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A Case of Distal Renal Tubular Acidosis

Journal of the Korean Pediatric Society 1994;37(1):115-112.
Published online January 15, 1994.
A Case of Distal Renal Tubular Acidosis
Bin Cho, Dong Sung Kim, Byung Churl Lee
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
원위형 신세뇨관 산혈증 1례
조빈, 김동성, 이병철
가톨릭대학교 의과대학 소아과학교실
Abstract
Distal renal tubular acidosis (RTA) is a biochemical syndrome as a dificiency of hydrogen ion secretion by the distal tubule and collecting duct. Owing to the nature of the defect, hyperchloremic non-anion gap metabolic acidosis and high urine pH despite severe systemic acidosis is characterized. Typical manifestations of distal RTA are growth retardation, rickets, polyuria and nephrocalcinosis. We experienced a case of distal renal tubular acidosis in a 4 years old female child who comlained of growth retardation an gait disturbance. She showed typical hyperchloremic non-anion gap metabolic acidosis and persistent high urine pH. In radiographic examination. We found delayed bone age and severe rachitic changes of wrist, elbow, and knee as well as nephrocalcinosis. In ammonium chloride loading test, high urine pH persisted despite the lowering blood HCO3- concentration. After alkali therapy, she is in well condition with excellent growth velocity and absence of rachitic changes.
Key Words: Distal renal tubular acidosis, Hyperchloremic metabolic acidosis


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