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Clinical features and treatment approaches in cystic fibrosis with pseudo-Bartter syndrome

Ebru Yalçin1, Nural Kiper, Deniz Doğru

  • 1Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. ebruy@hacettepe.edu.tr

Annals of Tropical Paediatrics
|June 14, 2005
PubMed

Insights

Cystic fibrosis (CF) infants can develop pseudo-Bartter syndrome (PB), a condition causing dehydration and metabolic alkalosis. Early recognition of symptoms like vomiting and loss of appetite is crucial for timely treatment and preventing complications.

Area of Science:

  • Pediatrics
  • Nephrology
  • Genetics

Background:

  • Infants with cystic fibrosis (CF) are susceptible to pseudo-Bartter syndrome (PB), characterized by hyponatremic, hypochloremic dehydration and metabolic alkalosis.
  • PB in CF infants presents with specific biochemical hallmarks requiring careful diagnostic consideration.

Purpose of the Study:

  • To review the clinical and laboratory features of CF patients diagnosed with PB.
  • To analyze treatment approaches and outcomes for children with CF and PB.

Main Methods:

  • Retrospective review of 29 children with CF and PB from May 1992 to November 2003.
  • Analysis of clinical presentations, laboratory findings, and management strategies.

Main Results:

  • PB was detected in 12% (29/241) of CF patients, with a median age of 4 months at the first episode.
  • Common symptoms included vomiting, loss of appetite, and dehydration; all patients received intravenous fluids and sodium chloride.
  • 12 out of 29 patients required hospitalization for recurrent PB attacks, with the last attack occurring by 48 months of age.

Conclusions:

  • Cystic fibrosis should be considered in the differential diagnosis of metabolic alkalosis in young children.
  • Vomiting and loss of appetite are key warning signs for PB in CF patients, especially before age 4.
  • Early recognition of PB by parents and physicians is vital for preventing severe complications.
Abstract

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