liu.seSearch for publications in DiVA
Change search
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • harvard1
  • ieee
  • modern-language-association-8th-edition
  • vancouver
  • oxford
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf
The epidemiology and clinical features of portal vein thrombosis: a multicentre study
Linköping University, Department of Clinical and Experimental Medicine. Linköping University, Faculty of Health Sciences.
Sahlgrenska University Hospital, Göteborg.
Karolinska University Hospital, Stockholm.
Umeå University Hospital, Umeå.
Show others and affiliations
2010 (English)In: ALIMENTARY PHARMACOLOGY and THERAPEUTICS, ISSN 0269-2813, Vol. 32, no 9, 1154-1162 p.Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: Reliable epidemiological data for portal vein thrombosis are lacking.

AIMS: To investigate the incidence, prevalence and survival rates for patients with portal vein thrombosis.

METHODS: Retrospective multicentre study of all patients registered with the diagnosis of portal vein thrombosis between 1995 and 2004.

RESULTS: A total of 173 patients (median age 57 years, 93 men) with portal vein thrombosis were identified and followed up for a median of 2.5 years (range 0-9.7). The mean age-standardized incidence and prevalence rates were 0.7 per 100,000 per year and 3.7 per 100,000 inhabitants, respectively. Liver disease was present in 70 patients (40%), malignancy in 27%, thrombophilic factors in 22% and myeloproliferative disorders in 11%. Two or more risk factors were identified in 80 patients (46%). At diagnosis, 65% were put on anticoagulant therapy. Thrombolysis, TIPS, surgical shunting and liver transplantation were performed in 6, 3, 2 and 8 patients, respectively. The overall survival at 1 year and 5 years was 69% and 54%. In the absence of malignancy and cirrhosis, the survival was 92% and 76%, respectively.

CONCLUSIONS: The incidence and prevalence rates of portal vein thrombosis were 0.7 per 100,000 inhabitants per year and 3.7 per 100,000 inhabitants, respectively. Concurrent prothrombotic risk factors are common. The prognosis is variable and highly dependent on underlying disease.

Place, publisher, year, edition, pages
Blackwell Publishing Ltd , 2010. Vol. 32, no 9, 1154-1162 p.
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:liu:diva-60716DOI: 10.1111/j.1365-2036.2010.04454.xISI: 000282570800012OAI: oai:DiVA.org:liu-60716DiVA: diva2:359784
Available from: 2010-10-29 Created: 2010-10-22 Last updated: 2011-05-31Bibliographically approved
In thesis
1. Hepatic and Portal Vein Thrombosis: studies on epidemiology and risk factors
Open this publication in new window or tab >>Hepatic and Portal Vein Thrombosis: studies on epidemiology and risk factors
2011 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Budd-Chiari syndrome (BCS) i.e. thrombosis in the hepatic veins and/or inferior vena cava, and portal vein thrombosis (PVT) are rare disorders. Epidemiological data are scarce and previous reports have been from highly specialised referral centres.

The aims of the thesis were: (i) to investigate the epidemiology, clinical features and survival of Swedish patients with BCS or PVT, and to (ii) determine common underlying risk factors i.e. thrombophilic factors and genetic markers of myeloproliferative disorders (MPD).

In the first two papers we retrospectively reviewed the medical records of all BCS (1986-2003) and PVT (1995-2004) patients identified by searching the computerized patient registers of 11 hospitals including all university hospitals and liver transplantation units. In the following two papers we excluded patients with malignancy and included new cases diagnosed during the years 2004-2009; blood samples were collected and compared with controls and other patient groups.

A total of 43 patients with BCS were identified (median age 40 years, 24 women). The mean agestandardised incidence and prevalence rates were 0.8 per million per year and 1.4 per million inhabitants respectively. Two or more risk factors were present in 44%. The overall transplantationfree survival at 1, 5 and 10 years was 47%, 28% and 17% respectively.

173 patients (median age 57 years, 93 men) with portal vein thrombosis were identified. The incidence and prevalence rates were 0.7 per 100 000 per year and 3.7 per 100 000 inhabitants, respectively. In the absence of cirrhosis and malignancy, being the most common risk factors, the survival at 1 year and 5 years was 92% and 76%, respectively.

We observed an increased plasma level of the procoagulant factor VIII in BCS (mean 1.63 kIE/L), PVT without cirrhosis (1.87 kIE/L), PVT with cirrhosis (1.97 kIE/L), deep vein thrombosis (1.41 kIE/L) and cirrhosis patients alone (2.22 kIE/L), all p <0.001 compared to healthy controls (1.04 kIE/L). Elevated factor VIII levels were found in 50% of BCS and in 85% of PVT patients without previously identified prothrombotic risk factors.

The somatic JAK2 V617F-mutation, a marker of MPD, was present in 63% of BCS and 14% of PVT patients. The frequency of the germline JAK2 46/1 haplotype was significantly higher in BCS (53%) and PVT (36%) patients compared to controls (27%) (p=0.02). However, the enrichment was only observed in JAK2 V617F positive patients.

Conclusions: The incidence and prevalence rates of BCS in Sweden were calculated to be 0.8/million inhabitants per year and 1.4/million inhabitants, respectively. The rates of PVT were higher; 0.7/100 000 inhabitants per year and 3.7/100 000 inhabitants, respectively. In BCS the transplantation-free survival was poor, whereas in PVT the survival was variable and highly dependent on the presence of underlying disease. Concurrent prothrombotic risk factors are common in both disorders. High plasma levels of procoagulant factor VIII was observed in a majority of idiopathic BCS and PVT. The prevalence of the somatic JAK2 V617F mutation was high in our cohort and associated with the presence of a germline JAK2 46/1 haplotype.

Place, publisher, year, edition, pages
Linköping: Linköping University Electronic Press, 2011. 70 p.
Series
Linköping University Medical Dissertations, ISSN 0345-0082 ; 1241
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:liu:diva-68727 (URN)978-91-7393-187-8 (ISBN)
Public defence
2011-06-09, Berzeliussalen, Campus US, Linköpings universitet, Linköping, 13:00 (English)
Opponent
Supervisors
Available from: 2011-05-31 Created: 2011-05-31 Last updated: 2012-03-22Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full text

Authority records BETA

Rajani, RupeshAlmer, Sven

Search in DiVA

By author/editor
Rajani, RupeshAlmer, Sven
By organisation
Department of Clinical and Experimental MedicineFaculty of Health SciencesGastroenterology and HepatologyDepartment of Endocrinology and Gastroenterology UHL
Medical and Health Sciences

Search outside of DiVA

GoogleGoogle Scholar

doi
urn-nbn

Altmetric score

doi
urn-nbn
Total: 73 hits
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • harvard1
  • ieee
  • modern-language-association-8th-edition
  • vancouver
  • oxford
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf