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Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience
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2009 (English)In: Liver international (Print), ISSN 1478-3223, E-ISSN 1478-3231, Vol. 29, no 2, 253-259 p.Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: The exact incidence and prevalence of Budd-Chiari syndrome (BCS) is unknown in the general population. Published reports differ in terms of the clinical characteristics, effects of therapy and survival. AIMS: To investigate the epidemiology, clinical presentation and survival in patients with BCS. METHODS: Retrospective multicentre study in Sweden reviewing the medical records of all patients with BCS 1986-2003, identified from the computerised diagnosis database of 11 hospitals, including all university hospitals and liver transplantation centres. RESULTS: Forty-three patients with BCS were identified, of whom nine (21%) had concomitant portal vein thrombosis. The mean age-standardised incidence and prevalence rates in 1990-2001 were calculated to be 0.8 per million per year and 1.4 per million inhabitants respectively. Myeloproliferative disorders (38%), thrombophilic factors (31%) and oral contraceptives (30%) were common aetiological factors. Two or more risk factors were present in 44%. In 23%, no risk factor was evident. The median follow-up time was 2.7 years. Seventy-two percent were on anticoagulant therapy during follow-up. Transjugular intrahepatic portosystemic shunting, surgical shunting procedures and liver transplantation were performed in 4, 6 and 18 patients respectively. Nineteen patients died. The overall transplantation-free survival at 1, 5 and 10 years was 47, 28 and 17% respectively. CONCLUSIONS: Budd-Chiari syndrome is a rare disorder; the mean age-standardised incidence and prevalence rates in Sweden in 1990-2001 were calculated to be 0.8 per million per year and 1.4 per million inhabitants respectively. The presence of a myeloproliferative disorder was a common aetiological factor in our cohort and about half of the patients had a multifactorial aetiology. The transplantation-free survival was poor.

Place, publisher, year, edition, pages
Oxford: Blackwell Munksgaard , 2009. Vol. 29, no 2, 253-259 p.
National Category
Medical and Health Sciences Gastroenterology and Hepatology
Research subject
Medicine
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URN: urn:nbn:se:oru:diva-6968DOI: 10.1111/j.1478-3231.2008.01838.xPubMedID: 18694401OAI: oai:DiVA.org:oru-6968DiVA: diva2:219486
Available from: 2009-05-27 Created: 2009-05-27 Last updated: 2017-12-13Bibliographically approved

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Gustavsson, Anders

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