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Budd–Chiari syndrome in children and outcome after liver transplant
Author(s) -
Gomes Ana Cristina,
Rubino Gina,
Pinto Carla,
Cipriano Augusta,
Furtado Emanuel,
Gonçalves Isabel
Publication year - 2012
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2012.01683.x
Subject(s) - medicine , budd–chiari syndrome , liver transplantation , fulminant , dipyridamole , methylenetetrahydrofolate reductase , aspirin , surgery , pediatrics , gastroenterology , liver disease , portal hypertension , cirrhosis , transplantation , inferior vena cava , biochemistry , chemistry , genotype , gene
Gomes AC, Rubino G, Pinto C, Cipriano A, Furtado E, Gonçalves I. Budd–Chiari syndrome in children and outcome after liver transplant. Abstract: BCS is a rare form of portal hypertension in children. The authors describe two cases of BCS with differing presentations. Case 1 : Previously healthy four‐yr‐old girl. BCS was diagnosed during the course of an episode of acute gastroenteritis with dehydration. Despite conservative therapy for two months, the condition was progressive resulting in liver failure leading ultimately to LT. Molecular studies showed that she was heterozygous for the Factor (F) V Leiden. At follow‐up, six yr post‐LT (two yr without anticoagulation therapy), no thromboembolic/bleeding events were apparent. Case 2 : Three‐yr‐old boy with IgA deficiency and liver disease. Following a febrile episode, he developed fulminant liver failure requiring urgent LT from a living donor (father). Molecular studies disclosed MTHFR C677T homozygosity and FV Leiden heterozygosity. The father was homozygous for the MTHFR mutation. Three months post‐LT, persistent graft dysfunction was associated with stenosis of the IVC, which improved upon stent placement. He received dipyridamole and aspirin for five yr, after which time dipyridamole was discontinued. Evidence is sparse on the follow‐up of BCS cases with liver transplant. The authors discuss their findings, particularly the need for long‐term anticoagulation.