>Acta Pediátrica de México
>Year 2005, Issue 6
Carbajal RL, Vargas QE, Ramírez MJA, Copto GA, Reynés MJN, Rodríguez HR, Barrios FR, Zarco RJ
Budd-Chiari syndrome (BCS) associated with antiphospholipid syndrome (APS)
Acta Pediatr Mex 2005; 26 (6)
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Background: Budd-Chiari syndrome (BCS) is an infrequent condition. It presents with intrahepatic venous thrombosis, and it may be caused by the antiphospolipid symdrome (APHS), which is characterized by the presence of anticardiolipin antibodies and lupic anticoagulant factor. There are two types of BCS: primary and secondary caused by infections, neoplasm, drugs and autoimmunity.
Materials and methods: Retrospective, transversal and observational study of 24 patients with APHS, seven of which had BCS. Age, gender, types, complications, thrombosis and laboratory findings were analyzed.
Results: Seven patients were between 10 and 15 years old, there were four females and three males. Hepatomegaly was present in all; one patient had splenomegaly, ascitis and bleeding problems. Every patient had portal vein thrombosis. TGO, TGP, GGT tests were done in all; CH50 was done in two patients. ACLA were measured in the seven patients; LAC in one; beta 2 glucoprotein in one.
Conclusions: Symptoms were not different in both types of BCS. Secondary BCS ones were caused by LEG, AACL, ACL . The B2GP1 it was most common in the primary BCS cases.
||Budd-Chiari syndrome, portal hypertension, thrombosis, antiphospholipid syndrome.
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>Acta Pediátrica de México
>Year 2005, Issue 6