Berrie Meijer1, Melek Simsek1, Hans Blokzijl2, Robert A de Man3, Minneke J Coenraad4, Gerard Dijkstra2, Carin Mj van Nieuwkerk1, Chris Jj Mulder1, Nanne Kh de Boer1. 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.
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.
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.
Authors: J S Radomski; K A Chojnacki; M J Moritz; R Rubin; V T Armenti; G A Wilson; S Herrine; M Conn Journal: Am Surg Date: 2000-11 Impact factor: 0.688
Authors: Erik H C J Buster; Hanneke J van Vuuren; Pieter E Zondervan; Herold J Metselaar; Hugo W Tilanus; Robert A de Man Journal: Eur J Gastroenterol Hepatol Date: 2008-01 Impact factor: 2.566
Authors: N K H de Boer; P E Zondervan; L P L Gilissen; G den Hartog; B D Westerveld; L J J Derijks; E Bloemena; L G J B Engels; A A van Bodegraven; C J J Mulder Journal: Dig Liver Dis Date: 2007-12-20 Impact factor: 4.088