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Episodic seasonal Pseudo-Bartter syndrome in cystic fibrosis
Brett Kintu1, Alex Brightwell1
1Department of Paediatrics, Norfolk and Norwich University Hospital.
Insights
Pseudo-Bartter syndrome (PBS) is a rare cystic fibrosis complication causing electrolyte imbalances. Clinicians should consider PBS in unwell cystic fibrosis patients during warmer months, especially with climate change.
Area of Science:
- Pediatric Nephrology
- Pulmonology
- Clinical Medicine
Background:
- Pseudo-Bartter syndrome (PBS) is an uncommon complication of cystic fibrosis (CF).
- It presents as hypochloraemic, hypokalaemic metabolic alkalosis.
- PBS is typically observed in infants and young children with CF.
Abstract:
Pseudo-Bartter syndrome (PBS) describes an uncommon but well recognised complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. Pseudo-Bartter syndrome is usually seen at initial presentation or within the first two years of life in children with cystic fibrosis. Risk factors for development of PBS include warm weather conditions, severe respiratory or pancreatic disease and gastrointestinal losses (e.g. vomiting and diarrhoea). PBS is rare in older children and adolescents although epidemics have been associated with heat wave conditions in warmer climates. In this era of climate change, it is crucial that clinicians consider Pseudo-Bartter syndrome when patients with cystic fibrosis present unwell during summer.
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