title: Budd-Chiari syndrome and extrahepatic portal obstruction associated with congenital antithrombin III deficiency.
abstract: 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.
authors.affiliation: Division of Gastroenterology, Nishi-Kobe Medical Center, Kobe, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Nakase
authors.forename: T
authors.initials: T
authors.lastname: Kawasaki
authors.forename: T
authors.initials: T
authors.lastname: Itani
authors.forename: J
authors.initials: J
authors.lastname: Mimura
authors.forename: H
authors.initials: H
authors.lastname: Komori
authors.forename: K
authors.initials: K
authors.lastname: Okazaki
authors.forename: T
authors.initials: T
authors.lastname: Chiba
mesh_terms: D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D020152:Antithrombin III Deficiency / Q000150:complications / Q000151:congenital*; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007564:Japan; D008297:Male; D011168:Portal System; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D000925:Anticoagulants; D014859:Warfarin