obliterative portal venopathy
Jump to navigation
Jump to search
Pathology
- lesions of the intrahepaticbranches of the portal vein
- occlusion or obliteration ofsmall branches of the portal vein
- may be responsible for many cases of portal hypertension in the absence of cirrhosis or obstruction of large portal veins or hepatic veins
Genetics
- associated with defects in C4orf54 (FOPV)
- autosomal dominant with incomplete penetrance & variable expressivity