Budd-Chiari syndrome and extrahepatic portal obstruction associated with congenital antithrombin III deficiency

• Published in: Journal of gastroenterology Vol. 36; no. 5; pp. 341 - 345
• Main Authors: Nakase, H, Kawasaki, T, Itani, T, Mimura, J, Komori, H, Okazaki, K, Chiba, T
• Format: Journal Article
• Language: English
• Published: Japan 01.05.2001
• Subjects: Adult; Anticoagulants - therapeutic use; Antithrombin III Deficiency - complications; Antithrombin III Deficiency - congenital; Budd-Chiari Syndrome - complications; Budd-Chiari Syndrome - diagnosis; Budd-Chiari Syndrome - drug therapy; Humans; Japan; Male; Portal System; Warfarin - therapeutic use; Japan
• ISSN: 0944-1174; 1435-5922
• DOI: 10.1007/s005350170101

We report a patient with Budd-Chiari syndrome (BCS) and extrahepatic portal obstruction (EHO) associated with congenital antithrombin (AT) III deficiency. A 35-year-old man was admitted to Nishi Kobe Medical Center for evaluation of abnormal intrahepatic veins. By various imaging modalities, BCS and EHO were diagnosed. Laboratory data revealed parallel decreases in activity and antigen concentration of AT III despite normal liver function. Taken together, the etiology of both BCS and EHO was considered to be thrombosis, associated with congenital AT III deficiency. Two years after beginning warfarin therapy, the patient has no symptoms and his liver function remains normal. Anticoagulant therapy is considered useful for preventing progression of the disease.