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.
VALDIG Database and Registry
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 firstname.lastname@example.org) 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.
For more information and the study descriptions see here
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.
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.
Idiopathic noncirrhotic portal hypertension (IPH) and liver transplantation (OLT), outcome and prognosis factors
Factors predicting the need of OLT and the real applicability of OLT in patients with IPH are not well known.
Aim 1: To identify factors predicting the need of LT and its real life applicability. We will evaluate a large international cohort of consecutive registered patients with IPH diagnosis followed in centers of the REHEVASC (Spanish Hepatic Vascular Disorders Registry) and/or VALDIG (Vascular Liver Disease Group). Only centers with active databases/registries.
Aim 2: To evaluate the long term outcome of patients with IPH who received LT (including data on survival, potential recurrence of the disease and comorbidities). We will evaluate both patients from centers participating in aim 1 and also patients actively collected in other transplant centers performing exhaustive analysis of the explanted livers that will be willing to participate.
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.