Factor XII (Hageman factor) is an important protease that plays a major role in the initiation of the intrinsic pathway of blood coagulation and fibrinolysis and kinin formation. It is still unclear whether factor XII...Factor XII (Hageman factor) is an important protease that plays a major role in the initiation of the intrinsic pathway of blood coagulation and fibrinolysis and kinin formation. It is still unclear whether factor XII deficiency causes any disorders during pregnancy. Because the main clinical feature in patients with factor XII deficiency is thrombosis rather than bleeding, low dose aspirin would be expected to prevent first trimester miscarriage and a decrease in factor XII level itself was found to be an independent risk factor in recurrent miscarriage. The woman in a 31-year-old patient, with personal and family antecedents without interest and preceding obstetrics of two spontaneous abortions in the first quarter of the pregnancy without apparent cause. In the study of infertility practiced emphasizes a partition of not more than one centimetre of length in the uterine found by hysteroscopy exploration and in the study of hipercoagulability a light deficiency of the factor XII. Himself guideline processing with low dose of aspirin (125 mgr/day) and preconception folic acid (5 mgr/day), remains expectant mother and in the week 12 of her third pregnancy itself guideline antitrombotic prophylaxis with heparin of low molecular weight by subcutaneous way. The pregnancy reaches the week 39 without incidents of interest and the expectant mother give birth of spontaneous form to health boy. Repeated abortions may be associated with reduced level of factor XII activity of unknown origin and low-dose aspirin may prevent miscarriage caused for decreased factor XII levels in patients with a history of recurrent first trimester miscarriage.展开更多
Background: Factor XII (Hageman Factor) is the initiating factor for the Intrinsic Pathway of Coagulation. Very low levels of Factor XII have been associated with increased levels of activated Partial Thromboplastin T...Background: Factor XII (Hageman Factor) is the initiating factor for the Intrinsic Pathway of Coagulation. Very low levels of Factor XII have been associated with increased levels of activated Partial Thromboplastin Time (aPTT). Association of Factor XII deficiency is more with thromboembolic disorders rather than bleeding tendencies. Aim: To learn more about the relationship of factor XII (Hageman Factor) deficiency and high levels of activated Partial Thromboplastin Time. Case Presentation: The Patient was admitted with complains of recurrent headaches and loin pain. Patient was incidentally found to have prolonged activated Partial Thromboplastin Time. This led to investigations which ultimately provided the evidence of severely low levels of Hageman Factor. Conclusion: Hageman Factor deficiency causes prolonged activated Partial Thromboplastin Time. However, most of the patients are asymptomatic for many years despite Hageman Factor deficiency.展开更多
BACKGROUND Pulmonary embolism(PE)is a fatal clinical syndrome that is generally caused by an embolus from unstable deep venous thrombosis(DVT).However,clinical and biochemical factors that are related to the stability...BACKGROUND Pulmonary embolism(PE)is a fatal clinical syndrome that is generally caused by an embolus from unstable deep venous thrombosis(DVT).However,clinical and biochemical factors that are related to the stability of DVT are not fully understood.AIM To evaluate the relationships between plasma antigen levels of factor XII(FXII:Ag)and factor XI(FXI:Ag)with the stability of DVT.METHODS Patients with DVT and no PE,DVT and PE,and controls with no DVT or PE that matched for age,gender,and comorbidities were included in this study.FXII:Ag and FXI:Ag in peripheral venous blood were measured using enzyme-linked immunosorbent assays.RESULTS Using the 95th percentile of FXI:Ag in patients with DVT and PE as the cut-off,a higher FXI:Ag was associated with a higher risk of unstable DVT(odds ratio:3.15,95%confidence interval:1.18-8.43,P=0.019).Stratified analyses showed consistent results in patients≤60 years(P=0.020),but not in those>60 years(P=0.346).CONCLUSION Higher plasma FXI:Ag might be a marker for unstable DVT,which might be associated with PE in these patients.展开更多
Hereditary angioedema(HAE)is an uncommon genetic disorder characterized by recurrent episodes of edema involving subcutaneous tissue and submucosa.The pathogenesis of HAE reflects an intricate coordinated regulation o...Hereditary angioedema(HAE)is an uncommon genetic disorder characterized by recurrent episodes of edema involving subcutaneous tissue and submucosa.The pathogenesis of HAE reflects an intricate coordinated regulation of components of complement,kinin and hemostatic pathway.Till date,mutations in 4 different genes have been identified to cause HAE which includes serine protease inhibitor G1(SERPING1),factor XII(F12),plasminogen(PLG)and angiopoietin 1(ANGPT 1).These mutations lead to increased bradykinin 2 receptor mediated signalling via increased production of bradykinin except mutations in ANGPT1 gene that disturbs the cytoskeletal assembly of vascular endothelial cells.In this review we aim to summarize the recent advances in the pathogenesis and genetics of HAE.We also provide an overview of possible future prospects in the identification of new genetic defects in HAE.展开更多
文摘Factor XII (Hageman factor) is an important protease that plays a major role in the initiation of the intrinsic pathway of blood coagulation and fibrinolysis and kinin formation. It is still unclear whether factor XII deficiency causes any disorders during pregnancy. Because the main clinical feature in patients with factor XII deficiency is thrombosis rather than bleeding, low dose aspirin would be expected to prevent first trimester miscarriage and a decrease in factor XII level itself was found to be an independent risk factor in recurrent miscarriage. The woman in a 31-year-old patient, with personal and family antecedents without interest and preceding obstetrics of two spontaneous abortions in the first quarter of the pregnancy without apparent cause. In the study of infertility practiced emphasizes a partition of not more than one centimetre of length in the uterine found by hysteroscopy exploration and in the study of hipercoagulability a light deficiency of the factor XII. Himself guideline processing with low dose of aspirin (125 mgr/day) and preconception folic acid (5 mgr/day), remains expectant mother and in the week 12 of her third pregnancy itself guideline antitrombotic prophylaxis with heparin of low molecular weight by subcutaneous way. The pregnancy reaches the week 39 without incidents of interest and the expectant mother give birth of spontaneous form to health boy. Repeated abortions may be associated with reduced level of factor XII activity of unknown origin and low-dose aspirin may prevent miscarriage caused for decreased factor XII levels in patients with a history of recurrent first trimester miscarriage.
文摘Background: Factor XII (Hageman Factor) is the initiating factor for the Intrinsic Pathway of Coagulation. Very low levels of Factor XII have been associated with increased levels of activated Partial Thromboplastin Time (aPTT). Association of Factor XII deficiency is more with thromboembolic disorders rather than bleeding tendencies. Aim: To learn more about the relationship of factor XII (Hageman Factor) deficiency and high levels of activated Partial Thromboplastin Time. Case Presentation: The Patient was admitted with complains of recurrent headaches and loin pain. Patient was incidentally found to have prolonged activated Partial Thromboplastin Time. This led to investigations which ultimately provided the evidence of severely low levels of Hageman Factor. Conclusion: Hageman Factor deficiency causes prolonged activated Partial Thromboplastin Time. However, most of the patients are asymptomatic for many years despite Hageman Factor deficiency.
基金Supported by the Fund for Key Research and Development Program Project of Shannxi ProvinceNo. 2017SF-254
文摘BACKGROUND Pulmonary embolism(PE)is a fatal clinical syndrome that is generally caused by an embolus from unstable deep venous thrombosis(DVT).However,clinical and biochemical factors that are related to the stability of DVT are not fully understood.AIM To evaluate the relationships between plasma antigen levels of factor XII(FXII:Ag)and factor XI(FXI:Ag)with the stability of DVT.METHODS Patients with DVT and no PE,DVT and PE,and controls with no DVT or PE that matched for age,gender,and comorbidities were included in this study.FXII:Ag and FXI:Ag in peripheral venous blood were measured using enzyme-linked immunosorbent assays.RESULTS Using the 95th percentile of FXI:Ag in patients with DVT and PE as the cut-off,a higher FXI:Ag was associated with a higher risk of unstable DVT(odds ratio:3.15,95%confidence interval:1.18-8.43,P=0.019).Stratified analyses showed consistent results in patients≤60 years(P=0.020),but not in those>60 years(P=0.346).CONCLUSION Higher plasma FXI:Ag might be a marker for unstable DVT,which might be associated with PE in these patients.
文摘Hereditary angioedema(HAE)is an uncommon genetic disorder characterized by recurrent episodes of edema involving subcutaneous tissue and submucosa.The pathogenesis of HAE reflects an intricate coordinated regulation of components of complement,kinin and hemostatic pathway.Till date,mutations in 4 different genes have been identified to cause HAE which includes serine protease inhibitor G1(SERPING1),factor XII(F12),plasminogen(PLG)and angiopoietin 1(ANGPT 1).These mutations lead to increased bradykinin 2 receptor mediated signalling via increased production of bradykinin except mutations in ANGPT1 gene that disturbs the cytoskeletal assembly of vascular endothelial cells.In this review we aim to summarize the recent advances in the pathogenesis and genetics of HAE.We also provide an overview of possible future prospects in the identification of new genetic defects in HAE.
