Blood Coagulation Factors ; antagonists & inhibitors ; Hemophilia A ; blood ; Humans

The authors conducted an investigation focusing mainly on the activities of the inhibitory factors of the coagulation and fibrinolysis processes in 35 normal adults and 72 liver cirrhosis and/or hepatoma patients. The activities of antithrombin III, protein C, and alpha 2-plasmin inhibitor were reduced to less than 50% in patients with decreased hepatic synthetic function while lupus anticoagulant was detected in more than 50% of patients with decreased hepatic synthetic function. Hemostatic abnormalities in advanced lived diseases may be caused partly by a decrease of coagulation and fibrinolysis inhibitors and the presence of lupus anticoagulant.
Adult ; Antifibrinolytic Agents/blood ; Blood Coagulation Factors/antagonists & inhibitors/immunology/metabolism ; Carcinoma, Hepatocellular/*blood ; Hemostasis ; Humans ; Liver Cirrhosis/*blood ; Liver Neoplasms/*blood ; Lupus Coagulation Inhibitor

Blood Coagulation Factor Inhibitors ; antagonists & inhibitors ; blood ; Factor VIII ; administration & dosage ; antagonists & inhibitors ; immunology ; Hemophilia A ; drug therapy ; genetics ; immunology ; Humans ; Immune Tolerance ; Isoantibodies ; blood ; immunology ; Recombinant Proteins ; adverse effects ; immunology ; therapeutic use ; Risk Factors ; Time Factors

OBJECTIVETo study the current situation of coagulation factor VIII (FVIII) inhibitor development in children with hemophilia A (HA) through a cross-sectional survey, and to explore the risk factors of inhibitor development in order to provide evidence for further prevention and management strategies.

METHODThe clinical data of outpatients with hemophilia A in Beijing Children's Hospital seen from November 2012 to May 2013 were collected, FVIII inhibitor was screened and analyzed its risk factors.

RESULTA total of 102 HA children were enrolled, 5 were mild cases, 32 were moderate, and 65 were severe cases; the median age on enrollment was 55.5 (3.0-200.0) months:19(18.6%) of patients had inhibitors and 9 (8.8%) had low-titer inhibitors, 10 (9.8%) had high-titer inhibitors. Receiving FVIII treatment for life-threatening bleeding (P = 0.03) ,OR 4.10 (95%CI:1.17-14.32) was a risk factor for inhibitor generation and patients within 20 exposure days have more chances of inhibitor development (P = 0.04) ,OR 3.32 (95%CI:1.02-10.86) . High and intense FVIII exposure within short term was the risk factor for high titer inhibitor development (P = 0.01) ,OR 5.25 (95%CI:1.45-21.92) .

CONCLUSIONIntense FVIII exposure for severe hemorrhage was the risk factor of inhibitors development especially of high titer inhibitors.

Adolescent ; Blood Coagulation Factor Inhibitors ; blood ; Child ; Child, Preschool ; Cross-Sectional Studies ; Dose-Response Relationship, Drug ; Factor VIII ; administration & dosage ; antagonists & inhibitors ; Female ; Hemophilia A ; blood ; therapy ; Humans ; Infant ; Male ; Multivariate Analysis ; Risk Factors ; Time Factors