Objevtive of the study is to analyze a European cohort of patients with INCIPH based on scientific analyses of clinical, laboratory and morphological data prospectively collected. HIV patients with known splenomagaly will be invited for abdominal…
ID
Source
Brief title
Condition
- Coagulopathies and bleeding diatheses (excl thrombocytopenic)
- Hepatic and hepatobiliary disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Prevalence of thrombophilic factors.
Clinical evolution.
Pathologic features
Fibroscan and ultrasound elements.
Presence of INCIPH in HIV patients
Secondary outcome
Not applicable
Background summary
Non-cirrhotic intrahepatic portal hypertension (NCIPH) is characterised by
portal hypertension with patent portal vein, in the absence of cirrhosis. In
the western countries the disorder is rare and its cause remains unknown. NCIPH
is therefore referred to as idiopathic non-cirrhotic intrahepatic portal
hypertension (INCIPH). Due to the rarity of the disease in Europe, solid
scientific knowledge on various aspects of NCIPH in this region is limited. In
1994, a Belgian cohort of 42 patients with incomplete septal cirrhosis (form of
INCIPH) was reported (including patients seen between 1968-1987). Since this
publication only one European cohort study (28 patients) has been performed
assessing manifestations, clinical course and causes, demonstrating a
prevalence of 50% of prothrombotic disorders in the studied cohort. This study
supports the theory of obstructive portal vasculopathy, posing injury
(thrombosis) to portal venules initiating alterations in liver architecture and
deposition of fibrin [6]. Nevertheless this thrombosis theory remains a
controversial issue.
Study objective
Objevtive of the study is to analyze a European cohort of patients with INCIPH
based on scientific analyses of clinical, laboratory and morphological data
prospectively collected. HIV patients with known splenomagaly will be invited
for abdominal ultrasound in order to diagnose portal hypertension. All patients
will be screened for thrombophilia and thrombin production (by use of the
thrombin generation test). Additionally fibroscan and abdominal ultrasound will
be performed. Histology available from previous performed liver biopsy will be
reassessed by 2 pathologists. Additional immunohistochemical stainings will be
performed to assess stellate cell activation.
Study design
Retrospective observational European multicentre study combined with assessment
of thrombophilia and performance of fibroscan and abdominal ultrasound in all
patients. Presence of portal hypertension in HIV patients with splenomegaly.
Study burden and risks
Patients participating the study (INCIPH patients and HIV patients with
splenomegaly) are invited for an interview at the hospital. Additionally an
abdominal ultrasound, fibroscan and blood examination will be performed (time:
1 hour). There are no risks associated with participation.
S Gravendijkwal 230
3015 CE Rotterdam
NL
S Gravendijkwal 230
3015 CE Rotterdam
NL
Listed location countries
Age
Inclusion criteria
1) Evidence of portal hypertension (oesophageal varices, hypersplenism or ascites)
2) Doppler ultrasound showing patent portal and hepatic veins
3) Liver biopsy showing no cirrhosis
4) Exclusion of conditions causing cirrhosis according to conventional diagnostic criteria (chronic viral hepatitis, alcoholic liver disease, NASH, obesity, hemochromatosis, autoimmune hepatitis or Wilson*s disease)
5) Exclusion of chronic vitam A intake, professional exposure to copper sulphate, vinyl chloride monomer, Spanish toxic oil or arsenic salts
6) Older than 18 years at diagnosis.
7) HIV patients
Exclusion criteria
1) Presence of cirrhosis
2) Presence of portal thrombosis at diagnosis
3) Presence of conditions causing cirrhosis according to conventional diagnostic criteria (chronic viral hepatitis, alcoholic liver disease, NASH, obesity, hemochromatosis, autoimmune hepatitis or Wilson*s disease).
4) Presence of chronic vitam A intake, professional exposure to copper sulphate, vinyl chloride monomer, Spanish toxic oil or arsenic salts
5) Younger than 18 years old at diagnosis.
6) intake oral anticoagulation
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL24296.078.08 |