title: Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: an interplay of unbalanced immune regulatory mechanisms.
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-alpha may be a promising therapy and reduce severe adverse outcomes.
authors.affiliation: Department of Pediatrics, West Virginia University School of Medicine, Morgantown, WV 26506-9214, USA. wakarnsakul@hsc.wvu.edu
authors.forename: Wikrom
authors.initials: W
authors.lastname: Karnsakul
authors.forename: Thaschawee
authors.initials: T
authors.lastname: Arkachaisri
authors.forename: Kanit
authors.initials: K
authors.lastname: Atisook
authors.forename: Wanee
authors.initials: W
authors.lastname: Wisuthsarewong
authors.forename: Yudhtana
authors.initials: Y
authors.lastname: Sattawatthamrong
authors.forename: Prapun
authors.initials: P
authors.lastname: Aanpreung
mesh_terms: D000890:Anti-Infective Agents / Q000009:adverse effects*; D001649:Bile Duct Diseases / Q000139:chemically induced* / Q000276:immunology / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000276:immunology / Q000473:pathology; D002648:Child; D004342:Drug Hypersensitivity / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017093:Liver Failure / Q000209:etiology / Q000473:pathology; D013262:Stevens-Johnson Syndrome / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000009:adverse effects*
chemical_list: D000890:Anti-Infective Agents; D007166:Immunosuppressive Agents; D015662:Trimethoprim, Sulfamethoxazole Drug Combination