Subject(s)
Amyloidosis/blood , Blood Coagulation Factors/therapeutic use , Blood Loss, Surgical/prevention & control , Carcinoma/surgery , Factor X Deficiency/blood , Thyroid Neoplasms/surgery , Amyloidosis/complications , Amyloidosis/pathology , Amyloidosis/surgery , Carcinoma/pathology , Carcinoma, Papillary , Factor X Deficiency/etiology , Factor X Deficiency/pathology , Factor X Deficiency/surgery , Female , Humans , Immunoglobulin Light Chains/blood , Immunoglobulin Light-chain Amyloidosis , Middle Aged , Thyroid Cancer, Papillary , Thyroid Neoplasms/pathologyABSTRACT
Factor X (FX) deficiency is a rare, autosomal-recessive coagulation disorder. Diagnosis can be confirmed by a factor X assay. Although fresh frozen plasma and prothrombin complex concentrates have been used as a temporary treatment of bleeding symptoms and preparation for surgery, frequent transfusion has its risk and prothrombin complex is not available in Korea. We report the first pediatric case of successful liver transplantation for the correction of a severe congenital FX deficiency in a child with recurrent life-threatening hemorrhagic episodes.
Subject(s)
Blood Coagulation/genetics , Factor X Deficiency/surgery , Hemorrhage/prevention & control , Liver Transplantation , Blood Coagulation Tests , DNA Mutational Analysis , Factor X Deficiency/blood , Factor X Deficiency/complications , Factor X Deficiency/diagnosis , Factor X Deficiency/genetics , Female , Hemorrhage/blood , Hemorrhage/genetics , Humans , Infant , Male , Severity of Illness Index , Treatment OutcomeSubject(s)
Factor VII Deficiency/surgery , Factor X Deficiency/surgery , Spinal Fusion/methods , Blood Coagulation Factors/therapeutic use , Child , Chromosome Deletion , Factor VII/therapeutic use , Factor VIIa , Female , Humans , Infant , Male , Phenotype , Recombinant Proteins/therapeutic use , Treatment OutcomeABSTRACT
Factor X deficiency is an important complication of amyloidosis. It is associated with severe bleeding that is difficult to control with plasma or prothrombin complex concentrates. Splenectomy ameliorates the factor X deficiency, but achieving satisfactory haemostasis for this operation is problematic. We report that a new clotting concentrate, recombinant factor VIIa, readily controls bleeding and makes splenectomy feasible.
