Long-term complications of primary distal renal tubular acidosis

• Published in: Pediatric nephrology (Berlin, West) Vol. 38; no. 3; pp. 635 - 642
• Main Authors: Santos, Fernando, Gil-Peña, Helena
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-03-2023; Springer; Springer Nature B.V
• Subjects: Acidification; Acidosis; Acidosis, Renal Tubular - complications; Acidosis, Renal Tubular - diagnosis; Acidosis, Renal Tubular - genetics; Adult; Adults; Bone growth; Calcinosis; Calcium phosphates; Care and treatment; Children; Complications and side effects; Cysts; Failure to thrive; Genes; Genetic aspects; Growth hormones; Humans; Hypokalemia; Hypokalemia - etiology; Kidney diseases; Lithiasis; Medicine; Medicine & Public Health; Metabolism; Mineralization; Mutation; Nephrocalcinosis - complications; Nephrocalcinosis - therapy; Nephrology; Pediatrics; Potassium; Renal failure; Renal Insufficiency - complications; Renal tubular acidosis; Review; Services; Urinary tract diseases; Urine; Urology; Spain; Hypokalemia; Distal renal tubular acidosis; Kidney failure; Growth; Metabolic acidosis
• ISSN: 0931-041X; 1432-198X; 1432-198X
• DOI: 10.1007/s00467-022-05546-w

The clinical manifestations of primary distal renal tubular acidosis usually begin in childhood, but the disease is caused by a genetic defect that persists throughout life. This review focuses on the complications of distal tubular acidosis that occur or remain long-term such as nephrocalcinosis and urolithiasis, growth impairment, bone mineralization, severe hypokalemia, kidney cysts, and progressive kidney failure, as well as other persistent manifestations that occur independent of acidosis but are associated with some inherited forms of the disease. The pathogenic factors responsible for kidney failure are discussed in particular because it is a complication to which different publications have recently drawn attention and which affects a high percentage of adults with primary distal renal tubular acidosis. The need to maintain optimal metabolic control of the disease and scheduled clinical follow-up throughout life and the importance of organizing protocols for the transition of patients to adult nephrology services are emphasized.