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Title: Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms
Authors: Wikrom Karnsakul
Thaschawee Arkachaisri
Kanit Atisook
Wanee Wisuthsarewong
Yudhtana Sattawatthamrong
Prapun Aanpreung
West Virginia University School of Medicine Morgantown
Mahidol University
Department of Pediatrics
Keywords: Medicine
Issue Date: 19-Jun-2006
Citation: Annals of Hepatology. Vol.5, No.2 (2006), 116-119
Abstract: Vanishing bile duct syndrome (VBDS) is a rare disorder and requires a liver biopsy for a diagnosis. The condition has not been reported in children with toxic epidermal necrolysis (TEN). The etiology of VBDS in our patient with TEN is most likely from drug hypersensitivity. A high index of suspicion will prompt clinicians to start more specific investigations and treatments. The use of immunosuppressive agents, intravenous immunoglobulin and ursodeoxycholic acid has not been consistently successful in these patients. A new approach with biologic agents such as anti-tumor necrosis factor-α may be a promising therapy and reduce severe adverse outcomes.
Appears in Collections:Scopus 2006-2010

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