Liver cirrhosis is the main cause of portal hypertension in the western world. The large number of patients with this pathology has enabled many studies to be made on both unicentral and multicentral scales, which has meant that there has been progress in the knowledge of this illness. There are however other liver diseases apart from cirrhosis which can also give rise to developing portal hypertension. This group of diseases is grouped together under the concept of non-cirrhotic portal hypertension (NCPH). The diagnosis of patients with NCPH is growing logarithmically, probably due to a number of patients now being identified who some years ago might wrongly have been diagnosed with cryptogenetic cirrhosis.
NCPH includes vascular liver disorders covering a number of conditions, all of them rare (with a prevalence under 5/10,000 inhabitants): the Budd-Chiari syndrome, non-cirrhotic portal thrombosis and idiopathic portal hypertension being the three main ones. Other far less prevalent entities are congenital hepatic fibrosis (CHF), schistosomiasis or hereditary haemorrhagic telangiectasia, amongst others.
Several centres in this country have over the last few years gradually acquired experience in the diagnosis and treatment of patients with NCPH. In these diseases the progress made in knowledge of their physiopathology, their natural history and the evaluation of new diagnostic and/or therapeutic alternatives is nevertheless hindered by the low number of patients diagnosed and individually visiting each of these centres. For this same reason the diagnosis and therapeutic approach is very often completely heterogeneous, depending on the doctor in charge of the patient. For all these reasons a “Grupo Cooperativo Nacional de Centros para el Estudio de la HTPNC” has been set up with the following main objectives:
The consolidation of a state cooperative group with interests in NCPH will enable estimating the incidence of these pathologies in Spain. It will also enable examining a larger number of patients with these pathologies who have been uniformly evaluated. All of this will enable progress to be made in knowledge of these diseases, their natural history and provide the capacity to make randomised or cohort-based multicentre studies.
If you have any patient(s) suffering from any of these diseases and are interested in registering at our Platform (http://rehevasc.ciberehd.org), please send an e-mail to email@example.com, specifying that you would like to register at this Platform and including your FULL NAME, MEDICAL ASSOCIATION NUMBER, HOSPITAL/CENTRE TO WHICH YOU BELONG and CONTACT NUMBER. After receiving this information, you will be given a user number and password to enter the Platform.