Primary Budd-Chiari syndrome in children: King’s College Hospital experience
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Authors
Nobre, Susana
Khanna, Rajeev
Bab, Natalie
Kyrana, Eirini
Height, Sue
Karani, John
Kane, Pauline
Heaton, Nigel
Dhawan, Anil
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Issue Date
2017
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Article
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Abstract
Primary Budd-Chiari syndrome is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary Budd-Chiari syndrome presenting from January 2001 to November 2015 to our hospital. Seven children were identified. Their presentation was mostly chronic. All had predisposing factors for thrombosis and were started on anticoagulation. Radiological interventions (2 transjugular intrahepatic portosystemic shunts and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2, and 1 patients, respectively; 1 child underwent bone marrow transplantation following transjugular intrahepatic portosystemic shunts and 1 child was managed only medically. After liver transplantation, one child died 3 years later as a result of subarachnoid haemorrhage, whereas others remain well at a median follow-up of 6 years. Despite high morbidity, the disease can have a good long-term outcome with a multidisciplinary approach.
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Journal
Journal of Pediatric Gastroenterology and Nutrition
Volume
65
Issue
1