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Rapidly Progressive Portal Cavernoma Cholangiopathy in a Patient With Infeasible Decompressive Shunt Surgery. | LitMetric

We present a 27-year-old man with a 2-year history of extrahepatic portal vein obstruction and selective immunoglobulin A deficiency, referred for acute cholangitis from portal cavernoma cholangiopathy (PCC). Because recurrent cholangitis rapidly led to liver failure, orthotopic liver transplantation (OLT) was successfully performed. To date, this is one of the few cases of patients with symptomatic PCC who required OLT and the first case who had a successful 6-year follow-up. Thus, OLT can be used for symptomatic PCC associated with nonshuntable anatomy, ineffective biliary drainage, and progressive liver damage. Selective immunoglobulin A deficiency may play a role in recurrent cholangitis.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10371324PMC
http://dx.doi.org/10.14309/crj.0000000000000778DOI Listing

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