Titre : Facteur IX

Facteur IX : Questions médicales fréquentes

Termes MeSH sélectionnés :

Factor IX
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"10.1055/s-0042-1744379" } }, { "@type": "ScholarlyArticle", "name": "Nanobodies against factor XI apple 3 domain inhibit binding of factor IX and reveal a novel binding site for high molecular weight kininogen.", "datePublished": "2022-07-20", "url": "https://questionsmedicales.fr/article/35815349", "identifier": { "@type": "PropertyValue", "propertyID": "DOI", "value": "10.1111/jth.15815" } } ], "breadcrumb": { "@type": "BreadcrumbList", "itemListElement": [ { "@type": "ListItem", "position": 1, "name": "questionsmedicales.fr", "item": "https://questionsmedicales.fr" }, { "@type": "ListItem", "position": 2, "name": "Facteurs biologiques", "item": "https://questionsmedicales.fr/mesh/D001685" }, { "@type": "ListItem", "position": 3, "name": "Facteurs de la coagulation sanguine", "item": "https://questionsmedicales.fr/mesh/D001779" }, { "@type": "ListItem", "position": 4, "name": "Facteur IX", "item": "https://questionsmedicales.fr/mesh/D005164" } ] } }, { "@type": "MedicalWebPage", 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internes ?\nLes saignements de nez sont-ils fréquents ?\nLes femmes peuvent-elles avoir des symptômes d'hémophilie B ?\nLes symptômes varient-ils selon l'âge ?", "url": "https://questionsmedicales.fr/mesh/D005164?mesh_terms=Factor+IX&page=2#section-symptômes" }, { "@type": "MedicalWebPage", "name": "Prévention", "headline": "Prévention sur Facteur IX", "description": "Comment prévenir les saignements chez les patients ?\nLes vaccinations sont-elles importantes pour ces patients ?\nQuels conseils de mode de vie peuvent aider ?\nLes patients doivent-ils porter un bracelet médical ?\nComment l'éducation des patients aide-t-elle ?", "url": "https://questionsmedicales.fr/mesh/D005164?mesh_terms=Factor+IX&page=2#section-prévention" }, { "@type": "MedicalWebPage", "name": "Traitements", "headline": "Traitements sur Facteur IX", "description": "Quel est le traitement principal pour l'hémophilie B ?\nY a-t-il des traitements préventifs ?\nLes patients peuvent-ils auto-administrer le facteur IX ?\nQuels sont les effets secondaires des traitements ?\nDes thérapies géniques existent-elles pour l'hémophilie B ?", "url": "https://questionsmedicales.fr/mesh/D005164?mesh_terms=Factor+IX&page=2#section-traitements" }, { "@type": "MedicalWebPage", "name": "Complications", "headline": "Complications sur Facteur IX", "description": "Quelles sont les complications possibles de l'hémophilie B ?\nLes saignements peuvent-ils causer des dommages permanents ?\nComment les infections peuvent-elles survenir ?\nLes patients sont-ils à risque de thrombose ?\nComment gérer les complications liées aux articulations ?", "url": "https://questionsmedicales.fr/mesh/D005164?mesh_terms=Factor+IX&page=2#section-complications" }, { "@type": "MedicalWebPage", "name": "Facteurs de risque", "headline": "Facteurs de risque sur Facteur IX", "description": "Quels sont les facteurs de risque pour l'hémophilie B ?\nLes hommes sont-ils plus à risque que les femmes ?\nLes antécédents médicaux influencent-ils le risque ?\nLes traitements antérieurs affectent-ils le risque ?\nL'âge joue-t-il un rôle dans le risque d'hémophilie B ?", "url": "https://questionsmedicales.fr/mesh/D005164?mesh_terms=Factor+IX&page=2#section-facteurs de risque" } ] }, { "@type": "FAQPage", "mainEntity": [ { "@type": "Question", "name": "Comment diagnostiquer une déficience en facteur IX ?", "position": 1, "acceptedAnswer": { "@type": "Answer", "text": "Un test sanguin mesure le niveau de facteur IX pour confirmer la déficience." } }, { "@type": "Question", "name": "Quels tests sont utilisés pour évaluer le facteur IX ?", "position": 2, "acceptedAnswer": { "@type": "Answer", "text": "Les tests de temps de prothrombine et de temps de thromboplastine partielle activée sont utilisés." } }, { "@type": "Question", "name": "Quels symptômes indiquent un déficit en facteur IX ?", "position": 3, "acceptedAnswer": { "@type": "Answer", "text": "Des saignements excessifs, des ecchymoses fréquentes et des douleurs articulaires peuvent indiquer un déficit." } }, { "@type": "Question", "name": "Le diagnostic de l'hémophilie B est-il génétique ?", "position": 4, "acceptedAnswer": { "@type": "Answer", "text": "Oui, des tests génétiques peuvent confirmer une mutation du gène du facteur IX." } }, { "@type": "Question", "name": "Quel rôle joue l'historique médical dans le diagnostic ?", "position": 5, "acceptedAnswer": { "@type": "Answer", "text": "L'historique médical aide à identifier des antécédents familiaux de troubles de la coagulation." } }, { "@type": "Question", "name": "Quels sont les symptômes courants de l'hémophilie B ?", "position": 6, "acceptedAnswer": { "@type": "Answer", "text": "Les symptômes incluent des saignements prolongés, des ecchymoses et des saignements internes." } }, { "@type": "Question", "name": "Comment se manifestent les saignements internes ?", "position": 7, "acceptedAnswer": { "@type": "Answer", "text": "Les saignements internes peuvent se manifester par des douleurs abdominales ou des douleurs articulaires." } }, { "@type": "Question", "name": "Les saignements de nez sont-ils fréquents ?", "position": 8, "acceptedAnswer": { "@type": "Answer", "text": "Oui, les saignements de nez fréquents peuvent être un symptôme d'hémophilie B." } }, { "@type": "Question", "name": "Les femmes peuvent-elles avoir des symptômes d'hémophilie B ?", "position": 9, "acceptedAnswer": { "@type": "Answer", "text": "Oui, bien que rare, les femmes peuvent être porteuses et présenter des symptômes légers." } }, { "@type": "Question", "name": "Les symptômes varient-ils selon l'âge ?", "position": 10, "acceptedAnswer": { "@type": "Answer", "text": "Oui, les symptômes peuvent varier en fonction de l'âge et de la gravité de la déficience." } }, { "@type": "Question", "name": "Comment prévenir les saignements chez les patients ?", "position": 11, "acceptedAnswer": { "@type": "Answer", "text": "Éviter les activités à risque et suivre un traitement prophylactique de facteur IX." } }, { "@type": "Question", "name": "Les vaccinations sont-elles importantes pour ces patients ?", "position": 12, "acceptedAnswer": { "@type": "Answer", "text": "Oui, les vaccinations sont cruciales pour prévenir les infections, surtout en cas de traitement." } }, { "@type": "Question", "name": "Quels conseils de mode de vie peuvent aider ?", "position": 13, "acceptedAnswer": { "@type": "Answer", "text": "Adopter un mode de vie sain, éviter les sports de contact et gérer le stress sont conseillés." } }, { "@type": "Question", "name": "Les patients doivent-ils porter un bracelet médical ?", "position": 14, "acceptedAnswer": { "@type": "Answer", "text": "Oui, un bracelet médical peut informer les autres de leur condition en cas d'urgence." } }, { "@type": "Question", "name": "Comment l'éducation des patients aide-t-elle ?", "position": 15, "acceptedAnswer": { "@type": "Answer", "text": "L'éducation aide les patients à comprendre leur condition et à gérer les saignements." } }, { "@type": "Question", "name": "Quel est le traitement principal pour l'hémophilie B ?", "position": 16, "acceptedAnswer": { "@type": "Answer", "text": "Le traitement principal est l'administration de facteur IX par voie intraveineuse." } }, { "@type": "Question", "name": "Y a-t-il des traitements préventifs ?", "position": 17, "acceptedAnswer": { "@type": "Answer", "text": "Oui, des traitements préventifs réguliers de facteur IX peuvent réduire les saignements." } }, { "@type": "Question", "name": "Les patients peuvent-ils auto-administrer le facteur IX ?", "position": 18, "acceptedAnswer": { "@type": "Answer", "text": "Oui, certains patients peuvent apprendre à s'auto-administrer le facteur IX à domicile." } }, { "@type": "Question", "name": "Quels sont les effets secondaires des traitements ?", "position": 19, "acceptedAnswer": { "@type": "Answer", "text": "Les effets secondaires peuvent inclure des réactions allergiques et des infections au site d'injection." } }, { "@type": "Question", "name": "Des thérapies géniques existent-elles pour l'hémophilie B ?", "position": 20, "acceptedAnswer": { "@type": "Answer", "text": "Oui, des thérapies géniques sont en développement pour traiter l'hémophilie B." } }, { "@type": "Question", "name": "Quelles sont les complications possibles de l'hémophilie B ?", "position": 21, "acceptedAnswer": { "@type": "Answer", "text": "Les complications incluent des saignements internes, des douleurs articulaires et des infections." } }, { "@type": "Question", "name": "Les saignements peuvent-ils causer des dommages permanents ?", "position": 22, "acceptedAnswer": { "@type": "Answer", "text": "Oui, des saignements répétés peuvent entraîner des dommages articulaires permanents." } }, { "@type": "Question", "name": "Comment les infections peuvent-elles survenir ?", "position": 23, "acceptedAnswer": { "@type": "Answer", "text": "Les infections peuvent survenir par l'utilisation de produits sanguins contaminés." } }, { "@type": "Question", "name": "Les patients sont-ils à risque de thrombose ?", "position": 24, "acceptedAnswer": { "@type": "Answer", "text": "Oui, certains traitements peuvent augmenter le risque de thrombose chez les patients." } }, { "@type": "Question", "name": "Comment gérer les complications liées aux articulations ?", "position": 25, "acceptedAnswer": { "@type": "Answer", "text": "La physiothérapie et des traitements anti-inflammatoires peuvent aider à gérer les douleurs articulaires." } }, { "@type": "Question", "name": "Quels sont les facteurs de risque pour l'hémophilie B ?", "position": 26, "acceptedAnswer": { "@type": "Answer", "text": "Les facteurs de risque incluent des antécédents familiaux et des mutations génétiques." } }, { "@type": "Question", "name": "Les hommes sont-ils plus à risque que les femmes ?", "position": 27, "acceptedAnswer": { "@type": "Answer", "text": "Oui, l'hémophilie B est plus fréquente chez les hommes en raison de son mode de transmission génétique." } }, { "@type": "Question", "name": "Les antécédents médicaux influencent-ils le risque ?", "position": 28, "acceptedAnswer": { "@type": "Answer", "text": "Oui, des antécédents médicaux de troubles de la coagulation augmentent le risque." } }, { "@type": "Question", "name": "Les traitements antérieurs affectent-ils le risque ?", "position": 29, "acceptedAnswer": { "@type": "Answer", "text": "Oui, des traitements antérieurs avec des produits sanguins peuvent augmenter le risque d'infections." } }, { "@type": "Question", "name": "L'âge joue-t-il un rôle dans le risque d'hémophilie B ?", "position": 30, "acceptedAnswer": { "@type": "Answer", "text": "L'âge n'est pas un facteur de risque direct, mais les complications peuvent augmenter avec l'âge." } } ] } ] }

Sources (10000 au total)

An updated interactive database for 1692 genetic variants in coagulation factor IX provides detailed insights into hemophilia B.

Genetic variants in coagulation factor IX (FIX) are associated with hemophilia B, a rare bleeding disease. F9 variants are widespread across the gene and were summarized in our FIX variant database in... We aimed to rationalize the molecular basis for 598 new F9 variants and 1645 new clinical cases, totaling 1692 F9 variants and 5358 related patient cases.... New F9 variants were identified from publications and online resources, and compiled into a MySQL database for comparison with the human FIXa protein structure.... The new total of 1692 F9 variants correspond to 406 (88%) of the 461 FIX residues and now include 70 additional residues. They comprise 945 unique point variants, 281 deletions, 352 polymorphisms, 63 ... The even distribution of the F9 variants revealed few mutational hotspots, and most variants were associated with small perturbations in the FIX protein structure. The updated database will assist cli...

Nanobodies against factor XI apple 3 domain inhibit binding of factor IX and reveal a novel binding site for high molecular weight kininogen.

Factor XI (FXI) is a promising target for novel anticoagulants because it shows a strong relation to thromboembolic diseases, while fulfilling a mostly supportive role in hemostasis. Anticoagulants ta... To generate nanobodies that can interfere with FXIa mediated activation of factor IX (FIX).... Nanobodies were selected for binding to the apple 3 domain of FXI and their effects on FXI and coagulation were measured in purified protein systems as well as in plasma-based coagulation assays. Addi... We have identified five nanobodies that inhibit FIX activation by FXI by competing with the FIX binding site on FXI. Interestingly, a sixth nanobody was found to target a different binding epitope in ... We have characterized a nanobody targeting the FXI apple 3 domain that elucidates the binding orientation of HK on FXI. Moreover, we have produced five nanobodies that can inhibit the FXI-FIX interact...

Comprehensive analysis and prediction of long-term durability of factor IX activity following etranacogene dezaparvovec gene therapy in the treatment of hemophilia B.

Congenital hemophilia B is a rare bleeding disorder caused by defects in the gene encoding factor IX (FIX) leading to coagulation deficiency. Recurrent bleeds may cause chronic pain, disability, and r... Participants from Phase 2 b (... Bayesian and Frequentist linear mixed models predicted no more than 6/55 (10.91%) observed participants would have FIX activity levels <2% up to 25.5 years post-infusion. Bayesian model-based predicti... People with hemophilia B receiving etranacogene dezaparvovec would likely achieve durable FIX activity levels and remain free of prophylactic FIX replacement products for up to 25.5 years following si...

Little discrepancy between one-stage and chromogenic factor VIII (FVIII)/IX assays in a large international cohort of persons with nonsevere hemophilia A and B.

Accurate measurements of coagulation factor activity form an essential part of hemophilia management and are performed by the one-stage or chromogenic assay. Current literature suggests that approxima... To investigate the extent of factor VIII/IX one-stage and chromogenic assay discrepancy in moderate and mild hemophilia A and B.... Persons with previously diagnosed nonsevere hemophilia A and B with a factor level of 2 to 35 IU/dL were included from the international DYNAMO cohort study. Central measurements of the factor VIII an... A total of 220 persons were included, of whom 3 (1%) showed assay discrepancy: 2/175 hemophilia A and 1/45 hemophilia B. Six persons (3%) exhibited an absolute difference >10 IU/dL between the assay r... Little assay discrepancy was observed despite the presence of mutations previously associated with discrepancy, suggesting that the presence and magnitude of assay discrepancy are largely determined b...

Activated partial thromboplastin time-based clot waveform analysis enables measurement of very low levels of factor IX activity in patients with severe hemophilia B.

The precise measurement of very low levels of factor IX activity (FIX:C < 1 IU/dL) is essential for understanding clinical severity and risk of inhibitor development in patients with severe hemophilia...

Surgical outcomes in patients with haemophilia A or B receiving extended half-life recombinant factor VIII and IX Fc fusion proteins: Real-world experience in the Nordic countries.

Perioperative dosing recommendations vary across Nordic haemophilia treatment centres (HTCs) for extended half-life (EHL) factor concentrates in haemophilia A/B (HA/HB) patients.... To summarise Nordic real-world surgical experiences with EHL recombinant factor VIII/IX Fc (rFVIIIFc/rFIXFc) fusion proteins using retrospective data from clinical records at four HTCs in Finland, Swe... Factor dosing and surgical outcomes were recorded from HA/HB patients who underwent surgery and were treated with rFVIIIFc/rFIXFc. Perioperative factor dosing regimens were clinician-determined based ... Twenty five surgeries were performed on 20 patients, all covered by bolus injections except one minor HA surgery; eight minor surgeries were in paediatric patients. Median preoperative rFVIIIFc dose f... Nordic real-world experiences suggest that EHL products can be used safely and effectively for peri-operative haemostasis. Further research is required to develop local dosing guidelines for optimised...