Results 1 to 10 of about 113,523 (138)

Successful immunosuppressive drug‐free immune tolerance induction in hemophilia B with inhibitor and anaphylaxis to factor IX: A case report [PDF]

Clinical Case Reports
Key Clinical Message Recommendations advise factor IX desensitization before immune tolerance induction in severe hemophilia B, supported by immunosuppression.
Ángeles Palomo Bravo, Rosario Prieto Bonilla, Daniel Bardan Rebollar, Francisco José López‐Jaime, Ihosvany Fernández‐Bello   +4 more
doaj   +2 more sources

A Post-Authorization Safety Surveillance Study to Report Clinical Experience with Purified Factor IX Concentrate in Pediatric Patients with Hemophilia B [PDF]

Journal of Blood Medicine
Zoran Igrutinović,1,2 Hélène Louise Hooimeijer,3 Karim Kentouche,4 Jaco Botha,5 Peter L Turecek,6 Marta Kokot-Kierepa,5 Hanna T Gazda7 1Clinic of Pediatrics, Department of Hemato-Oncology, University Clinical Center of Kragujevac, Kragujevac ...
Igrutinović Z, Hooimeijer HL, Kentouche K, Botha J, Turecek PL, Kokot-Kierepa M, Gazda HT   +6 more
doaj   +2 more sources

Peri-operative Management and the Role of Minimally Invasive Spine Surgery in a Case of Hemophilia B [PDF]

Journal of Minimally Invasive Spine Surgery and Technique, 2022
Hemophilia A and B are rare X-chromosome-linked recessive bleeding disorders caused by mutations in the genes causing abnormalities of blood clotting factors VIII and IX, respectively.
VNR Praveen Goparaju, Amit Chugh, Ameya Rangnekar, Vishal Kundnani   +3 more
doaj   +1 more source

Design of a Prospective Study on Pharmacokinetic-Guided Dosing of Prophylactic Factor Replacement in Hemophilia A and B (OPTI-CLOT TARGET Study)

TH Open, 2022
In resource-rich countries, almost all severe hemophilia patients receive prophylactic replacement therapy with factor concentrates to prevent spontaneous bleeding in joints and muscles to decrease the development of arthropathy and risk of long-term ...
Tine M.H.J. Goedhart, Laura H. Bukkems, Michiel Coppens, Karin J. Fijnvandraat, Saskia E.M. Schols, Roger E.G. Schutgens, Jeroen Eikenboom, Floor C.J.I. Heubel-Moenen, Paula F. Ypma, L. Nieuwenhuizen, K. Meijer, Frank W. G. Leebeek, Ron A.A. Mathôt, Marjon H. Cnossen   +13 more
doaj   +1 more source

The Liver That Cured Christmas: Case Report of Orthotopic Liver Transplant in a Patient with Hemophilia B

Case Reports in Transplantation, 2020
Herein, we discuss a case of a 39-year-old male with hemophilia B, who developed end-stage liver disease secondary to nonalcoholic steatohepatitis, that underwent orthotopic liver transplantation (OLT) as a curative means for his liver disease and ...
Jesse E. Harris, Jonathan L. Balk, Constance M. Mobley, Kirk Heyne   +3 more
doaj   +1 more source

A Case of Heel Reconstruction with a Reverse Sural Artery Flap in a Hemophilia B Patient

Archives of Plastic Surgery, 2012
Hemophilia B is a rare blood coagulation disorder. Complications such as bleeding and hematoma can cause necrosis of flaps, wound disruption, and the disturbance of wound healing.
Byung Kwon Lee, Jeong Su Shim
doaj   +1 more source

Enhanced Factor IX Activity following Administration of AAV5-R338L “Padua” Factor IX versus AAV5 WT Human Factor IX in NHPs

Molecular Therapy: Methods & Clinical Development, 2019
Gene therapy for severe hemophilia B is advancing and offers sustained disease amelioration with a single treatment. We have reported the efficacy and safety of AMT-060, an investigational gene therapy comprising an adeno-associated virus serotype 5 ...
Elisabeth A. Spronck, Ying Poi Liu, Jacek Lubelski, Erich Ehlert, Sander Gielen, Paula Montenegro-Miranda, Martin de Haan, Bart Nijmeijer, Valerie Ferreira, Harald Petry, Sander J. van Deventer   +10 more
doaj   +1 more source

Identification of the Profile of the Patients with Hemophilia B Eligible for Treatment with Nonacog Alfa Once-Weekly

Reports, 2020
This study aimed to identify the characteristics of patients with hemophilia B eligible for once-weekly treatment with Nonacog alfa. Methods: A survey was conducted in 14 Hemophilia (HCs) of Italy.
Dorina Cultrera, Raimondo De Cristofaro, Paola Giordano, Silvia Linari, Silvia Macchi, Renato Marino, Angelo Claudio Molinari, Angiola Rocino, Cristina Santoro, Piercarla Schinco, Sergio Siragusa, Giuseppe Tagariello, Annarita Tagliaferri, Ezio Zanon, Massimo Morfini   +14 more
doaj   +1 more source

Factor IX p.A37V mutation causes severe bleeding in a patient with phenprocoumon therapy

European Journal of Medical Research, 2021
Background Bleeding is the most common complication of oral anticoagulants, due to inadequate dosing. Case presentation This report describes the clinical course of a patient who developed severe bleeding under therapy with phenprocoumon, despite an INR ...
Nils Mülling, Vivian Rosery, H. Christian Reinhardt, Maher Hanoun   +3 more
doaj   +1 more source

Activation of Factor IX by Factor XIa - A Spectrophotometric Assay for Factor IX in Human Plasma [PDF]

Thrombosis and Haemostasis, 1982
SummaryThe activation of Factor IX by partially purified Factor XIa was followed by active site titration, gelelectrophoresis and by a spectrophotometric assay. The assay is based on the finding that the rate of Factor X activation in the presence of phospholipid and Ca2+ is linear in time and proportional to the amount of Factor IXa present and can be
Tans, G., Janssen-Claesssen, T., van Dieijen, G., Hemker, H.C., Rosing, J.   +4 more
openaire   +2 more sources