Distal renal tubular acidosis (dRTA) presents with low plasma bicarbonate and concomitant hypokalaemia. Severity at diagnosis varies — from marked acidosis to cases detected through family screening with near-normal bicarbonate levels. Management must be adapted to the patient’s age and biochemical status at presentation, from infancy through adulthood.
First-line management centres on alkali supplementation. The choice of alkali formulation is guided by the patient’s potassium status; depending on the formulation selected, separate electrolyte supplementation may also be required. Alongside pharmacological treatment, dietary measures that reduce acid load are recommended.
Full dosing algorithm, formulation selection, monitoring schedule, and complete age-specific regimen available in the structured protocol →The objective is to maintain plasma bicarbonate (HCO₃⁻), chloride (Cl⁻), and potassium (K⁺), as well as urinary calcium excretion, within age-appropriate normal ranges. In infants, plasma bicarbonate should be monitored closely and doses adjusted until a stable normal level is reached.
DOI: 10.1093/ndt/gfab171