Results 91 to 100 of about 31,702 (234)

Hemophilia treatment and transfusion medicine

Zhongguo shuxue zazhi
The improvement of hemophilia treatment is attributed to the development of transfusion medicine, and the discovery of cryoprecipitate has opened up a new era of hemophilia treatment, laying the foundation for the development of modern hemophilia ...
FANG Yunhai, CHENG Yan, ZHANG Xinsheng
doaj   +1 more source

Perioperative Outcomes in Factor XI Deficiency: A Systematic Review

Haemophilia, EarlyView.
ABSTRACT Background Congenital factor XI (FXI) deficiency has a variable bleeding phenotype, and FXI activity alone does not predict perioperative bleeding. Management practices remain heterogeneous, and available evidence derives from case reports, small case series, and retrospective cohorts.
Zainab Al‐Housni, Raeesah Mohammed, Silvio Ligia, Jim Douketis, Chatree Chai‐Adisaksopha, Alfonso Iorio, Davide Matino   +6 more
wiley   +1 more source

Optimizing Perioperative Management of Haemophilia B With rFIX‐FP: Pharmacokinetic Validation of the Hemoptidose Tool

Haemophilia, EarlyView.
Abstract Background Surgical management of haemophilia B requires precise factor IX replacement to ensure adequate haemostasis while optimizing factor consumption. Extended half‐life rFIX‐FP simplifies perioperative management but exhibits substantial pharmacokinetic variability.
Xavier Delavenne, Célia Vollard, Nicolas Drillaud, Sabine Castet, Nicolas Luyck, Emilie Hénin, Julien Denis‐Le Seve, Fabienne Volot, Brigitte Tardy, Stéphanie Désage, Benoît Guillet   +10 more
wiley   +1 more source

Responsiveness and Minimal Important Change of the Haemophilia Activities List in Patients With Inherited Bleeding Disorders

Haemophilia, EarlyView.
ABSTRACT Introduction The Haemophilia Activities List (HAL) is a disease‐specific patient‐reported outcome measure (PROM) that is widely used in both healthcare and research in people with inherited bleeding disorders (PWBD), but information on responsiveness and minimal important change (MIC) is lacking.
Johan Blokzijl, Isolde A. R. Kuijlaars, Martijn F. Pisters, Cindy Veenhof, Janjaap van der Net, Roger E. G. Schutgens, Kathelijn Fischer, Merel A. Timmer   +7 more
wiley   +1 more source

Functional evaluation of joint in moderate to severe hemophilia patients treated with on-demand factor replacement: insights from a single hemophilia treatment center in Bangladesh

The Egyptian Journal of Internal Medicine
Background Hemophilia patients are prone to spontaneous or post-traumatic bleeding, particularly in joints and various other body sites. The high incidence of joint hemorrhages in hemophilia patients underscores the importance of routine joint ...
Sujan Ghosh, Sanzina Sadia Tory, Humayra Nazneen, Nurul Farhad, Salwa Islam, Mohammad Jahid Hasan, Akhil Ranjan Biswas   +6 more
doaj   +1 more source

Evaluation of Safety and Efficacy of Emicizumab Prophylaxis in Egyptian Pediatric Patients with Hemophilia A

Turkish Journal of Hematology
Objective: Hemophilia A (HA) is a hereditary X-linked bleeding disorder secondary to deficiency of the clotting factor VIII (FVIII). Emicizumab is a monoclonal antibody that replaces the function of the activated FVIII and prevents bleeding in HA ...
Tamer Hassan, Marwa Zakaria, Manar Fathy, Ahmed Farag, Eman Abdelhady, Dalia Gameil, Mustafa Abu Hashem   +6 more
doaj   +1 more source

An Ex Vivo Pharmacodynamic Study of KN057, a Tissue Factor Pathway Inhibitor Neutralizing Antibody, in Plasma Samples From Patients With Haemophilia or VWD3

Haemophilia, EarlyView.
ABSTRACT Introduction Tissue factor pathway inhibitor (TFPI), a key regulator of tissue factor‐initiated coagulation through FXa‐dependent inhibition of the tissue factor‐FVIIa complex, has emerged as a promising target for restoring thrombin generation.
Mankai Ju, Feng Xue, Wei Sun, Fang Li, Ting Xu, Renchi Yang   +5 more
wiley   +1 more source

Italian Patients Journey for Gene Therapy in Haemophilia A

Haemophilia, EarlyView.
ABSTRACT Introduction Gene therapy (GT) provides sustained FVIII levels without repeated infusions in Hemophilia A (HA) patients thus overcoming a major limitation of replacement therapy. However, issues remain e.g., patient selection criteria, duration and variability of transgene expression, quality of life and long‐term safety.
Giovanni Di Minno, Gaia Spadarella, Alessandra Borchiellini, Anna Borrelli, Andrea Buzzi, Giancarlo Castaman, Michele Cecchi, Raimondo De Cristofaro, Vincenzo La Mura, Angelo Lupi, Giuseppe Malcangi, Maria Elisa Mancuso, Maria Francesca Mansueto, Cristina Santoro, Rita Carlotta Santoro, Luigi Piero Solimeno, Annarita Tagliaferri, Ezio Zanon, Isabella Garagiola, Flora Peyvandi   +19 more
wiley   +1 more source

Optimizing Emergency Department Care for People With Bleeding Disorders: A Scoping Review of Barriers and Interventions for Improvement

Haemophilia, EarlyView.
ABSTRACT Background Emergency department (ED) care is critical for managing acute bleeding events in people with bleeding disorders. Despite international guidelines recommending haemostatic treatment within 30–60 min, delays and deviations from best practices are common and associated with poorer outcomes.
Ling‐Yi Guo, Diane L. Lorenzetti, Natalia Rydz, Dawn Goodyear, Julia Hews‐Girard, Garrick Mok, Roy Khalife, Kelsey Uminski   +7 more
wiley   +1 more source

GENETIC DIAGNOSTICS OF HEMOPHILIA A

Zdravniški Vestnik, 2004
Background. Hemophilia A is a chromosome X-linked bleeding disorder due to mutations in the FVIII gene. There are 163 hemophilia A patients in Slovene registry for Hemophilia.
Maruša Debeljak, Majda Benedik Dolničar, Lana Strmecki   +2 more
doaj