Vanishing bile duct syndrome in human immunodeficiency virus infected adults: A report of two cases
Ana Paula Oppenheimer, John C Williamson, Kevin P High, Section of Infectious Diseases, Department of Medicine, Wake Forest School of Medicine, Winston-Salem, NC 27109-7205, United States.World Journal of Gastroenterology (Impact Factor: 2.37). 01/2013; 19(1):115-21. DOI: 10.3748/wjg.v19.i1.115
Vanishing bile duct syndrome (VBDS) is a group of rare disorders characterized by ductopenia, the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis. Described in association with medications, autoimmune disorders, cancer, transplantation, and infections, the specific mechanisms of disease are not known. To date, only 4 cases of VBDS have been reported in human immunodeficiency virus (HIV) infected patients. We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases. Presentation includes hyperbilirubinemia, normal liver imaging, and negative viral and autoimmune hepatitis studies. In HIV-infected subjects, VBDS occurred at a range of CD4+ T-cell counts, in some cases following initiation or change in antiretroviral therapy. Lymphoma was associated with two cases; nevirapine, antibiotics, and viral co-infection were suggested as etiologies in the other cases. In HIV-positive patients with progressive cholestasis, early identification of VBDS and referral for transplantation may improve outcomes.
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ABSTRACT: Unlabelled: Biliary complications (BCs) remain one of the most outstanding factors influencing long-term results after orthotopic liver transplantation. The authors carried out a systematic overview of 1720 papers since 2008, and focused on 45 relevant ones. Among 14,411 transplanted patients the incidence of BCs was 23%. Biliary leakage occurred in 8.5%, biliary stricture in 14.7%, mortality rate was 1-3%. Risk factors: preoperative sodium level; p = 0.037, model of end-stage liver disease score >25; p = 0.048, primary sclerosing cholangitis; p = 0.001, malignancy; p = 0.026, donor age >60, macrovesicular graft steatosis; p = 0.001, duct-to-duct anastomosis; p = 0.004, long anhepatic phase; p = 0.04, cold ischemic time >12 h; p = 0.043, use of T-tube; p = 0.032, insufficient flush of bile ducts; p = 0.001, acute rejection; p = 0.003, cytomegalovirus infection; p = 0.004 and hepatic artery thrombosis; p = 0.001. The management was surgical in case of biliary leakage, and interventional radiology or endoscopic retrograde cholangiopancreatography in case of biliary stricture. Mapping of miRNA profile is a new field of research. Nemes-Doros score is a useful tool in the estimation of hepatic artery thrombosis. Management of BCs requires a multidisciplinary expert team.
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