| Literature DB >> 30454738 |
Rosanna Fulchiero1, Patricia Seo-Mayer2.
Abstract
Bartter and Gitelman syndromes are conditions characterized by renal salt-wasting. Clinical presentations range from severe antenatal disease to asymptomatic with incidental diagnosis. Hypokalemic hypochloremic metabolic alkalosis is the common feature. Bartter variants may be associated with polyuria and weakness. Gitelman syndrome is often subtle, and typically diagnosed later life with incidental hypokalemia and hypomagnesemia. Treatment may involve fluid and electrolyte replenishment, prostaglandin inhibition, and renin-angiotensin-aldosterone system axis disruption. Investigators have identified causative mutations but genotypic-phenotypic correlations are still being characterized. Collaborative registries will allow improved classification schema and development of effective treatments.Entities:
Keywords: Bartter syndrome (BS); Failure to thrive; Gitelman syndrome (GS); Hypokalemic hypochloremic metabolic alkalosis; Polyuria; Salt-losing tubulopathy
Mesh:
Year: 2019 PMID: 30454738 DOI: 10.1016/j.pcl.2018.08.010
Source DB: PubMed Journal: Pediatr Clin North Am ISSN: 0031-3955 Impact factor: 3.278