A5051926280- Hemophilia Treatment and Research
- Blood Coagulation and Thrombosis Mechanisms
- Platelet Disorders and Treatments
- Hemostasis and retained surgical items
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Chronic Myeloid Leukemia Treatments
- Transplantation: Methods and Outcomes
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Organ Transplantation Techniques and Outcomes
- Cancer-related gene regulation
- CAR-T cell therapy research
- Orthopaedic implants and arthroplasty
- Clinical Laboratory Practices and Quality Control
- Renal Transplantation Outcomes and Treatments
- Liver Disease Diagnosis and Treatment
- Metabolism and Genetic Disorders
- Total Knee Arthroplasty Outcomes
- Virus-based gene therapy research
- Protein purification and stability
- Surgical Simulation and Training
- Heme Oxygenase-1 and Carbon Monoxide
- Porphyrin Metabolism and Disorders
- Heparin-Induced Thrombocytopenia and Thrombosis
- Antiplatelet Therapy and Cardiovascular Diseases
- Trauma, Hemostasis, Coagulopathy, Resuscitation
Lille’s Cardiology Hospital
2017-2025
Hôpital Louis Pradel
2016-2025
Hospices Civils de Lyon
2005-2025
Hôpital Edouard Herriot
2008-2023
Université Claude Bernard Lyon 1
2015-2020
Hôpital Saint-Antoine
1990-1991
Centre Hospitalier Universitaire de Grenoble
1991
Sorbonne Université
1990-1991
Summary In haemophilia patients, a relationship is usually observed between the clinical expression of disease and plasmatic factor VIII/factor IX (FVIII/FIX) activity. However, it known from experience, that some despite similar FVIII/FIX plasma levels, could exhibit different bleeding phenotype. After determining preanalytical test conditions, we evaluated thrombin generation capacity samples in various conditions potential usefulness (TGT) patients. series 46 patients (34 A 12 B...
Emicizumab is a recombinant, humanized, bispecific, monoclonal antibody that bridges activated factor IX and X to restore the function of deficient VIII. Treatment bleeding in patients with hemophilia inhibitors involves use bypassing agents (BPA).These molecules are also used for management breakthrough bleeds on prophylaxis emicizumab, increased concerns about risks combining two procoagulant drugs. Using thrombin generation assay, we tailored dosage prothrombin complex concentrate (APCC)...
Abstract Introduction Early prophylaxis is the gold standard of care for severe haemophilia. The development subcutaneous Factor VIII (FVIII) mimetics, such as emicizumab, has significantly reduced disease burden and improved protection against bleeding episodes. Despite its benefits, emicizumab does not fully normalize haemostasis, requiring additional FVIII treatment surgical procedures management breakthrough bleeding. In these cases, extended or ultra‐extended half‐life products are most...
Summary. In haemophilia patients with inhibitor, elective orthopaedic surgery is usually performed under recombinant activated factor VII (rFVIIa). We report here the case of a severe A patient high inhibitor who needed bilateral total knee arthroplasty. Recombinant FVIIa was previously shown to be ineffective for treatment muscle and joint bleedings, he had history excessive postoperative bleeding prothrombin complex concentrate (APCC). Thrombin generation test (TGT) used assess efficacy...
Summary Thirty per cent of patients with mild haemophilia A (MHA) present markedly different FVIII: C level when assayed by one‐stage clotting and two‐stage chromogenic assays. It is, therefore, a real clinical challenge to predict the individual bleeding risk these patients. The aim work was study relationship between tendency results panel phenotypic genotypic tools. Thirty‐six MHA were included in this multicentre prospective study. severity symptoms evaluated using ISTH/SSC score....
Background and Objectives Total knee replacement (TKR) is the treatment of choice in case end‐stage arthropathy, main complication haemophilia. We report here a retrospective evaluation 72 total 51 haemophilia A B patients using continuous infusion factor concentrates (CIFC). Materials Methods Patients were evaluated on basis following efficacy safety criteria: range motion, surgery‐related blood loss by three different methods, consumption occurrence short long term complications. Results...
Summary Factor VIII Inhibitor Bypassing Activity ( FEIBA ) can effectively achieve haemostasis in haemophilia patients with inhibitors. Further evaluation of surgical settings is significant interest considering the relatively limited prospective data published to date. The aim study evaluate perioperative efficacy and safety Haemophilia inhibitors who underwent procedures received for haemostatic control were prospectively enrolled an open‐label, noninterventional, postauthorization [ SUR...
Recombinant activated factor VII (rFVIIa, NovoSeven®) was first developed for treating those haemophilia patients with inhibitors who cannot benefit from conventional therapies. Several clinical trials have clearly demonstrated that rFVIIa is a safe and effective therapy home treatment of mild-to-moderate bleeding episodes. Its theoretical inability to abnormally activate the coagulation system has also prompted many clinicians use it in elective surgical procedures. Recommended dose ranges...
Introduction Recombinant factor IX fusion protein (r ‐ FP ) has been developed to improve the pharmacokinetic ( PK profile of FIX ), allowing maintenance desired activity between injections at extended intervals, ultimately optimizing haemophilia B treatment. Aim To determine efficacy and safety r in perioperative setting. Methods Subjects were adult paediatric patients with severe moderately ≤ 2%) participating three Phase III clinical trials undergoing a surgical procedure. profiles...
For patients with severe haemophilia A, guidelines recommend prophylactic treatment FVIII, dose calculations targeting a predetermined FVIII trough level. However, this pharmacokinetic (PK) approach is suboptimal, some experiencing breakthrough bleeds. We aimed to improve dosing by incorporating the thrombin generation assay, global haemostasis assay whose main pharmacodynamic (PD) parameter, endogenous potential (ETP), predicts spontaneous bleeding risk.We performed post hoc combined PK-PD...
Summary. It has been reported that thrombin generation test (TGT) may be a useful tool to monitor recombinant factor VIIa (rFVIIa). However, TGT does not reflect the stability of fibrin clot and its resistance fibrinolysis which are crucial. Using whole-blood thromboelastography (TEG) tissue plasminogen activator (tPA), we developed an in-vitro model assess stability. Fibrin fibres were thicker in haemophiliacs compared with controls (P < 0.0001). After addition rFVIIa 90 μg kg−1, diameter...
The thrombin generation (TG) assay evaluates haemostatic balance, which is influenced by the levels of many coagulation factors and inhibitors. Our objective was to identify determinant TG in haemophilia A (HA) B (HB) patients compare them those healthy controls. Coagulation factor inhibitor levels, TG, were measured platelet-poor plasma from 40 with HA, 32 HB subjects. Data analysed using multiple regression models. In HA patients, VIII a positive endogenous potential (ETP) peak, whereas...
Abstract Introduction Hemophilia is a rare constitutional bleeding disorder due to deficiency in Factor VIII or IX. Recurrent hemarthroses, one of the major complications disease, lead hemophilic arthropathy, disabling condition that requires early diagnosis. Traditionally, clinical examination and plain film radiography have been used diagnose arthropathy. Magnetic resonance imaging (MRI) ultrasound can be more useful for diagnosing soft‐tissue changes. However, but each these methods has...
Bleeding severity in severe haemophilic patients, with low thrombin generation (TG) capacity, can vary widely between possibly reflecting differences tissue factor pathway inhibitor (TFPI) level.
Venous thrombosis is a very rare occurrence in patients with haemophilia A. The majority of these cases occurred during or after the administration clotting factor concentrates. We report case patient severe A, who spontaneously developed deep venous (DVT). superficial femoral vein an extension profunda femoris vein. Neither any local anatomic compression nor predisposing thrombophilic risk factors were identified. Treatment recombinant VIII at prophylactic doses associated unfractionated...
Abstract Introduction With current molecular diagnosis, about 1 to 5% of haemophilia A (HA) patients remain genetically unresolved. In these cases, deep intronic variation or structural disrupting the F8 gene could be causal. Aim To identify causal in four unresolved mild‐to‐severe HA using an mRNA analysis approach. Methods Ectopic was performed unrelated patients. An vitro minigene assay order confirm deleterious splicing impact each identified. Results all probands, revealed aberrant...
In patients with FXI deficiency, the risk of surgery-related bleeding is poorly correlated plasma activity (FXI:C); latter can therefore not be used as a reliable predictor in surgeries.The aim this retrospective study was to determine whether thrombin generation assay (TGA) could evaluate FXI-deficient patients. TGA parameters were compared FXI:C values, haemostatic treatments and surgical outcomes.All followed at haemophilia treatment care centre (Lyon, France) < 50IU/dL, for whom baseline...