We have reported on a case of hemophilia B (Christmas disease) of 6 month old Korean who was have admitted to our hospital in duration of 7 days on Aug. 1974. Clinical study review of literature for a case were made briefly.
Hemophilia B ; Humans ; Infant

No abstract available.
Down Syndrome* ; Hemophilia A* ; Hemophilia B* ; Humans

No abstract available.
Hematoma, Subdural ; Hemophilia A ; Hemophilia B ; Humans ; Infant

Factor VIII ; Hemophilia A ; Hemophilia B ; Humans

China ; Hemophilia A ; therapy ; Hemophilia B ; therapy ; Humans ; Quality of Life

China ; epidemiology ; Hemophilia A ; epidemiology ; Hemophilia B ; epidemiology ; Humans ; Prevalence

Genetic Therapy ; Hemophilia B ; therapy ; Humans

Anticoagulants ; pharmacokinetics ; Child ; Hemophilia A ; drug therapy ; Hemophilia B ; drug therapy ; Humans ; Precision Medicine

BACKGROUND: Korean National Health Insurance reimburses factor VIII (FVIII) and factor IX (FIX) clotting factor concentrate (CFC) infusions to discrepant activity levels, allowing elevation of FVIII activity to 60 IU/dL and FIX to 40 IU/dL. We aimed to assess hemostatic response to these target levels using global hemostatic assays. METHODS: We enrolled 34 normal healthy men, 34 patients with hemophilia A, and 36 with hemophilia B, with residual factor activity of 3 IU/dL or less and without inhibitors. Patients with hemophilia A and B received injected CFCs according to reimbursement guidelines. Fifteen minutes after injection, we assessed hemostatic response with global hemostatic assays: thrombin generation assay (TGA), thromboelastography (TEG), and clot waveform analysis (CWA). RESULTS: Normal healthy men and patients with hemophilia A and B were 36.7, 37.2, and 35.1 years old, respectively. FVIII and recombinant FIX concentrate doses were 28.8 IU/kg and 43.6 IU/kg. Post-infusion FVIII activity rose from 0.5 IU/dL to 69.4 IU/dL, while FIX activity rose from 1.4 IU/dL to 46.8 IU/dL. Post-infusion peak thrombin concentrations in hemophilia A and B were 116.6 nM/L and 76.4 nM/L (P < 0.001). Post-infusion endogenous thrombin potential (ETP) in hemophilia A and B was 1349.8 nM/min and 915.6 nM (P < 0.001). TEG index of hemophilia A and B was 0.11 and −0.51 (P=0.006). CONCLUSION: Current reimbursed doses for FIX concentrates are insufficient to achieve hemostatic responses comparable to those after reimbursed doses for FVIII concentrates in terms of peak thrombin concentration, ETP, and TEG index.
Factor IX* ; Factor VIII* ; Hemophilia A ; Hemophilia B ; Humans ; Male ; National Health Programs ; Thrombelastography ; Thrombin

Hemophilia is an X-linked recessive disorder, which is classified into hemophilia A, defined by factor VIII deficiency and hemophilia B, defined by factor IX deficiency. The usual clinical presentation is spontaneous bleeding and prolonged activated partial thromboplastin time in a person without history of a coagulation disorder. The severity of hemophilia describes how serious a problem is and has been defined by a traditional classification into three forms: severe, moderate, mild. Hemophilia has never been reported after a rhinosinus surgery in otorhinolaryngology in Korea, but we encountered a 37-year-old man with hemophilia B who had undergone a rhinosinus surgery. He had no bleeding tendency in the past nor a family history for bleeding. But the patient presented with continuous nasal bleeding for a few days after surgery. We report this case of hemophilia B diagnosed after rhinosinus surgery that was cured with Factor IX replacement therapy with a review of the relevant literature.
Adult ; Classification ; Endoscopy ; Epistaxis ; Factor IX ; Hemophilia A* ; Hemophilia B* ; Hemorrhage ; Humans ; Korea ; Otolaryngology ; Partial Thromboplastin Time