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

<b>OBJECTIVEb>To analyze the clinical characteristics, diagnosis and treatment of pediatric hemophilia in single center over the decade.

<b>METHODSb>A retrospective study was conducted with 520 hemophilic children hospitalized in our medical center between January 2002 and December 2012.

<b>RESULTSb>All the patients were male including 438 hemophilia A (HA) and 82 hemophilia B (HB). There were significant differences in APTT between severe and mild- to moderate hemophilia (P<0.05). In pediatric HA and HB, delay time of diagnosis were 1.42 and 1.17 year, respectively. Children of 7-12 years were the largest population of visiting a doctor, and the spontaneous bleeding episode was the main cause. The most common hemorrhage site was soft tissue in early childhood, but joint was increasingly affected with age as children growth. All bleeding sites and frequencies were not associated with plasma factor level of patient (P>0.05). Knee and anKle were mainly involved in early child, while elbow and shoulder were involved increasingly in later childhood. Additionally, in HA and HB, inhibitor occurrence were 8.9%(19/214) and 12.8%(5/39), inducing 78.9%(15/19) and 40.0%(2/5) of high titer inhalator, and antiHCV-positive rate were 2.8%(11/397) and 2.5%(2/79), respectively.

<b>CONCLUSIONb>Our data highlights that delay in diagnosis and blood-borne infections were significantly reduced over the decade, but the development of inhibitor still remains a major challenge with wide-scale usage of factor in replacement therapy.

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