Abstract

Renal tubular acidosis is a constellation of syndromes arising from different derangements of tubular acid transport. Recent advances in the biology of urinary acidification have allowed us to discern various molecular mechanisms responsible for these syndromes. This report relates clinical disorders of acidification to the underlying defective mechanisms responsible for them. A clinical classification of these disorders is presented, integrating each disorder with the prevailing serum potassium concentration. That renal tubular acidosis can be associated with low, normal, or high serum potassium concentration is now explainable by identifying the specific defect in transport causing each syndrome.