Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis.
BACKGROUND
Infants with cystic fibrosis (CF) can develop episodes of hyponatremic hypochloremic dehydration with metabolic alkalosis when they sweat excessively, which is not caused by sweating in normal infants. We investigated the incidence of the metabolic alkalosis with hypoelectrolytemia in CF infants, the possible risk factors for its occurrence and the importance of the manifestation in the diagnosis of CF.


METHODS
In order to evaluate the incidence and the risk factors for the development of this sweat-related metabolic disorder in CF, we reviewed the records of all children diagnosed as having CF before the age of 12 months in a 10-year period. Data analysis included medical history data, clinical features, biochemical parameters (blood pH, serum bicarbonate, sodium, chloride and potassium levels), sweat chloride test values, as well as genetic analysis data.


RESULTS
The prevalence of metabolic alkalosis in association with low serum electrolyte concentrations (hyponatremia, hypochloremia, and hypokalemia) in infant CF population in our region was 16.5%. We found no season predilection in its occurrence. Early infant age, breast-feeding, delayed CF diagnosis, heat exhaustion and the presence of severe CF transmembrane conductance regulator mutations are predisposed factors for the development of metabolic alkalosis with hypoelectrolytemia.


CONCLUSIONS
The results from our study suggest that metabolic alkalosis with hypoelectrolytemia is a relatively common manifestation of CF in infancy. The possibility of CF should be seriously considered in any infant with this metabolic disorder.
Fustik
Stojka
S
Department of Pediatrics, Clinical Center, Skopje, Republic of Macedonia. fustik@ukim.edu.mk
Pop-Jordanova
Nada
N
Slaveska
Nevenka
N
Koceva
Svetlana
S
Efremov
Gorgi
G
D000471:Alkalosis / Q000209:etiology*; D002712:Chlorides / Q000097:blood*; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics; D005260:Female; D005838:Genotype; D006801:Humans; D007008:Hypokalemia / Q000209:etiology*; D007010:Hyponatremia / Q000209:etiology*; D007223:Infant; D008297:Male; D012189:Retrospective Studies
D002712:Chlorides