Renal Tubular Acidosis (2017)

1. Jonathan Pelletier, MD*
2. Rasheed Gbadegesin, MD, MBBS†
3. Betty Staples, MD*


 

1. *Department of Pediatrics and

2. †Department of Pediatric Nephrology, Duke Children's Hospital and Health Center, Durham, NC

1. 1. Reddy P
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 [OpenUrl][1][CrossRef][2][PubMed][3]

2. 1. Batlle D, 2. Haque SK
 Genetic Causes and Mechanisms of Distal Renal Tubular Acidosis. Batlle D, Haque SK. Nephrol Dial Transplant. 2012;27(10):3691–3704
 [OpenUrl][4][CrossRef][5][PubMed][6][Web of Science][7]

3. 1. Karet FE
 Mechanisms in Hyperkalemic Renal Tubular Acidosis. Karet FE. J Am Soc Nephrol. 2009;20(2):251–254
 [OpenUrl][8][Abstract/FREE Full Text][9]

4. 1. Haque SK, 2. Ariceta G, 3. Batlle D
 Proximal Renal Tubular Acidosis: A Not So Rare Disorder of Multiple Etiologies. Haque SK, Ariceta G, Batlle D Nephrol Dial Transplant. 2012;27(12):4273–4287
 [OpenUrl][10][CrossRef][11][PubMed][12][Web of Science][13]

5. 1. Gbadegesin R, 2. Foreman W
 1. Chand DH, 2. Valentini RP
 Renal Tubular Acidosis. Gbadegesin R, Foreman W In: Chand DH, Valentini RP, eds. Clinician's Manual of Pediatric Nephrology. 1st ed. Singapore: World Scientific Publishing Co; 2011
 

The body temporarily buffers hydrogen ions (H+) with plasma proteins, hemoglobin, and bicarbonate (HCO3−), but H+ must be excreted to prevent acidosis. The major functions of the kidneys in acid-base homeostasis are to excrete H+ and reabsorb HCO3−. Failure to perform these functions results in HCO3− wasting, leading to renal tubular acidosis (RTA), which is categorized into 3 major groups: distal (type I), proximal (type II), and hyperkalemic (type IV) RTA.

Excretion of H+ to balance acid production is primarily the function of the distal convoluted tubule and collecting duct (DCT/CD), where α-intercalated cells actively transport H+ into the tubule. Secreted H+ combines with ammonia to form ammonium in the DCT/CD lumen. Because of its positive charge, ammonium cannot diffuse out of the tubular lumen, and it is passed in the urine.

In distal RTA, α-intercalated cells cannot secrete sufficient H+ into the tubular lumen, resulting in decreased …

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