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Budd-Chiari Syndrome-A Single Center Experience From the United Kingdom.
Mann, J P; Ikram, F; Modin, L; Kelgeri, C; Sharif, K; Olliff, S; McGuirk, S; Gupte, G L.
Afiliação
  • Mann JP; From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK.
  • Ikram F; From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK.
  • Modin L; From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK.
  • Kelgeri C; From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK.
  • Sharif K; From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK.
  • Olliff S; Interventional Radiology Department, Queen Elizabeth Hospital Birmingham, Birmingham, UK.
  • McGuirk S; the Radiology Department, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
  • Gupte GL; From the Liver Unit, Birmingham Women's & Children's Hospital, Birmingham, UK.
J Pediatr Gastroenterol Nutr ; 77(4): 455-459, 2023 10 01.
Article em En | MEDLINE | ID: mdl-37314703
Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Derivação Portossistêmica Transjugular Intra-Hepática / Síndrome de Budd-Chiari Tipo de estudo: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Child / Humans País/Região como assunto: Europa Idioma: En Ano de publicação: 2023 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Derivação Portossistêmica Transjugular Intra-Hepática / Síndrome de Budd-Chiari Tipo de estudo: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Child / Humans País/Região como assunto: Europa Idioma: En Ano de publicação: 2023 Tipo de documento: Article