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 |
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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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