Primary sclerosing cholangitis: a new cause of distal renal tubular acidosis

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.

Source: CKJ: Clinical Kidney Journal - December 16, 2016 Category: Urology & Nephrology Authors: Goutaudier, V., Szwarc, I., Serre, J.-E., Pageaux, G.-P., Argiles, A., Ribstein, J. Tags: RARE DISEASES Source Type: research