Although the definition of renal tubular acidosis (RTA) is simple, understa
nding the physiologic basis underlying the various types of this clinical e
ntity is much more difficult. The pathophysiology of this disorder is revie
wed using the normal acid-base functions of the involved segments of the ne
phron as a guide to understanding. Clinical and laboratory features of the
subtypes of RTA are addressed, and diagnosis and treatment discussed. New d
evelopments in the knowledge and understanding of the associated growth dis
turbances, mineral metabolism, and molecular biology of RTA are also review
ed to provide the most current view of this relatively common pediatric ent
ity.