Riassunto analitico
Background and Aims: INCPH is a rare liver disorder characterized by portal hypertension, patent portal and hepatic veins, and absence of cirrhosis at biopsy. Its pathophysiology is largely unknown. Thrombophila, infections, drugs, immunological and genetic factors may be involved. Information on etiology, inherited factors, and treatment is lacking. We aimed at identifying incident/prevalent cases as a basis for observational, prognostic and interventional studies.
Methods: Biopsy-confirmed INCPH subjects. Assessment of comorbidities, comprehensive case-report form compilation.
Results: 112 patients, 70 males, at 6 centers. Age at diagnosis: 50 (10-79) years.
Conclusions: INCPH occurs without relevant comorbidities in about 60% of cases, highlighting the role of unrecognized factors. Comorbidities include mainly immune-mediated disorders, previous solid cancer, myeloproliferative neoplasia, and thrombophilia.
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