Publication: Distal renal tubular acidosis and high urine carbon dioxide tension in a patient with Southeast Asian ovalocytosis
Issued Date
1999-01-01
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ISSN
02726386
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2-s2.0-0033017395
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Mahidol University
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SCOPUS
Bibliographic Citation
American Journal of Kidney Diseases. Vol.33, No.6 (1999), 1147-1152
Suggested Citation
C. Kaitwatcharachai, S. Vasuvattakul, P. T. Yenchitsomanus, P. Thuwajit, P. Malasit, D. Chuawatana, S. Mingkum, M. L. Halperin, P. Wilairat, S. Nimmannit Distal renal tubular acidosis and high urine carbon dioxide tension in a patient with Southeast Asian ovalocytosis. American Journal of Kidney Diseases. Vol.33, No.6 (1999), 1147-1152. doi:10.1016/S0272-6386(99)70154-X Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/25696
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Title
Distal renal tubular acidosis and high urine carbon dioxide tension in a patient with Southeast Asian ovalocytosis
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Abstract
Southeast Asian ovalocytosis (SAO) is the best-documented disease in which mutation in the anion exchanger-1 (AE1) causes decreased anion (chloride [Cl-]/bicarbonate [HCO3-]) transport. Because AE1 is also found in the basolateral membrane of type A intercalated cells of the kidney, distal renal tubular acidosis (dRTA) might develop if the function of AE1 is critical for the net excretion of acid. Studies were performed in a 33-year- old woman with SAO who presented with proximal muscle weakness, hypokalemia (potassium, 2.7 mmol/L), a normal anion gap type of metabolic acidosis (venous plasma pH, 7.32; bicarbonate, 17 mmol/L; anion gap, 11 mEq/L), and a low rate of ammonium (NH4+) excretion in the face of metabolic acidosis (26 μmol/min). However, the capacity to produce NH4+did not appear to be low because during a furosemide-induced diuresis, NH4+excretion increased almost threefold to a near-normal value (75 μmol/L/min). Nevertheless, her minimum urine pH (6.3) did not decrease appreciably with this diuresis. The basis of the renal acidification defect was most likely a low distal H+secretion rate, the result of an alkalinized type A intercalated cell in the distal nephron. Unexpectedly, when her urine pH increased to 7.7 after sodium bicarbonate administration, her urine minus blood carbon dioxide tension difference (U-B PCO2) was 27 mm Hg. We speculate that the increase in U-B PCO2might arise from a misdirection of AE1 to the apical membrane of type A intercalated cells.