Table 2.

Treatment options and clinical controversies in Bartter syndrome

Drugs	Rationale for using	Limitations and clinical controversies
KCl supplements	• Correction of hypokalemia	• Hypokalemia usually persists but less pronounced
Spironolactone/eplerenone (aldosterone receptor blockers)	• K+-sparing diuretics (help correction of hypokalemia)	• Aldosterone levels could be lower because of hypokalemia • Gynecomastia can limit spironolactone use
Amiloride (ENaC blocker)	• K+-sparing diuretics (help correction of hypokalemia)	• Could work better than spironolactone and eplerenone to raise serum K+ levels and reverse metabolic alkalosis
ACEi and ARB	• Help to correct hypokalemia • Reduce proteinuria if present	• Caution is necessary due to the risk of hypotension and AKI
NSAIDs	• Reduce urinary volume helping to further correct hypokalemia	• Gastrointestinal side effects • Potential nephrotoxicity • Not established which NSAID provides best efficacy/less side effects • Gradual discontinuation during school age or lifelong maintenance? • Potential risks vs benefits of antenatal treatment

Abbreviations: ACEi, angiotensin-converting enzyme inhibitor; AKI, acute kidney injury; ARB, angiotensin receptor blocker; ENaC, epithelial Na⁺ channel; KCl, potassium chloride; NSAIDs, nonsteroidal anti-inflammatory drugs.