Clinical approach to renal tubular acidosis in children

Renal tubular acidosis (RTA) is a common inherited tubulopathy in children. Proximal RTA, usually secondary to a systemic metabolic disease, is characterized by a generalized dysfunction of the proximal tubule resulting in Fanconi syndrome. Distal RTA occurs due to mutation in the transporters of the distal tubule resulting in acidification defects. Hyperchloremic metabolic acidosis with normal anion gap is the characteristic feature of RTA. In addition to supportive therapy, specific treatment for the underlying etiology and regular monitoring of growth and laboratory parameters are of utmost importance.