title: Primary Budd-Chiari Syndrome in Children: King's College Hospital Experience.
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.
authors.affiliation: *Paediatric Liver, GI and Nutrition Center†Paediatric Hematology Unit‡Department of Radiology§Liver Transplant Service Institute of Liver Studies, King's College Hospital, London, UK.
authors.forename: Susana
authors.initials: S
authors.lastname: Nobre
authors.forename: Rajeev
authors.initials: R
authors.lastname: Khanna
authors.forename: Natalie
authors.initials: N
authors.lastname: Bab
authors.forename: Eirini
authors.initials: E
authors.lastname: Kyrana
authors.forename: Sue
authors.initials: S
authors.lastname: Height
authors.forename: John
authors.initials: J
authors.lastname: Karani
authors.forename: Pauline
authors.initials: P
authors.lastname: Kane
authors.forename: Nigel
authors.initials: N
authors.lastname: Heaton
authors.forename: Anil
authors.initials: A
authors.lastname: Dhawan
mesh_terms: D000293:Adolescent; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome