Yılmaz BİLGİÇ, Muhammet CENGİZ, Ayşe Nuransoy CENGİZ, Cengiz YILMAZ, Yasir Furkan ÇAĞIN, Fatih SANCAKTAR, Oğuzhan YILDIRIM

Postpartum Dönemde Homozigot Faktör-V Leiden Mutasyonuna Bağlı Akut Budd-Chiari Sendromu

Budd--Chiari Sendromuküçük hepatik verilerden sağ atriuma kadar olan damarsal yapılarda ortaya çıkan darlık veya obstrüksiyonlar sonucunda karaciğer venöz dönüşünün bozulmuş olduğunadir görülen bir durumdur. Klinik spektrumu, kronik asemptomatik hast alıktarı akut fulminan karaci-- ğer yetmezliği arasında değişkenlik gösterir ve karaciğer sirozu ile neticelenebilir. Trombotiliye eğilim oluşturan kalıtsal veya edinsel nedenler, damar sistemine dıştan bası gibi birçok neden etiyolojide yer alır. Gebelik ve postpartum dönem tromboza eğilimi arttırır. Yazımızda postpartum dönemde akut Budd--Chiari Sendromutamsı konulmuş olanve Faktör--V Leiden mutasyonuhomozigot pozitif saptanmış olan nadir bir vakayı sunmaktayız.

A C ase of Acute Budd-Chiari Syndrome Due to Homozygous Factor-V Leiden Mutation in the Postpartum Period

Budd-Chiari Syndrome is a rare disorder charecterized by obstruction of hepatic outflow tract at any level between small hepati c venules and right atrium. Clinical spectrum changes in a range between chronic asymptomatic patients to fulminant liver failure, it may also re sults with cirrhosis. T here are many etiologies of the syndrome like inherited and secondary thrombophilias and external compression of veins. Pregnancy and postpartum period increase the tendency to thrombosis. In this paper, we presented a rare case of acute Budd Chiari syndrome, which was found to be homozygous for factor-V leiden mutation in the postpartum period.

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1. Martens P, Nevens F. Budd-Chiari syndronıe. United European Gastroenterol 2015; 3: 489- 500.
2. Aydoğan T, Türkay C. Budd-Chiari Sendromu. Güncel Gastroenteroloji. 2008; 12: 53-8.
3. DeLeve I_D, Valla DC, Garcia-Tsao G, et al. Vascular disorders of the liver. Hepatology 2009; 49: 1729-64.
4. Hadengue A, Poliquin M, Vilgrain V, et al. The changing scene of hepatic vein thrombosis: Recognition of asymptomatic cases. Gastroenterology1994;106: 1042-7.
5. Bolondi L, Gaiani S, Li BS, et al. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology 1991;100: 1324-31.
6. Darwish Murad S, Plessier A, HernandezGuerra M, et al. Etiologv, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009;151: 167--75.
7. Marshall AL. Diagnosis, treatment, and prevention of venous thromboembolismin pregnancy. Postgrad Med 2014; 126: 25-34.
8. Coriu L, Ungureanu R, Talmaci R, et al. Hereditary thrombophilia ant thrombotic events in pregnancy: single center experience. Med Life 2014; 7: 567-71.
9. Konkle BA. Diagnosis and management of thrombosis in pregnancy. Birth Defects Res Embryo Today 2015; 105: 185-9.
10. Goldman's Cecil Medicine 24th Edition, 2012: 1149-53.
11. Hidaka M, Eguchi S. Budd-Chiari syndrome: Focus on surgical treatment. Hepatol Res 2016 Jun 1.
12. Merz WM, Rüland AM, Hippe V, et al. Pregnancy in Budd-Chiari Syndrome: Case report and proposed risk score. Medicine 2016; 95: 3817.
13. Schafer A. Thrombotic Disorders: Hypercoagu-- lable States in: Goldman L, Schafer A. Eds. Cecil Medicine, Saunders Co. Section 14.
14. Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology 2003; 38: 793-803.
15. Zhang C, Gu Y, Bian C, Zhu G, Luo T, Yan C, Wang Z. Hybrid treatment for Budd-Chiari Syndrome: case report by 11-year follow-up. Ann Vasc Surg 2016; 38: 319.