pediatrics
Bartter Syndrome and Gitelman Syndrome
Treatment
For Bartter syndrome, nonsteroidal anti-inflammatory drugs (NSAIDs) Sodium, potassium, and magnesium supplements 5 to 10 mg/kg 3 times a day) ( Treatment references , , such as aspartate, citrate, or lactate, have better bioavailability. A high solute load, such as that resulting from sodium supplementation, should be avoided in patients who have a urinary concentrating defect, secondary Nephrogenic Diabetes Insipidus , or both because it exacerbates polyuria and polydipsia resulting from obligate water loss and could precipitate significant hypernatremia. In general, electrolyte supplementation should try to maintain adequate serum levels with minimal fluctuation, thus, dosing should be spread out as long as it does not significantly increase the risk of nonadherence. Although potassium-sparing diuretics, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers have been used in some patients, current consensus is that these therapies are largely unproved ( Treatment references , Thiazide diuretics for management of hypercalciuria are generally not recommended, but this may complicate sodium supplementation, which can worsen the risk of nephrolithiasis and nephrocalcinosis. Nutritional optimization is important, especially for infants and young children. Exogenous growth hormone can be considered to treat short stature. 1.Konrad M, Nijenhuis T, Ariceta G, et al: Diagnosis and management of Bartter syndrome: Executive summary of the consensus and recommendations from the European Rare Kidney Disease Reference Network Working Group for Tubular Disorders.Kidney Int99(2):324–335, 2021. doi: 10.1016/j.kint.2020.10.035 2.Konrad M, Nijenhuis T, Ariceta G, et al: Diagnosis and management of Bartter syndrome: Consensus and recommendations from the ERKNetWorking Group for Tubular Disorders. 2020. Accessed November 21, 2022. 