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hepatoportale sclerose (aandoening)
hepatoportale sclerose
Hepatoportal sclerosis
Obliterative portal venopathy
A rare disorder with characteristics of sclerosis of the intrahepatic portal veins, non-cirrhotic portal hypertension, asymptomatic splenomegaly and recurrent variceal bleeding. Most commonly, the condition is detected in investigating a fortuitous finding of hypersplenism or splenomegaly. Main histopathologic findings are periportal fibrosis, occlusion of small portal veins, sclerosis of the portal venous system, and proliferation of small vascular channels within/around portal tracts. The disease is slowly progressive. Exposure to toxic substances or drugs, autoimmune and connective tissue diseases, systemic or intraabdominal infections, and clotting abnormalities have been incriminated. A genetic background has been suggested.
Id718096004
StatusPrimitive
SNOMED CT to Orphanet simple map64743
SNOMED CT to ICD-10 extended map
TargetK74.1
RuleTRUE
AdviceALWAYS K74.1
CorrelationSNOMED CT source code to target map code correlation not specified