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28,010 results
  • Role of L-Arginine on the Expression of Coagulation Factor VIII Gene. [Journal Article]
    Altern Ther Health Med. 2023 Feb 03 [Online ahead of print]Zhang ZW, Xu DM, … Yin J
  • CONCLUSIONS: A1 and A2 domain genes had promoter function and could initiate the transcription and expression of CAT gene, but A3, C1 and C2 domain genes could not. Moreover, L-arginine can significantly enhance transcription and expression of human coagulation factor VIII via A2 domain.
  • Complement protein C3a enhances adaptive immune responses towards FVIII products. [Journal Article]
    Haematologica. 2023 Feb 02 [Online ahead of print]Ringler E, Iannazzo SO, … Waibler Z
  • The most serious complication in hemophilia A (HA) treatment is the development of factor (F)VIII inhibitors or anti-drug antibodies (ADA) occurring in 25-35% of patients with severe HA. The immunological mechanisms underlying the development of ADA against FVIII products have not been completely understood yet. Immunological danger signals associated with events such as infection or surgery have…
  • Efanesoctocog Alfa Prophylaxis for Patients with Severe Hemophilia A. [Clinical Trial, Phase III]
    N Engl J Med. 2023 Jan 26; 388(4):310-318.von Drygalski A, Chowdary P, … XTEND-1 Trial Group
  • CONCLUSIONS: In patients with severe hemophilia A, once-weekly efanesoctocog alfa provided superior bleeding prevention to prestudy prophylaxis, normal to near-normal factor VIII activity, and improvements in physical health, pain, and joint health. (Funded by Sanofi and Sobi; XTEND-1 ClinicalTrials.gov number, NCT04161495.).
  • [NEW INNOVATIONS IN THE TREATMENT OF HEMOPHILIA]. [Review]
    Harefuah. 2023 Jan; 162(1):47-51.Levy-Mendelovich S, Brutman-Barazani T, Kenet G
  • Hemophilia is a congenital bleeding disorder with a deficiency of coagulation factor 8 or 9 (hemophilia A or B, respectively) and a tendency for recurrent bleeding, especially into muscles and joints, which may cause orthopedic damage and necessitate joint replacement surgeries at a young age. In recent years, there has been a huge breakthrough in the treatment of hemophilia. Until recently, the …
  • Protein S Deficiency with Recurrent Deep Vein Thrombosis and Post Thrombotic Syndrome: A Case Report. [Case Reports]
    JNMA J Nepal Med Assoc. 2022 Oct 01; 60(254):892-894.Neupane S, Pudasaini P, … Subedi B
  • Protein S is a vitamin K-dependent protein that acts as a break in secondary hemostasis by inactivating activated factor V and activated factor VIII. We report a case of a 40 years old male who had the first episode of deep vein thrombosis of the left lower limb 10 years back, which despite treatment, reoccurred 3 months later in the bilateral lower limb. Thrombophilic screening showed severe pro…
  • Therapeutic potential of fetal liver cells transplantation in hemophilia A mice. [Journal Article]
    Haematologica. 2023 Jan 26 [Online ahead of print]Merlin S, Akula S, … Follenzi A
  • Hemophilia A (HA) cell therapy approaches in pediatric individuals require suitable factor (F)VIII-producing cells for stable engraftment. Liver sinusoidal endothelial cells (LSEC) and hematopoietic stem cells (HSC) have been demonstrated to be suitable for the treatment of adult HA-mice. However, after transplantation in busulfan (BU)-conditioned newborn mice, adult LSEC/HSC cannot efficiently e…
  • Acquired Hemophilia A In Adults: A Multicenter Study from Turkey. [Journal Article]
    Indian J Hematol Blood Transfus. 2023 Jan; 39(1):107-115.Arslan Davulcu E, Demirci Z, … Şahin F
  • Acquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression. In this multicenter study, we aimed to demonstrate the clinical characteristics, management details, and survival of A…
  • Gene therapy for hemophilia: looking beyond factor expression. [Review]
    Exp Biol Med (Maywood). 2022 Dec; 247(24):2223-2232.Yamaguti-Hayakawa GG, Ozelo MC
  • Hemophilia A (factor VIII [FVIII] deficiency) and hemophilia B (factor IX [FIX] deficiency) are the X-linked recessive bleeding disorders that clinically manifest with recurrent bleeding, predominantly into muscles and joints. In its severe presentation, when factor activity is less than 1% of normal, hemophilia presents with spontaneous musculoskeletal bleeds and may progress to debilitating chr…
  • Factor assay in victims of snake bite: Experience from a tertiary care institute in South India. [Journal Article]
    Asian J Transfus Sci. 2022 Jul-Dec; 16(2):175-179.Rafi AM, Innah SJ
  • CONCLUSIONS: Early detection and treatment of envenomation remains the cornerstone of managing snake venom-induced consumptive coagulopathy. Anti-snake venom plays a major role in the reversal of coagulopathy. Blood and blood products would be useful when coagulopathy does not revert by ASV alone. Evidence-based transfusion can be implemented and cryoprecipitate may be used as many of the patients had factor XIII and fibrinogen deficiency as part of venom-induced coagulopathy. To improve patient management and thereby the outcome of patients CMEs and training programs for the treating physicians also has to be implemented so that guidelines are formulated and followed.
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