Factor VII deficiency is rare. It is an autosomal recessive inherited disease with an estimated prevalence of 1/1,000,000. We report the case of a newborn male from first-degree consanguineous parents admitted at 15 d...Factor VII deficiency is rare. It is an autosomal recessive inherited disease with an estimated prevalence of 1/1,000,000. We report the case of a newborn male from first-degree consanguineous parents admitted at 15 days of life due to a hemorrhagic syndrome. Hemostasis tests showed low prothrombin time (PT) and normal activated partial thromboplastin time (aPTT). A coagulation panel revealed isolated factor VII deficiency. In this case, we highlight the clinical, biological, and therapeutic aspects of this condition during the neonatal period.展开更多
BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth ...BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth of pregnant women with congenital FVIID is difficult.Recombinant factor VIIa is the most common replacement therapy for FVIID.However,no unified diagnosis and treatment plan for pregnant women with congenital FVIID has been established.CASE SUMMARY We report the clinical history of a pregnant woman who was considered to have congenital FVIID.Recombinant factor VIIa was prophylactically administered to the pregnant woman at the time of cervical fully opening.She successfully delivered a live infant without any complications,such as postpartum hemorrhage,neonatal abnormalities,and so on.CONCLUSION Prophylaxis of recombinant factor VIIa during delivery can effectively reduce the incidence of postpartum hemorrhage among pregnant women with congenital FVIID associated with a high risk of bleeding.展开更多
Breast cancer is a leading cause of cancer death in women, worldwide. Fortunately, breast cancer is relatively chemosensitive, with recent advances leading to the development of effective therapeutic strategies, signi...Breast cancer is a leading cause of cancer death in women, worldwide. Fortunately, breast cancer is relatively chemosensitive, with recent advances leading to the development of effective therapeutic strategies, significantly increasing disease cure rate. However, disease recurrence and treatment of cases lacking therapeutic molecular targets, such as epidermal growth factor receptor 2 and hormone receptors, referred to as triple-negative breast cancers, still pose major hurdles in the treatment of breast cancer. Thus, novel therapeutic approaches to treat aggressive breast cancers are essential. Blood coagulation factor VII(fV II) is produced in the liver and secreted into the blood stream. Tissue factor(TF), the cellular receptor for fV II, is an integral membrane protein that plays key roles in the extrinsic coagulation cascade. TF is overexpressed in breast cancer tissues. The TF-fV II complex may be formed in the absence of injury, because f VII potentially exists in the tissue fluid within cancer tissues. The active form of this complex(TF-fV IIa) may stimulate the expression of numerous malignant phenotypes in breast cancer cells. Thus, the TF-fV II pathway is a potentially attractive target for breast cancer treatment. To date, a number of studies investigating the mecha-nisms by which TF-fV II signaling contributes to breast cancer progression, have been conducted. In this review, we summarize the mechanisms controlling TF and fV II synthesis and regulation in breast cancer cells. Our current understanding of the TF-fV II pathway as a mediator of breast cancer progression will be also described. Finally, we will discuss how this knowledge can be applied to the design of future therapeutic strategies.展开更多
To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prol...To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prolongation of the prothrombin time(PT)with a normal activated partial thromboplastin time(aPTT).Factor assay and genotyping for FVII can be done to confirm the diagnosis.1,2 Patients may present with menorrhagia,and bleeding tendencies may vary from slight gingival bleeding,epistaxis,and ecchymosis,to severe bleeding,such as gastrointestinal and intracranial bleeding.展开更多
A study was carried out on the alternate activation of factor IX （FIX） by bovine FVII and human tissue factor （TF） rather than by activated factor XI （FXI）. The reaction product of bovine FVII and human TF funct...A study was carried out on the alternate activation of factor IX （FIX） by bovine FVII and human tissue factor （TF） rather than by activated factor XI （FXI）. The reaction product of bovine FVII and human TF functioned as a FIX activator in the assay system used. Published studies suggest that in the presence of Caions, the complex of human FVII-TF readily activates both human FIX and human FX,and at low TF concentrations, FIX appears to be the preferred substrate for the reaction product of FVII and TF. This may explain the discrepancy between the mild bleeding of hereditary FXI deficiency and the severe bleeding of hereditery FIX deficieney. The results obtained with bovine FVII and a crude human TF preparation confirm that at low TF concentrations,bovine FIX is the preferred substrate rather than FX. At higher TF concentrations, bovine FX was rapidly activated.展开更多
BACKGROUND： Activated clotting factor VII has been demonstrated to exhibit obvious anti-apoptosis effects. OBJECTIVE： To observe the effect of activated clotting factor VII on neuronal apoptosis at different time po...BACKGROUND： Activated clotting factor VII has been demonstrated to exhibit obvious anti-apoptosis effects. OBJECTIVE： To observe the effect of activated clotting factor VII on neuronal apoptosis at different time points following rat intracerebral hemorrhage （ICH）. DESIGN, TIME AND SETTING： A randomized, controlled, animal experiment was performed at the Neurobiological Laboratory of Second Military Medical University from October 2005 to April 2006. MATERIALS： Recombinant-activated clotting factor Vlla （rFVtla） was purchased from Danish Novo Nordisk, Denmark. In situ cell death detection kit-POD kit was purchased from Roche, Switzerland. Caspase-3 activity determination kit from Biovision, USA. METHODS： A total of 72 healthy, male, Sprague Dawley rats, aged 5-8 months, were randomly assigned to three groups （n = 24）： sham-operated, ICH model, and rFVIla. In the ICH model and rFVIla groups, 80.0μL autologous non-clotting blood from rat tails was injected into the right caudate putamen to establish the ICH. The empty microinjector was inserted into the caudate putamen in the sham-operated group. The ICH model and rFVIla groups were subdivided into four subsets separately： 6, 24, 72 hours and 7 days following ICH. The rats in the rFVIla group were injected with 160 μg/kg rFVIla via the dorsal vein of the penis. MAIN OUTCOME MEASURES： Apoptotic cells were detected in the right caudate putamen by TUNEL; caspase-3 activity by spectrophotometry; and rat neurological function was evaluated by neurological functional impairment scales. RESULTS： Rat neurological function was deteriorated at 24, 72 hours, and 7 days following ICH. The TUNEL-positive cells and caspase-3 activity in the right caudate putamen was significantly increased in the ICH rats （P 〈 0.05）; rFVlla treatment reduced the number of TUNEL-positive cells and caspase-3 activity in the right caudate putamen （P 〈 0.05）, and neurological function was significantly improved （P 〈 0.05）. CONCLUSION： rFVIla was applied within 72 hours after tCH, which reduced the amount of neuronal apoptosis and promoted neurological function restoration by possibly inhibiting caspase-3 activity.展开更多
目的观察凝血因子VII(coagulationf actor VII,FVII)R353Q基因多态性在华南汉族健康者人群中的分布。方法提取华南汉族60名正常健康者基因组DNA,应用多聚酶链反应(PCR)技术和限制性内切酶片段长度多态性技术检测上述人群的FVII R353Q...目的观察凝血因子VII(coagulationf actor VII,FVII)R353Q基因多态性在华南汉族健康者人群中的分布。方法提取华南汉族60名正常健康者基因组DNA,应用多聚酶链反应(PCR)技术和限制性内切酶片段长度多态性技术检测上述人群的FVII R353Q基因型。结果华南汉族人群FVII基因R353Q具3种基因型(RR,RQ,QQ),FVII等位基因R、Q基因频率在人群中分别为90.9%,9.1%。基因型频率符合Hardy-Weinberg平衡定律。结论华南汉族健康人群凝血因子VII基因R353Q多态具3种基因型(RR,RQ,QQ),R等位基因携带频率明显高于Q等位基因。展开更多
文摘Factor VII deficiency is rare. It is an autosomal recessive inherited disease with an estimated prevalence of 1/1,000,000. We report the case of a newborn male from first-degree consanguineous parents admitted at 15 days of life due to a hemorrhagic syndrome. Hemostasis tests showed low prothrombin time (PT) and normal activated partial thromboplastin time (aPTT). A coagulation panel revealed isolated factor VII deficiency. In this case, we highlight the clinical, biological, and therapeutic aspects of this condition during the neonatal period.
基金Supported by Natural Science Foundation of Guangxi,No.2018JJB140171Medical Excellence Award Funded by the Creative Research Development Grant from the First Affiliated Hospital of Guangxi Medical University(2020)+1 种基金Development and Application of Appropriate Medical and Health Technologies in Guangxi,No.S2018111Self-funded Scientific Research Project of Guangxi Health Commission,No.Z20190512.
文摘BACKGROUND Congenital factor VII deficiency(FVIID)is a rare autosomal recessive genetic disorder.The clinical manifestations of this deficiency vary greatly.Predicting the risk of bleeding during and after childbirth of pregnant women with congenital FVIID is difficult.Recombinant factor VIIa is the most common replacement therapy for FVIID.However,no unified diagnosis and treatment plan for pregnant women with congenital FVIID has been established.CASE SUMMARY We report the clinical history of a pregnant woman who was considered to have congenital FVIID.Recombinant factor VIIa was prophylactically administered to the pregnant woman at the time of cervical fully opening.She successfully delivered a live infant without any complications,such as postpartum hemorrhage,neonatal abnormalities,and so on.CONCLUSION Prophylaxis of recombinant factor VIIa during delivery can effectively reduce the incidence of postpartum hemorrhage among pregnant women with congenital FVIID associated with a high risk of bleeding.
文摘Breast cancer is a leading cause of cancer death in women, worldwide. Fortunately, breast cancer is relatively chemosensitive, with recent advances leading to the development of effective therapeutic strategies, significantly increasing disease cure rate. However, disease recurrence and treatment of cases lacking therapeutic molecular targets, such as epidermal growth factor receptor 2 and hormone receptors, referred to as triple-negative breast cancers, still pose major hurdles in the treatment of breast cancer. Thus, novel therapeutic approaches to treat aggressive breast cancers are essential. Blood coagulation factor VII(fV II) is produced in the liver and secreted into the blood stream. Tissue factor(TF), the cellular receptor for fV II, is an integral membrane protein that plays key roles in the extrinsic coagulation cascade. TF is overexpressed in breast cancer tissues. The TF-fV II complex may be formed in the absence of injury, because f VII potentially exists in the tissue fluid within cancer tissues. The active form of this complex(TF-fV IIa) may stimulate the expression of numerous malignant phenotypes in breast cancer cells. Thus, the TF-fV II pathway is a potentially attractive target for breast cancer treatment. To date, a number of studies investigating the mecha-nisms by which TF-fV II signaling contributes to breast cancer progression, have been conducted. In this review, we summarize the mechanisms controlling TF and fV II synthesis and regulation in breast cancer cells. Our current understanding of the TF-fV II pathway as a mediator of breast cancer progression will be also described. Finally, we will discuss how this knowledge can be applied to the design of future therapeutic strategies.
文摘To editor:Factor VII(FVII)is a determining factor in activating the exogenous coagulation pathway;F7 gene mutation decreases the FVII number or function.Factor VII deficiency(FVIID)is characterized by an isolated prolongation of the prothrombin time(PT)with a normal activated partial thromboplastin time(aPTT).Factor assay and genotyping for FVII can be done to confirm the diagnosis.1,2 Patients may present with menorrhagia,and bleeding tendencies may vary from slight gingival bleeding,epistaxis,and ecchymosis,to severe bleeding,such as gastrointestinal and intracranial bleeding.
文摘A study was carried out on the alternate activation of factor IX （FIX） by bovine FVII and human tissue factor （TF） rather than by activated factor XI （FXI）. The reaction product of bovine FVII and human TF functioned as a FIX activator in the assay system used. Published studies suggest that in the presence of Caions, the complex of human FVII-TF readily activates both human FIX and human FX,and at low TF concentrations, FIX appears to be the preferred substrate for the reaction product of FVII and TF. This may explain the discrepancy between the mild bleeding of hereditary FXI deficiency and the severe bleeding of hereditery FIX deficieney. The results obtained with bovine FVII and a crude human TF preparation confirm that at low TF concentrations,bovine FIX is the preferred substrate rather than FX. At higher TF concentrations, bovine FX was rapidly activated.
文摘BACKGROUND： Activated clotting factor VII has been demonstrated to exhibit obvious anti-apoptosis effects. OBJECTIVE： To observe the effect of activated clotting factor VII on neuronal apoptosis at different time points following rat intracerebral hemorrhage （ICH）. DESIGN, TIME AND SETTING： A randomized, controlled, animal experiment was performed at the Neurobiological Laboratory of Second Military Medical University from October 2005 to April 2006. MATERIALS： Recombinant-activated clotting factor Vlla （rFVtla） was purchased from Danish Novo Nordisk, Denmark. In situ cell death detection kit-POD kit was purchased from Roche, Switzerland. Caspase-3 activity determination kit from Biovision, USA. METHODS： A total of 72 healthy, male, Sprague Dawley rats, aged 5-8 months, were randomly assigned to three groups （n = 24）： sham-operated, ICH model, and rFVIla. In the ICH model and rFVIla groups, 80.0μL autologous non-clotting blood from rat tails was injected into the right caudate putamen to establish the ICH. The empty microinjector was inserted into the caudate putamen in the sham-operated group. The ICH model and rFVIla groups were subdivided into four subsets separately： 6, 24, 72 hours and 7 days following ICH. The rats in the rFVIla group were injected with 160 μg/kg rFVIla via the dorsal vein of the penis. MAIN OUTCOME MEASURES： Apoptotic cells were detected in the right caudate putamen by TUNEL; caspase-3 activity by spectrophotometry; and rat neurological function was evaluated by neurological functional impairment scales. RESULTS： Rat neurological function was deteriorated at 24, 72 hours, and 7 days following ICH. The TUNEL-positive cells and caspase-3 activity in the right caudate putamen was significantly increased in the ICH rats （P 〈 0.05）; rFVlla treatment reduced the number of TUNEL-positive cells and caspase-3 activity in the right caudate putamen （P 〈 0.05）, and neurological function was significantly improved （P 〈 0.05）. CONCLUSION： rFVIla was applied within 72 hours after tCH, which reduced the amount of neuronal apoptosis and promoted neurological function restoration by possibly inhibiting caspase-3 activity.
文摘目的观察凝血因子VII(coagulationf actor VII,FVII)R353Q基因多态性在华南汉族健康者人群中的分布。方法提取华南汉族60名正常健康者基因组DNA,应用多聚酶链反应(PCR)技术和限制性内切酶片段长度多态性技术检测上述人群的FVII R353Q基因型。结果华南汉族人群FVII基因R353Q具3种基因型(RR,RQ,QQ),FVII等位基因R、Q基因频率在人群中分别为90.9%,9.1%。基因型频率符合Hardy-Weinberg平衡定律。结论华南汉族健康人群凝血因子VII基因R353Q多态具3种基因型(RR,RQ,QQ),R等位基因携带频率明显高于Q等位基因。
