Biochemical and genetic advances in distal renal tubular acidosis

Distal 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 article relates clinical disorders of distal acidification to the underlying defective mechanisms responsible for them. A clinical classification of these disorders is presented which integrates each disorder with the prevailing serum potassium concentration. That distal 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.