Liver transplant in Budd-Chiari syndrome: a single-center experience in Saudi Arabia.

作者: Yahia Saleh , , Firas Zahr Eldeen , Yasser Kamel , Monther Kabbani

DOI: 10.6002/ECT.2013.0153

关键词: VeinPortal vein thrombosisMedicineBudd–Chiari syndromeSingle CenterWarfarinSurgeryCoagulopathyImmunosuppressionIntensive care unit

摘要: OBJECTIVES If they do not respond to other treatments, patients with Budd-Chiari syndrome are potential candidates for a liver transplant. Timing transplant is controversial; however, before systems deteriorate, early intervention in relatively stable patient may improve the outcome and survival of these patients. MATERIALS AND METHODS Six (2 women 4 men) had (1.2%) among 475 who undergone at our center between 2001 2012. Imaging modalities including duplex ultrasound, abdominal computed tomography angiography, hematologic evaluation were part routine diagnostic work-up. Although we perform mostly living-donor transplants, received from deceased donor, because there was enough evidence justify We thought that replacing caval vein might negatively influence outcome. Postoperatively, recipients started on heparin infusion triple therapy immunosuppression; only then warfarin introduced as long-term anticoagulant. RESULTS Two died, 1 uncontrollable bleeding disseminated intravascular coagulopathy, died intensive care unit after 5 months multiorgan failure sepsis. One portal thrombosis 9 transplant; needed retransplant years owing failure, secondary chronic rejection. Graft rate 75%, 66.6%. CONCLUSIONS This first article Saudi Arabia describe this subgroup syndrome. Treatment follows therapeutic algorithm should start anticoagulation end up it be considered if treatments fail.

参考文章(10)
Dominique-Charles Valla, Primary Budd-Chiari syndrome. Journal of Hepatology. ,vol. 50, pp. 195- 203 ,(2009) , 10.1016/J.JHEP.2008.10.007
Aurélie Plessier, Annie Sibert, Yann Consigny, Antoine Hakime, Magaly Zappa, Marie-Hélène Denninger, Bertrand Condat, Olivier Farges, Carine Chagneau, Victor de Ledinghen, Claire Francoz, Alain Sauvanet, Valérie Vilgrain, Jacques Belghiti, François Durand, Dominique Valla, Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome Hepatology. ,vol. 44, pp. 1308- 1316 ,(2006) , 10.1002/HEP.21354
Antonia Perelló, Juan Carlos García-Pagán, Rosa Gilabert, Yanette Suárez, Eduardo Moitinho, Francisco Cervantes, Juan Carlos Reverter, Angels Escorsell, Jaume Bosch, Juan Rodés, TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy Hepatology. ,vol. 35, pp. 132- 139 ,(2002) , 10.1053/JHEP.2002.30274
Martin Rössle, Manfred Olschewski, Volker Siegerstetter, Elisabeth Berger, Katharina Kurz, Daniel Grandt, The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. ,vol. 135, pp. 394- 403 ,(2004) , 10.1016/J.SURG.2003.09.005
Laurie D. DeLeve, Dominique-Charles Valla, Guadalupe Garcia-Tsao, Vascular disorders of the liver. Hepatology. ,vol. 49, pp. 1729- 1764 ,(2009) , 10.1002/HEP.22772
Barbara Rosado, Patrick S Kamath, Transjugular intrahepatic portosystemic shunts: An update Liver Transplantation. ,vol. 9, pp. 207- 217 ,(2003) , 10.1053/JLTS.2003.50045
JANG B. DILAWARI, PRADEEP BAMBERY, YOGESH CHAWLA, UPJEET KAUR, SHIVAYOGI R. BHUSNURMATH, HARINDER S. MALHOTRA, GAGAN K. SOOD, SOMEN K. MITRA, SATISH K. KHANNA, BRIJNANDAN S. WALIA, Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine. ,vol. 73, pp. 21- 36 ,(1994) , 10.1097/00005792-199401000-00003
Sarwa Darwish Murad, Dominique-Charles Valla, Piet C. de Groen, Guy Zeitoun, Judith A.M. Hopmans, Elizabeth B. Haagsma, Bart van Hoek, Bettina E. Hansen, Frits R. Rosendaal, Harry L.A. Janssen, Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome Hepatology. ,vol. 39, pp. 500- 508 ,(2004) , 10.1002/HEP.20064
T. Yamada, K. Tanaka, Y. Ogura, S. Ko, Y. Nakajima, Y. Takada, S. Uemoto, Surgical techniques and long-term outcomes of living donor liver transplantation for Budd-Chiari syndrome. American Journal of Transplantation. ,vol. 6, pp. 2463- 2469 ,(2006) , 10.1111/J.1600-6143.2006.01505.X
Gilles Mentha, Emiliano Giostra, Pietro E. Majno, Wolf O. Bechstein, Peter Neuhaus, John O'Grady, Raaj K. Praseedom, Andrew K. Burroughs, Yves P. Le Treut, Preben Kirkegaard, Xavier Rogiers, Bo-Goran Ericzon, Krister Hockerstedt, René Adam, Juergen Klempnauer, Liver transplantation for Budd–Chiari syndrome: A European study on 248 patients from 51 centres Journal of Hepatology. ,vol. 44, pp. 520- 528 ,(2006) , 10.1016/J.JHEP.2005.12.002