Journal
CLINICAL KIDNEY JOURNAL
Volume 9, Issue 6, Pages 811-813Publisher
OXFORD UNIV PRESS
DOI: 10.1093/ckj/sfw085
Keywords
distal renal tubular acidosis; liver transplantation; primary sclerosing cholangitis
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We describe the first case of distal renal tubular acidosis (dRTA) associated with primary sclerosing cholangitis. A 26-year-old Lao-Thai male patient presented with severe jaundice, metabolic acidosis and hypokalaemia. He was diagnosed of dRTA. Liver transplantation resulted in correction of electrolyte disturbances and hyperbilirubinaemia. A fludrocortisone-furosemide test revealed normal urinary acidification, demonstrating no residual dRTA. This observation suggests that dRTA may be an early manifestation of bilirubin-associated nephropathy or the consequence of an immune mechanism.
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