ABSTRACT
In renal tubular acidosis (RTA), defective renal tubular function leads to metabolic acidosis, despite normal daily acid production from metabolism and dietary intake. RTA is typically characterized by hyperchloremic metabolic acidosis (HCMA). It can occur sporadically or as a heritable disorder, either as an autosomal dominant or recessive trait, or as part of a more generalized tubular disorder, such as Fanconi syndrome. In its early descriptions, RTA was dened by numerous terms, such as Lightwood disease and infantile tubular acidosis. Consensus around the classication of RTA into distal and proximal types emerged only aer demonstration of defects in urinary acidication and bicarbonate reabsorption in such patients.1
