PNH and Splanchnic Vein Thrombosis
Data from patients with PNH and splanchnic vein thrombosis from the European vascular disease interest group VALDIG and French vascular network will be retrospectively reviewed. Only patients with a diagnosis of splanchnic vein thrombosis made between 2000 and 2016 will be included.
We will compare the evolution of thrombosis in patients who received a “curative” treatment for PNH, including eculizumab and bone marrow transplantation, to patients who did not receive one of these 2 types of treatments, either because it was not available or when the patient did not have HLA compatible donor.
Vascular diseases of the liver are a heterogeneous group of rare and if untreated fatal disorders that include portal vein thrombosis, Budd-Chiari syndrome, non-cirrhotic portal hypertension, sinusoidal obstruction syndrome, hereditary hemorrhagic teleangiectasia and others.
Current knowledge about patients suffering from these disorders is insufficient and no biological samples are available to study the mechanisms of these diseases and the effects of treatments.
Therefore, with the support of a grant from EASL, we are running this cohort study by including and following up patients on the platform redcap.ctu.unibe.ch. If you are member of VALDIG you can request a password and username (contact email@example.com) to participate to this study by including patients from your center into the database (a complete set of data) or into the registry (a limited set of data).
- To characterize the population with vascular liver disease in Switzerland by defining history, outcome and risk factors.
- To build a bio-bank with blood from these patients.
Idiopathic portal hypertension, non-cirrhotic portal fibrosis and idiopathic non-cirrhotic portal hypertension (INCPH) indicate the same clinical entity. This includes the histological diagnosis of obliterative portal venopathy, hepatoportal sclerosis, nodular regenerative hyperplasia and incomplete septal cirrhosis. These terms, thereafter referred to as INCPH, designate a heterogeneous group of liver diseases causing portal hypertension and characterized by the absence of cirrhotic modification of the liver parenchyma and the patency of the portal and hepatic veins.
To retrospectively assess the fetal and maternal outcome of women who became pregnant after INCPH had been recognized and treated.
Hepatic Angiosarcoma is a highly aggressive and rare neoplasm (2% of primary liver tumors) arising from endothelial cells. Molecular pathogenesis of hepatic angiosarcoma is largely unknown. Patient survival is very poor with a median life expectancy of 6 months. No effective therapy has been estab- lished to date; antiangiogenic compounds such as sorafenib have shown only limited effects in patients.
To establish and enlarge a collection of liver biopsies from patients with hepatic angiosarcoma for immunohistochemical and molecular analysis. The ultimate goal of the study is to gain insight into the molecular pathogenesis of hepatic angiosarcoma and to identify potential therapeutic targets.
Congenital extrahepatic portosystemic shunts (Abernethy malformation): European Survey of current management strategies and clinical outcome.
Abernethy malformation is a rare disease with few reported cases in the literature and therefore its natural history is not well known.
Knowing the clinical onset and the outcome of this condition by gathering a large number of patients in a multicentric European study will allow to better know the natural history, long-term outcome and results of the treatments applied so far.
- Describing natural history in Abernethy malformation.
- Propose new follow up and therapeutic strategies according with the observed natural history, long-term outcome and results of the treatment strategies applied so far.
This study will follow “Good Clinical Practice”(GCP) standards and Declaration of Helsinki (1964) and Edinburgh revision (2000). All clinical data and clinical files of every patient will be managed confidentially and case treatment will be with a codification.
The Fontan procedure refers to a set of cardiac surgery techniques used to treat univentricular congenital heart disease. In this setting, liver disease results from hypoxia secondary to low cardiac output and hepatic venous outflow obstruction.
To assess the severity of liver damage, the presence of protein losing enteropathy and their clinical impact in patients with Fontan surgery.