Clinical features and treatment approaches in cystic fibrosis with pseudo-Bartter syndrome.
BACKGROUND
Infants with cystic fibrosis (CF) are prone to develop episodes of hyponatraemic, hypochloraemic dehydration with metabolic alkalosis, which are biochemical hallmarks of the pseudo-Bartter syndrome (PB).


METHODS
We reviewed the clinical and laboratory features and treatment approaches of 29 children with CF and PB who were under follow-up in our institution from May 1992 to November 2003.


RESULTS
Of 241 patients with CF, PB was detected in 29 (12%) with a median age of 4 months at the time of the first attack. Most patients experienced vomiting, loss of appetite and dehydration during episodes of PB. All patients were managed with intravenous fluids and sodium chloride solutions. During follow-up, 12/29 cases required hospital admission for recurrent PB attacks. The oldest age at the time of the last attack was 48 months.


CONCLUSIONS
CF should be considered in the differential diagnosis of metabolic alkalosis in young children. Vomiting and loss of appetite are important warning signs of possible PB in CF patients, particularly before 4 years of age. To prevent serious complications, it is crucial that parents and physicians recognise PB as early as possible.
Yalçin
Ebru
E
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. ebruy@hacettepe.edu.tr
Kiper
Nural
N
Doğru
Deniz
D
Ozçelik
Uğur
U
Aslan
Ayşe Tana
AT
D000293:Adolescent; D000328:Adult; D001477:Bartter Syndrome / Q000150:complications* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000628:therapy*; D003937:Diagnosis, Differential; D001068:Feeding and Eating Disorders / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D015532:Rehydration Solutions / Q000008:administration & dosage; D012189:Retrospective Studies; D012965:Sodium Chloride / Q000008:administration & dosage; D016896:Treatment Outcome; D014839:Vomiting / Q000209:etiology
D015532:Rehydration Solutions; D012965:Sodium Chloride