Budd Chiari syndrome associated with Antithrombin III and Protein S deficiency, a case report
DOI:
https://doi.org/10.35622/j.ram.2023.01.007Keywords:
Budd Chari, hypersplenism, antithrombin III, protein S, thrombophiliaAbstract
Budd Chari syndrome is a rare pathology characterized by obstruction of the venous flow of the liver, caused by thrombosis as a consequence of thrombophilia, myeloproliferative disorders, cancer, infections, or any disorder that obstructs the flow of the portal vein. The case was presented in a 21-year-old man who debuted with upper gastrointestinal bleeding, associated with esophageal varices and hypersplenism. In this patient, anticoagulation was started after ligation of esophageal varices, and after splenectomy, due to its association with thrombocytopenia, which subsequently increased, starting with low molecular weight heparin and later factor Xa inhibitors. (Rivaroxaban 20 mg) every day with good tolerance is discharged in good clinical condition. Timely diagnosis and treatment represent a challenge for the prognosis of patients.
References
Muñoz Maya OG, Vergara Cadavid JA, Cajiao Castro L, Marín Zuluaga JI, Restrepo Gutiérrez JC, Santos Sánchez OM. Síndrome de Budd-Chiari: etiología, manejo y resultados en una cohorte de 35 pacientes del Hospital Pablo Tobón Uribe. Rev Colomb Gastroenterol. 30 de septiembre de 2020;35(3):280-6. Disponible en: https://revistagastrocol.com/index.php/rcg/article/view/431
Jiménez Jiménez C, Randial Tagliapetra L, Silva I, Alférez F. Tratamiento endovascular del síndrome de Budd-Chiari, a propósito de un caso. Rev Colomb Cir. 13 de diciembre de 2019;34(4):386-93. Disponible en: http://www.scielo.org.co/pdf/rcci/v34n4/2011-7582-rcci-34-04-00386.pdf
Bosch J. Síndrome de Budd-Chiari. Gastroenterol Hepatol. 2000;23. Disponible en: https://www.elsevier.es/es-revista-gastroenterologia-hepatologia-14-articulo-sindrome-budd-chiari-12848
Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An Update on the Management of Budd-Chiari Syndrome. Dig Dis Sci. junio de 2021;66(6):1780-90. DOI: 10.1007/s10620-020-06485-y. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32691382/
Grus T, Lambert L, Grusová G, Banerjee R, Burgetova A. Budd-Chiari Syndrome. Prague Med Rep. 1 de septiembre de 2017; 118:69-80. Disponible en: https://pubmed.ncbi.nlm.nih.gov/28922103/
Li Y, De Stefano V, Li H, Zheng K, Bai Z, Guo X, et al. Epidemiology of Budd-Chiari syndrome: A systematic review and meta-analysis. Clin Res Hepatol Gastroenterol. 1 de agosto de 2019;43(4):468-74. DOI: 10.1016/j.clinre.2018.10.014. Disponible en:https://pubmed.ncbi.nlm.nih.gov/30528513/
Ferreira J, Mieres M, Rivero F, Elizondo M, Facal J, Ferreira J, et al. Síndrome de Budd-Chiari asociado a síndrome antifosfolípido. Rev Médica Urug. marzo de 2019;35(1):150-66. Disponible en: http://www.scielo.edu.uy/scielo.php?script=sci_arttext&pid=S1688
Rey L R, Elizondo B M, Rostán S S, Valverde G M, Gerona S S. Síndrome de Budd-Chiari. Análisis de una serie de casos y revisión del tema. Rev Gastroenterol Latinoam. 2020;(3):127-35. Disponible en: https://gastrolat.org/gastrolat2020003-02/
Tomoya H. Budd–Chiari syndrome caused by latent hepatic metastasis from a thymoma. Respir Med Case Rep. 1 de enero de 2021; 34:101492. Disponible en: https://pubmed.ncbi.nlm.nih.gov/34401316/
Karna R, Nasereddin T, Kaur H, Strahotin C. An unusual case of hepatic venous outflow obstruction: sequelae of immune checkpoint inhibitors. J Gastrointest Liver Dis JGLD. 20 de marzo de 2022;31(1):146-7. Disponible en: https://pubmed.ncbi.nlm.nih.gov/35306557/
Gioia S, De Santis E, Cerbelli B, Nardelli S, Ridola L, De Santis A, et al. Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report. Pathologica. junio de 2020;112(2):102-4. DOI: 10.32074/1591-951X-4-20https://pubmed.ncbi.nlm.nih.gov/32760053/
Ramírez AC, Gaviria SC, Herrera YPE, Marín JI, Muñoz O, Santos O, et al. Síndrome de Budd Chiari: Reporte de tres casos y revisión de la literatura. :4. https://pesquisa.bvsalud.org/portal/resource/pt/lil-729583
Correa Gaviria S, Ramírez AC, Espinoza Herrera YP, Restrepo Gutiérrez JC. Síndrome de Budd Chiari: revisión de tema. Rev Colomb Gastroenterol. septiembre de 2016;31(3):242-52. DOI: https://doi.org/10.22516/25007440.97
Hernández-Gea V, De Gottardi A, Leebeek FWG, Rautou PE, Salem R, Garcia-Pagan JC. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. J Hepatol. julio de 2019;71(1):175-99. Disponible en: https://pubmed.ncbi.nlm.nih.gov/30822449/
Hamano G, Takemura S, Tanaka S, Shinkawa H, Aota T, Fujii H, et al. Intrahepatic cholangiocarcinoma with a tumor thrombus extending from the inferior vena cava to the right atrium: a case report. Surg Case Rep. 6 de enero de 2021;7:3. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7788148/
Benali F, Aqodad N. Budd-Chiari primitif: défi diagnostique et thérapeutique. Pan Afr Med J. 8 de octubre de 2020; 37:142. Disponible en: https://www.panafrican-med-journal.com/content/article/37/142/full/
Krämer DA, Valdés F, Cruz F, González S. SINDROME DE BUDD-CHIARI, TRATAMIENTO QUIRURGICO DE 2 CASOS. :6. Disponible e: https://institutovascular.cl/wp-content/uploads/2017/01/1
Fan J, Wang Q, Luo B, Chen H, Wang Z, Niu J, et al. Prevalence of prothrombotic factors in patients with Budd–Chiari syndrome or non‐cirrhotic nonmalignant portal vein thrombosis: A hospital‐based observational study. J Gastroenterol Hepatol. julio de 2020;35(7):1215-22. https://pubmed.ncbi.nlm.nih.gov/31711259/
Blas PB, Ros RE, Garcia AB, Moral PS. Colecistitis parece, sindrome de budd chiari es. Atalaya MédicaTurol.2020;(19):44-8.Disponible en: https://dialnet.unirioja.es/servlet/articulo?codigo=7907042
Semmler G, Lindorfer A, Schäfer B, Bartl S, Hametner-Schreil S, Gensluckner S, et al. Outcome of Budd-Chiari Syndrome Patients Treated With Direct Oral Anticoagulants: An Austrian Multicenter Study. Clin Gastroenterol Hepatol. mayo de 2022; S1542356522004499. Disponible en: https://pubmed.ncbi.nlm.nih.gov/35533994/
Valla DC. Primary Budd-Chiari syndrome. J Hepatol. enero de 2009;50(1):195-203. DOI: 10.1016/j.jhep.2008.10.007.Disponible en: https://pubmed.ncbi.nlm.nih.gov/35533994/
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