Hereditary Distal Renal Tubular Acidosis: Genotypic Correlation, Evolution to Long Term, and New Therapeutic Perspectives.

Distal renal tubular acidosis (DRTA) is a rare disease resulting from a failure in the normal urine acidification process at the distal tubule and collecting duct level. It is characterised by persistent hyperchloremic metabolic acidosis, with a normal anion gap in plasma, in the presence of high urinary pH and low urinary excretion of ammonium.