J Korean Soc Pediatr Nephrol > Volume 14(2); 2010 > Article
J Korean Soc Pediatr Nephrol 2010;14(2): 120-131. doi: https://doi.org/10.3339/jkspn.2010.14.2.120
신세뇨관 산증
박혜원
분당서울대학교병원 소아청소년과
Renal Tubular Acidosis
Hye-Won Park
Department of Pediatrics, Seoul National University Bundang Hospital
Received: September 20, 2010;  Revised: September 20, 2010.  Accepted: September 29, 2010.
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ABSTRACT
Renal tubular acidosis (RTA) is a metabolic acidosis due to impaired excretion of hydrogen ion, or reabsorption of bicarbonate, or both by the kidney. These renal tubular abnormalities can occur as an inherited disease or can result from other disorders or toxins that affect the renal tubules. Disorders of bicarbonate reclamation by the proximal tubule are classified as proximal RTA, whereas disorders resulting from a primary defect in distal tubular net hydrogen secretion or from a reduced buffer trapping in the tubular lumen are called distal RTA. Hyperkalemic RTA may occur as a result of aldosterone deficiency or tubular insensitivity to its effects. The clinical classification of renal tubular acidosis has been correlated with our current physiological model of how the nephron excretes acid, and this has facilitated genetic studies that have identified mutations in several genes encoding acid and base ion transporters. Growth retardation is a consistent feature of RTA in infants. Identification and correction of acidosis are important in preventing symptoms and guide approved genetic counseling and testing.
Key words: Renal tubular acidosis | NBC1 | NHE-3 | AE1 | Proton pump
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