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Renal Tubular Acidosis

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Pediatric Kidney Disease

Abstract

A hyperchloremic non-anion gap metabolic acidosis, in the absence of grastrointestinal bicarbonate loss, is due to either renal loss of bicarbonate or an inability of the kidney to excrete protons in the urine. This chapter outlines renal tubular mechanisms mediating bicarbonate reabsorption by the nephron and acid secretion by the kidney and the pathologies that disturb them. Bicarbonate reabsorption predominantly occurs in the proximal tubule while acid secretion takes place in the distal nephron. Consequently, specific pathologies, which prevent bicarbonate reclamation and lead to a metabolic acidosis are referred to as proximal renal tubular acidosis (pRTA). Conversely, a failure of the kidney to excrete acid is known as distal renal tubular acidosis (dRTA). These forms of renal tubular acidosis are typically accompanied by hypokalemia. In the distal nephron acid secretion is facilitated by the electrogenic reabsorption of sodium in response to aldosterone, a process that also permits potassium secretion. Consequently actual or functional absence of aldosterone leads to a hyperkalemic metabolic acidosis. After identifying the clinical presentation of these types of disorders, with particular emphasis on genetic causes of RTA, we highlight a diagnostic approach to and treatment for them.

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  1. Department of Pediatrics and Physiology, Stollery Children’s Hospital, 11405-87 Avenue, Edmonton, Alberta, T6G 1C9, Canada

    R. Todd Alexander

  2. Department of Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London, WC1N 3JH, UK

    Detlef Bockenhauer

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  1. Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada

    Denis F. Geary

  2. Division of Pediatric Nephrology, University of Heidelberg, Heidelberg, Germany

    Franz Schaefer

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Alexander, R.T., Bockenhauer, D. (2016). Renal Tubular Acidosis. In: Geary, D., Schaefer, F. (eds) Pediatric Kidney Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-52972-0_36

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