal dialysis wa
s needed to correct hydroelectrolytic changes. A sweat test performed
on the 26(th) day of life was also abnormal. Investigations performed
during the periods of dehydration, and repeated later, showed transien
t functional kidney failure. None of the four patients had any respira
tory or gastrointestinal clinical manifestations of cystic fibrosis. C
onclusion. - Metabolic alkalosis with hypochloronatremia plus progress
ive, severe dehydration in infants whose diuresis is paradoxically nor
mal must be followed by examination for cystic fibrosis.