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Hepatorenal syndrome

An Author Correction to this article was published on 15 October 2018

This article has been updated

Abstract

Hepatorenal syndrome (HRS) is a form of kidney function impairment that characteristically occurs in cirrhosis. Recent changes in terminology have led to acute HRS being referred to as acute kidney injury (AKI)-HRS and chronic HRS as chronic kidney disease (CKD)-HRS. AKI-HRS is characterized by a severe impairment of kidney function owing to vasoconstriction of the renal arteries in the absence of substantial abnormalities in kidney histology. Pathogenetic mechanisms involve disturbances in circulatory function due to a marked splanchnic arterial vasodilation, which triggers the activation of vasoconstrictor factors. An intense systemic inflammatory reaction that is characteristic of advanced cirrhosis may also be involved. The main triggering factors of AKI-HRS are bacterial infections, particularly spontaneous bacterial peritonitis. The diagnosis of AKI-HRS is a challenge because of a lack of specific diagnostic tools and mainly involves the differential diagnosis from other forms of AKI, particularly acute tubular necrosis. The prognosis of patients with AKI-HRS is poor, with a median survival of ≤3 months. The ideal treatment for AKI-HRS is liver transplantation in patients without contraindications. Medical therapy consists of vasoconstrictor drugs to counteract splanchnic arterial vasodilation together with volume expansion with albumin. Effective measures to prevent AKI-HRS include early identification and treatment of bacterial infections and the administration of albumin in patients with spontaneous bacterial peritonitis.

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Fig. 1: Factors involved in the pathogenesis of HRS.
Fig. 2: Inflammatory mediators and pathways that can affect the circulation.
Fig. 3: Algorithm for liver transplant alone versus simultaneous liver and kidney transplant in HRS.
Fig. 4: An algorithm for diagnosis and management of AKI in cirrhosis.

Change history

  • 15 October 2018

    The original version of this article omitted an initial from the name of contributing author Patrick S. Kamath, who was listed as Patrick Kamath. The article has now been corrected.

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Acknowledgements

Some of the work cited has been funded from public grants from Instituto de Salud Carlos III through the Plan Estatal de Investigación Científica y Técnica y de Innovación 2013–2016 (project reference PI 16/00043). This grant was co-funded by the European Regional Development Fund (FEDER), Agencia de Gestió d’Ajuts Universitaris I de Recerca (AGAUR) 2014/SGR 1281 and AGAUR 2017/SGR 1281 and the European Union (EU)-funded HORIZON 2020 project number 731875 (Acronym: LIVERHOPE). P.G. is a recipient of an ICREA Academia award. This review article is dedicated to the memory of Juan Rodés (1938–2017), who was a mentor and a friend.

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Introduction (P.G.); Epidemiology (M.K.N.); Mechanisms/pathophysiology (E.S.); Diagnosis, screening and prevention (E.S.); Management (P.A. and F.W.); Quality of life (P.S.K.); Outlook (P.G.); Overview of the Primer (P.G.).

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Correspondence to Pere Ginès.

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P.G. declares that he is a member of advisory boards for Ferring Pharmaceuticals, Intercept Pharmaceuticals, Martin Pharmaceuticals and Novartis. He has received research funds from Grifols and Sequana Medical AG. P.A. declares that he is a member of the advisory board for Sequana Medical AG and that he has no financial disclosures. F.W. declares grant support from and consultancy for Mallinckrodt and consultancy for Conatus and Ferring Pharmaceuticals. E.S., M.K.N. and P.S.K. declare no competing interests.

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Ginès, P., Solà, E., Angeli, P. et al. Hepatorenal syndrome. Nat Rev Dis Primers 4, 23 (2018). https://doi.org/10.1038/s41572-018-0022-7

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