Budd-Chiari syndrome associated with coagulation abnormalities in a child with carbohydrate deficient glycoprotein syndrome type Ix

A 6-year-old male patient presented with Budd-Chiari syndrome and glycoprot ein abnormalities associated with carbohydrate deficient glycoprotein syndr ome type I with yet unidentified molecular defect (type Ix). Budd-Chiari sy ndrome most likely developed after hepatic venous thrombosis caused by coag ulation abnormalities resulting from hypoglycosylation and functional impai rment of anticoagulant proteins.