Extended Half-Life Recombinant Factor VIII Conjugated with Modifying Substances Does Not Affect Fibrin Clot Formation or Stability in Haemophilia A Blood Samples [0.03%]
带有修饰物的长效重组凝血因子VIII不影响甲型血友病血液样品中纤维蛋白凝块的形成或稳定性
Naruto Shimonishi,Keiji Nogami
Naruto Shimonishi
Introduction: Various extended half-life recombinant factor VIII (EHL-FVIII) products have been designed to improve the pharmacokinetic properties of FVIII, allowing prolonged haemostatic coverage and reducing the injecti...
The Analysis of the FIX-inhibitor Risks Associated With the F9 Genotype in Patients With Haemophilia B Exposes the Involvement of Nonsense Mediated-decay: Argentinean and International Series [0.03%]
FIX抑制剂的风险与血友病B患者的F9基因型相关的分析揭示了 nonsense介导的衰减作用：阿根廷和国际系列研究
Betiana Michelle Ziegler,Miguel Martín Abelleyro,María Emilia Villegas et al.
Betiana Michelle Ziegler et al.
Introduction: Haemophilia B (HB) associates with deleterious variants in F9. HB management with FIX-concentrate infusions may be ineffective in 3%-9% patients who develop FIX-inhibitors. ...
Prevalence of F8 Intron 22 Inversion in Severe Haemophilia A: Molecular Insights From a Cohort of Punjab Province of Pakistan [0.03%]
巴基斯坦旁遮普省重度甲型血友病患者中F8第22内含子倒位的流行率：队列分子见解
Muhammad Kashif Mughal,Ghulam Mustafa,Yusra Rashid et al.
Muhammad Kashif Mughal et al.
Introduction: Haemophilia A (HA) is caused by an inherited deficiency of factor VIII. Intron 22 inversion is the most common genetic mutation that causes severe disease. The study aims to determine the prevalence of F8 In...
Symptoms, Diagnosis, and Treatment for Women and Girls With Hemophilia: A Narrative Review [0.03%]
女性血友病的症状、诊断与治疗：文献综述
Sam Hirniak,Carla Samuel,Faria Aditi et al.
Sam Hirniak et al.
Introduction: Hemophilia is an X-linked bleeding disorder previously thought to present only in men. This sentiment is rapidly changing as the information around women and girls' experiences of bleeding symptoms has evolv...
Pathogenesis of Haemophilia A in a Chinese Family With F8-Intron 22 Inversion and Skewed X-Chromosome Inactivation [0.03%]
F8基因第22内含子倒位及X染色体非整倍性遗传异常华氏甲型病人的发病机制研究
Yingli Ren,Haihao Shen,Beibei Sun et al.
Yingli Ren et al.
Smartphone-Based Teledentistry to Support Clinical Triage and Risk-Informed Dental Care in Patients With Inherited Bleeding and Haemoglobin Disorders: A Cross-Sectional Diagnostic Agreement Study [0.03%]
基于智能手机的牙科远程医疗支持临床分诊及血液病和血红蛋白病患者的循证牙科治疗：诊断一致性横断面研究
Victor Cordeiro da Silva,Carolina Mendes Frusca do Monte,Leandro Tavares da Silva et al.
Victor Cordeiro da Silva et al.
Introduction: Patients with inherited bleeding and haemoglobin disorders face barriers to accessing timely dental care, increasing the risk of untreated oral disease and complications related to invasive procedures. ...
Haemophilia Management in Natural Disasters-From Field to Consensus Report [0.03%]
从现场到共识报告：血友病自然灾害管理
Ahmet Muzaffer Demir,Selin Aytaç,Serap Karaman et al.
Ahmet Muzaffer Demir et al.
Objective: The earthquake in Türkiye had a profound impact on both haemophilia patients and healthcare services in the region. This experience highlighted the need for an effective action plan for patients with bleeding ...
Preimplantation Genetic Testing for Families at Risk of Haemophilia: Ten-Year Single-Centre Experience [0.03%]
血友病高风险家庭的植入前遗传学检测：单中心十年经验回顾
Mimosa Mortarino,Isabella Garagiola,Valeria Nicotra et al.
Mimosa Mortarino et al.
Introduction: Preimplantation genetic testing for monogenic diseases (PGT-M) is a reproductive option for couples at high risk of transmitting inherited disorders. We report a ten-year single-centre PGT-M experience in fa...
Real-World Outcomes of Prophylaxis With rIX-FP in Germany: A Prospective, Non-Interventional Study in Haemophilia B [0.03%]
德国使用rIX-FP预防治疗的研究：血友病B患者的前瞻性、非干预性研究
Johannes Oldenburg,Swee Wenning,Susanne Holzhauer et al.
Johannes Oldenburg et al.
Introduction: Efficacy and safety of albutrepenonacog alfa (rIX-FP) in people with haemophilia B (PwHB) has been previously demonstrated in clinical trials. However, real-world data insights are required. ...
Prophylactic Factor Replacement Versus on-Demand Factor Replacement Following Primary Total Knee Arthroplasty in Patients With Hemophilia: A Retrospective Study With an Average 10-Year Follow-up [0.03%]
一项平均随访期长达十年的回顾性研究：血友病患者初次全膝关节置换术后实施预防性凝血因子替代疗法与按需治疗的效果比较
Zhongyu Luo,Xiaoying Zhong,Chen Zhang et al.
Zhongyu Luo et al.
Background: The long-term benefits of prophylactic factor replacement following total knee arthroplasty in patients with hemophilia remain insufficiently evaluated. ...