CLINICAL KIDNEY JOURNAL
Clinical Kidney Journal Advance Access published August 31, 2016 Clinical Kidney Journal, 2016, 1–3 doi: 10.1093/ckj/sfw085 Exceptional Case
EXCEPTIONAL CASE
Primary sclerosing cholangitis: a new cause of distal renal tubular acidosis
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Department of Nephrology, Dialysis and Transplantation, Hôpital Lapeyronie, University of Montpellier Medical School, Montpellier, France, 2Department of Hepato-Gastro-Enterology and Liver Transplantation, Hôpital Saint Eloi, University of Montpellier Medical School, Montpellier, France, 3SAS RD – Nephrology, Montpellier, France, 4 Department of Nephrology and Dialysis Saint-Guilhem, Sète, France and 5Department of Hypertension and Internal Medicine, Hôpital Lapeyronie, University of Montpellier Medical School, Montpellier, France Correspondence and offprint request to: Jean Ribstein; E-mail:
[email protected]
Abstract 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. Key words: distal renal tubular acidosis, liver transplantation, primary sclerosing cholangitis
Background Renal tubular acidosis is considered when a relatively normal glomerular filtration rate is associated with metabolic acidosis, hyperchloremia and a normal plasma anion gap [1]. Distal renal tubular acidosis (dRTA) is characterized by an impairment of net acid secretion by the distal tubule and may be associated with hypokalaemia (type I) or hyperkalemia (type IV). Nephrocalcinosis, recurrent renal calculi and reduced bone mineral density are often present. It can be either inherited, idiopathic or acquired secondary to a variety of conditions [1, 2]. For example, autosomal dominant or recessive mutations in the anion exchanger 1 (AE1) gene encoding the erythroid and kidney anion exchanger 1 (chloride-bicarbonate) may result in dRTA with ovalocytosis or spherocytosis in patients from Southeast Asia [3]. The most
common causes of secondary dRTA are autoimmune disorders such as systemic lupus erythematosus [4], Sjögren’s syndrome [5] and primary biliary cirrhosis [6]. Of note, primary sclerosing cholangitis (PSC) is often compared to primary biliary cirrhosis, but no PSC-associated dRTA has been previously reported. We report a case of type I dRTA occurring during the course of PSC that was corrected by a successful liver transplantation.
Case report A 26-year-old Lao-Thai male patient with uneventful previous medical records was diagnosed as severe (Child score B7, Model for End-Stage Liver Disease (MELD) score 24) hepatic cirrhosis secondary to PSC in August 2012. Biliary magnetic resonance imaging revealed an irregular aspect of intrahepatic bile ducts with
Received: September 29, 2015. Accepted: June 17, 2016 © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact
[email protected]
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Valentin Goutaudier1, Ilan Szwarc1, Jean-Emmanuel Serre1, Georges-Philippe Pageaux2, Àngel Argilés3,4 and Jean Ribstein5
CLINICAL KIDNEY JOURNAL
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| V. Goutaudier et al.
831 µmol/L (normal
