Результаты поиска

[Use of recombinant activated factor VII (NovoSeven) in the treatment of a patient with idiopathic thrombocytopenic purpura complicated with subarachnoid and parenchimatous hemorrhage].

Efficiency of recombinant activated factor VII (rFVIIa) (NovoSeven) in treatment of patients with idiopathic thrombocytopenic purpura (ITP) complicated with subarachnoid and parenchimatous hemorrhage was shown in the article. The action of NovoSeven in patients with an affected megakaryocytic system depends on the binding of rFVIIa with activated thrombocites and on their surface rFVIIa provides the activation of X factor and thrombin formation. NovoSeven proved to be called a universal hemostatic medication for the idiopathic thrombocytopenic purpura treatment. Taking into account that transf…

Changes in protein C, factor VII and endothelial markers after autologous bone marrow transplantation: possible implications in the pathogenesis of veno-occlusive disease.

In a recent prospective study of allogenic bone marrow transplantation we reported that decreases in factor VII and protein C were predictive markers for high risk of veno-occlusive disease (VOD). In order to determine the relative involvement of endothelial and hepatocyte injury in the genesis of VOD, 34 consecutive patients undergoing autologous bone marrow transplantation (BMT) were studied. Conditioning was performed by chemotherapy alone or associated with total body irradiation (TBI). Protein C and factor VII, the endothelial markers Von Willebrand factor (vWF and t-PA, fibrinogen and fi…

Hereditary coagulation factor VII deficiency caused by novel compound heterozygous mutations c.572-1G>A and c.1037A>C in a Chinese pedigree.

Hereditary coagulation factor VII (FVII) deficiency is a rare autosomal recessive bleeding disorder. The aims of this study were to identify and verify the pathogenic mutation sites in a family with hereditary coagulation FVII deficiency, and preliminarily explore the underlying mechanisms. We identified a novel combination of compound heterozygous mutations, c.572-1G>A and c.1037A>C in F7 gene, associated with FVII deficiency. The splice site mutation c.572-1G>A led to a truncation, resulting in the loss of the essential catalytic domain of the FVII protein. The c.1037A>C missense mutation ha…

Приобретенный изолированный фактор VII Недостаток в ячейке Плазмы: краткое представление Two Дела, связанные с Плазмой-Келл-Леукемией, и обзор литературы.

Приобретенный изолированный фактор VII (FVII) недостаток является редким, но важным открытием у пациентов с расстройствами плазменных клеток, имеющими значительные терапевтические и прогнозные последствия. Цель настоящего анализа и обзора случаев, о которых сообщается в литературе, состоит в том, чтобы высветить связанные с болезнями характеристики, связанные с этим редким свертываемым дефектом, клиническими проявлениями и результатами, а также потенциальными основополагающими механизмами и дать руководящие указания в отношении методов лечения этих пациентов с точки зрения профилактических и т

Совпадение в аннотации оригинала
Acquired isolated factor VII (FVII) deficiency is a rare but important discovery in patients with plasma cell disorders with significant therapeutic and prognostic implications. The present analysis and review of cases reported in the literature is intended to highlight disease-related characteristics associated with this rare clotting defect, clinical manif…

Septic miscarriage with toxic shock syndrome and disseminated intravascular coagulation (DIC): The role of surgery, recombinant activated factor VII and intravenous immunoglobulin (IVIG).

Severe sepsis with multi-organ failure is associated with a high mortality rate. This case report highlights the challenges and modalities available in the management of a lady with refractory shock and disseminated intravascular coagulation (DIC) due to toxic shock syndrome (TSS) from genital tract sepsis. Early surgical intervention to remove the source of infection, the use of recombinant activated factor VII to treat intractable disseminated intravascular coagulation and intravenous immunoglobulin to neutralise the circulating exotoxins, have been employed and shown to drastically improve …

Experience with recombinant activated factor VII for severe post-partum hemorrhage in Japan, investigated by Perinatology Committee, Japan Society of Obstetrics and Gynecology.

AIM: To investigate the utility of recombinant activated Factor VII (rFVIIa) for severe post-partum hemorrhage (PPH) in Japan. METHODS: We studied 69 patients treated with rFVIIa for severe PPH; 44 patients were from the registry of Japan Society of Obstetrical, Gynecological and Neonatal Hematology, and 25 were identified by a survey of the Japan Society of Obstetrics and Gynecology. RESULTS: Overall, the mean and median blood loss were 11 835 mL and 8639 mL, respectively. Treatment before rFVIIa included transarterial embolization in 23 patients and hysterectomy in 38. Forty-two patients h…

Consensus protocol for the use of recombinant activated factor VII [eptacog alfa (activated); NovoSeven] in elective orthopaedic surgery in haemophilic patients with inhibitors.

Patients with haemophilia complicated by inhibitors have a significant burden of joint disease, which is associated with a negative impact on their quality of life. Successful elective orthopaedic surgery can result in decreased bleed frequency into a new joint, less time spent in hospital, increased mobility and improved well being. This paper describes a new protocol for use of recombinant activated factor VII (rFVIIa) in elective orthopaedic surgery, based on a review of published data as well as the personal experience of a group of expert physicians. The protocol offers guidance on the pl…

Decreased plasma levels of factor II + VII + X correlate with increased levels of soluble cytokine receptors in patients with malaria and meningococcal infections.

The levels of coagulation factors II + VII + X and of blood platelets (thrombocytes) as well as of cytokines and soluble cytokine receptors were studied in the patients with malaria or meningococcal infections. The coagulation factors were decreased particularly in the meningococcal patients, while thrombocytes were lowest in the Plasmodium falciparum malaria patients. There was no correlation between factors II + VII + X and thrombocytes, but plasma levels of coagulation factors II + VII + X were found to correlate inversely with levels of soluble interleukin-2 receptor (sIL-2R) and soluble t

Combined factor VII and protein C deficiency found in a patient with peripheral pulmonary artery stenosis accompanied by progressive pulmonary hypertension and hemoptysis.

A congenital deficiency of factor VII and protein C was found in a 21-year-old female suffering from recurrent and progressive attacks of dyspnea and hemoptysis over the last four years. She has been followed in our Department since the age of 17 under a diagnosis of peripheral pulmonary artery stenosis and pulmonary hypertension as confirmed by cardiac catheterization and angiography. Prolonged prothrombin time repeatedly examined during this time period prompted us to perform detailed coagulation studies. We found that factor VII and protein C were both half normal in activity as well as in …

A Case Report of Rare Coagulation Factor Abnormalities (Factors VII, XI, and XII) in a Patient With Chronic Subdural Hematoma and Treatment With Middle Meningeal Artery Embolization.

Acquired coagulopathy is uncommon. Cases of hemophilia A, which is characterized by reduced coagulation factor Ⅷ, have been reported but cas … , and XII. Emergency burr-hole irrigation was performed after administering recombinant coagulation factor VIIa. Two months later, the patient developed aphasia and recurrent hematoma. MMA embolization using N-butyl cyanoacrylate was performed to avoid recurrence, and burr-hole drainage reduced brain compression. The symptoms were relieved, and no recurrence …

Evaluation of Postoperative Thromboembolic Events in Orthotopic Heart Transplant Patients Receiving 4-Factor Prothrombin Complex Concentrate Monotherapy Versus Combination Therapy With Very Low-Dose Recombinant Activated Factor VII: A Retrospective Cohort Study.

OBJECTIVES: To explore whether the addition of very low-dose recombinant activated factor VII (rFVIIa) to 4-factor prothrombin complex concentrate (4F-PCC) increases postoperative thromboembolic events in patients undergoing orthotopic heart transplantation (OHT) complicated by severe coagulopathy. DESIGN: Single-center, retrospective cohort study. SETTING: Tertiary academic medical center in Boston, Massachusetts. PARTICIPANTS: Adults (aged ≥ 18 years) who underwent OHT between January 1 and December 31, 2023, and received 4F-PCC, with or without rFVIIa, for perioperative coagulopathy. IN…

Reduced plasma factor X is associated with a lack of response to recombinant activated factor VII in patients with hemophilia A and inhibitor, but does not impair emicizumab-driven hemostasis in vitro.

BACKGROUND: The hemostatic effect of recombinant (r) factor (F)VIIa after repetitive intermittent administration may be attenuated in patients with hemophilia A (PwHA) with inhibitors (PwHAwI) creating a clinically unresponsive status, although mechanism(s) remain to be clarified. In patients receiving prophylaxis treatment with emicizumab, concomitant rFVIIa is sometimes utilized in multiple doses for surgical procedures or breakthrough bleeding. AIM AND METHODS: We identified 'unresponsiveness' to rFVIIa, based on global coagulation function monitored using rotational thromboelastometry (RO

Analysis of the effect of initial hemostasis resuscitation with recombinant human coagulation factor VII a on the treatment of postoperative hemorrhage in cardiac surgery.

OBJECTIVE: To investigate the effectiveness of initial hemostatic resuscitation(IHR) on the treatment of bleeding with recombinant human coagulation factor VIIa after cardiac surgery. METHODS: The clinical data of patients who received rFVIIa hemostatic treatment after cardiac surgery at Beijing Anzhen Hospital, Capital Medical University, from January 1, 2021, to December 31, 2021 were retrospectively collected. A total of 152 cases were included in the study. In this study, initial hemostatic resuscitation was defined as a platelet count > 50,000 per µL and fibrinogen > 1.5 g/L when rFVIIa …

Factor VII deficiency and developmental abnormalities in a patient with partial monosomy of 13q and trisomy of 16p: case report and review of the literature.

BACKGROUND: Unbalanced chromosomal translocations may present with a variety of clinical and laboratory findings and provide insight into the functions of genes on the involved chromosomal segments. CASE PRESENTATION: A 9 year-old boy presented to our clinic with Factor VII deficiency, microcephaly, a seizure disorder, multiple midline abnormalities (agenesis of the corpus callosum, imperforate anus, bilateral optic nerve hypoplasia), developmental delay, hypopigmented macules, short 5th fingers, and sleep apnea due to enlarged tonsils. Cytogenetic and fluorescence in situ hybridization analy…

Human acute Chagas disease: changes in factor VII, activated protein C and hepatic enzymes from patients of oral outbreaks in Pará State (Brazilian Amazon).

Oral transmission of Chagas disease has been increasing in Latin American countries. The present study aimed to investigate changes in hepat … zole. Moreover, ACD individuals had higher plasma levels of activated protein C and lower levels of factor VII of the coagulation cascade. Patients with the highest parasite load had also the most increased transaminase levels. Also, ALT and AST were associated moderately (r = 0.429) and strongly (r = 0.595) with parasite load respectively. In conclusion, th …

Elevated prothrombin time on routine preoperative laboratory results in a healthy infant undergoing craniosynostosis repair: Diagnosis and perioperative management of congenital factor VII deficiency.

INTRODUCTION: Congenital factor VII deficiency is a rare bleeding disorder with high phenotypic variability. It is critical that children with congenital Factor VII deficiency be identified early when high-risk surgery is planned. Cranial vault surgery is common for children with craniosynostosis, and these surgeries are associated with significant morbidity mostly secondary to the risk of massive blood loss. PRESENTATION OF CASE: A two-month old infant who presented for elective craniosynostosis repair was noted to have an elevated prothrombin time (PT) with a normal activated partial thromb…

Raised protein levels and altered cellular expression of factor VII activating protease (FSAP) in the lungs of patients with acute respiratory distress syndrome (ARDS).

BACKGROUND: The acute respiratory distress syndrome (ARDS) is characterised by inflammation of the lung parenchyma and changes in alveolar haemostasis with extravascular fibrin deposition. Factor VII activating protease (FSAP) is a recently described serine protease in plasma and tissues known to be involved in haemostasis, cell proliferation and migration. METHODS: The level of FSAP protein expression was examined by western blotting/ELISA/immunohistochemistry and its activity was investigated by coagulation/fibrinolysis assays in plasma, bronchoalveolar lavage (BAL) fluid and lung tissue of…

Age estimates of ancestral mutations causing factor VII deficiency and Dubin-Johnson syndrome in Iranian and Moroccan Jews are consistent with ancient Jewish migrations.

Factor VII (FVII) deficiency and Dubin-Johnson syndrome (DJS) are rare autosomal recessive disorders caused by mutations in F7 and MRP2 genes, respectively. Both disorders are relatively frequent among Iranian and Moroccan Jews. FVII deficiency in both populations is caused by a founder A244V mutation in the F7 gene and DJS is caused by two founder mutations, I1173F and R1150H in the MRP2 gene that are specific for Iranian and Moroccan Jewish patients, respectively. We estimated the age of FVII A244V and MRP2 I1173F by analysis of microsatellite markers flanking F7 and MRP2 genes, respectively…

Risk factors of diffuse alveolar hemorrhage after acute ischemic stroke treated with tissue-type plasminogen activator. The effectiveness of activated recombinant factor VII treatment.

BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a rare and frequently life-threatening complication of a variety of conditions. DAH may result from coagulation disorders, inhaled toxins, or infections. We report a series of patients who developed DAH after receiving a tissue-type plasminogen activator (tPA) for acute cerebral infarction. We aimed to find risk factors of DAH in patients receiving tPA and the effectiveness of activated recombinant factor VII (rFVIIa) treatment for the same. CASE DESCRIPTION: A total of 1023 acute ischemic stroke (AIS) patients who received tPA in our departmen…

A thrombophilic allele of clotting Factor VII/VIIa promoting recurrent pulmonary emboli, clinical details, and a structural model of the altered protein: a case report.

BACKGROUND: The clotting or hemostasis system is a meticulously regulated set of enzymatic reactions that occur in the blood and culminate i … prior to elective surgery for an umbilical hernia. He was given low doses of NovoSeven (therapeutic Factor VIIa) and had no unusual bleeding or clotting during the surgery. In fact, during his entire clinical course he had no unprovoked bleeding. Bleeding instances occurred with hemostatic stresses such as gastritis, kidney calculus, orthopedic surgery, or t …