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United European Gastroenterol J. 2017 Aug;5(5):658-667. doi: 10.1177/2050640616680550. Epub 2016 Nov 16.

Nodular regenerative hyperplasia rarely leads to liver transplantation: A 20-year cohort study in all Dutch liver transplant units.

United European gastroenterology journal

Berrie Meijer, Melek Simsek, Hans Blokzijl, Robert A de Man, Minneke J Coenraad, Gerard Dijkstra, Carin Mj van Nieuwkerk, Chris Jj Mulder, Nanne Kh de Boer

Affiliations

  1. Department of Gastroenterology and Hepatology, VU University Medical Center Amsterdam, The Netherlands.
  2. Department of Gastroenterology and Hepatology, University Medical Center Groningen, Groningen, The Netherlands.
  3. Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands.
  4. Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, The Netherlands.

PMID: 28815029 PMCID: PMC5548351 DOI: 10.1177/2050640616680550

Abstract

BACKGROUND: Nodular regenerative hyperplasia is an uncommon liver condition associated with several autoimmune disorders and drugs. The clinical symptoms of nodular regenerative hyperplasia vary from asymptomatic to severe complications of portal hypertension (nodular regenerative hyperplasia-syndrome).

OBJECTIVE: The purpose of this study was to identify the prognosis and optimal management, as well as the role of liver transplantation, in nodular regenerative hyperplasia.

METHODS: The pathology databases of all three Dutch liver transplant units were retrospectively scrutinised for explanted livers diagnosed with nodular regenerative hyperplasia or without clear diagnosis. Pre- and post-transplantation clinical, biochemical, radiological and histological information was obtained from electronic and paper records.

RESULTS: In total, 1886 patients received a liver transplant. In 255 patients, nodular regenerative hyperplasia could not be excluded. After detailed chart review, the native livers of 11 patients (0.6%) (82% male, median age: 44 years) displayed nodular regenerative hyperplasia. Seven patients (64%) had underlying disorders or drug exposure which possibly caused nodular regenerative hyperplasia. Laboratory and imaging abnormalities were present in all patients but did not contribute to the diagnosis of nodular regenerative hyperplasia. Five-year survival was 73% (median follow-up: four years, range: 2-248 months).

CONCLUSION: Nodular regenerative hyperplasia is a rare finding in patients, predominantly young males, transplanted for end-stage liver disease with unknown aetiology. Nonetheless, liver transplantation may have an important role in end-stage nodular regenerative hyperplasia-syndrome.

Keywords: Liver transplantation; drug-induced liver injury; hepatotoxicity; nodular regenerative hyperplasia; portal hypertension

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