Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms
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...
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Elsevier
2006-04-01
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| Series: | Annals of Hepatology |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1665268119320290 |
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doaj-7e75cbfbfbbb4a608dca085419bf3ede2021-06-08T04:36:19ZengElsevierAnnals of Hepatology1665-26812006-04-0152116119Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanismsWikrom Karnsakul0Thaschawee Arkachaisri1Kanit Atisook2Wanee Wisuthsarewong3Yudhtana Sattawatthamrong4Prapun Aanpreung5West Virginia University School of Medicine. Department of Pediatrics, Section of Gastroenterology and Nutrition, Morgantown, WV Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; Department of Pediatrics; Address for correspondence:Department of PediatricsDepartment of PathologyDepartment of PediatricsDepartment of Internal MedicineDepartment of PediatricsVanishing 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.http://www.sciencedirect.com/science/article/pii/S1665268119320290CholestasisductopeniaDrug rashtrimethoprim-sulfamethoxazole |
| collection |
DOAJ |
| language |
English |
| format |
Article |
| sources |
DOAJ |
| author |
Wikrom Karnsakul Thaschawee Arkachaisri Kanit Atisook Wanee Wisuthsarewong Yudhtana Sattawatthamrong Prapun Aanpreung |
| spellingShingle |
Wikrom Karnsakul Thaschawee Arkachaisri Kanit Atisook Wanee Wisuthsarewong Yudhtana Sattawatthamrong Prapun Aanpreung Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms Annals of Hepatology Cholestasis ductopenia Drug rash trimethoprim-sulfamethoxazole |
| author_facet |
Wikrom Karnsakul Thaschawee Arkachaisri Kanit Atisook Wanee Wisuthsarewong Yudhtana Sattawatthamrong Prapun Aanpreung |
| author_sort |
Wikrom Karnsakul |
| title |
Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms |
| title_short |
Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms |
| title_full |
Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms |
| title_fullStr |
Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms |
| title_full_unstemmed |
Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms |
| title_sort |
vanishing bile duct syndrome in a child with toxic epidermal necrolysis: an interplay of unbalanced immune regulatory mechanisms |
| publisher |
Elsevier |
| series |
Annals of Hepatology |
| issn |
1665-2681 |
| publishDate |
2006-04-01 |
| description |
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. |
| topic |
Cholestasis ductopenia Drug rash trimethoprim-sulfamethoxazole |
| url |
http://www.sciencedirect.com/science/article/pii/S1665268119320290 |
| work_keys_str_mv |
AT wikromkarnsakul vanishingbileductsyndromeinachildwithtoxicepidermalnecrolysisaninterplayofunbalancedimmuneregulatorymechanisms AT thaschaweearkachaisri vanishingbileductsyndromeinachildwithtoxicepidermalnecrolysisaninterplayofunbalancedimmuneregulatorymechanisms AT kanitatisook vanishingbileductsyndromeinachildwithtoxicepidermalnecrolysisaninterplayofunbalancedimmuneregulatorymechanisms AT waneewisuthsarewong vanishingbileductsyndromeinachildwithtoxicepidermalnecrolysisaninterplayofunbalancedimmuneregulatorymechanisms AT yudhtanasattawatthamrong vanishingbileductsyndromeinachildwithtoxicepidermalnecrolysisaninterplayofunbalancedimmuneregulatorymechanisms AT prapunaanpreung vanishingbileductsyndromeinachildwithtoxicepidermalnecrolysisaninterplayofunbalancedimmuneregulatorymechanisms |
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1721390927507881984 |