Results 21 to 30 of about 115,636 (264)

Factor VIII/V C-domain swaps reveal discrete C-domain roles in factor VIII function and intracellular trafficking

Haematologica, 2017
Factor VIII C-domains are believed to have specific functions in cofactor activity and in interactions with von Willebrand factor. We have previously shown that factor VIII is co-targeted with von Willebrand factor to the Weibel-Palade bodies in blood ...
Eduard H.T.M. Ebberink, Eveline A.M. Bouwens, Esther Bloem, Mariëtte Boon-Spijker, Maartje van den Biggelaar, Jan Voorberg, Alexander B. Meijer, Koen Mertens   +7 more
doaj   +1 more source

Inhibitor development upon switching from plasma-derived to recombinant factor VIII in previously untreated patients with severe hemophilia A: the PUP-SWITCH study

Research and Practice in Thrombosis and Haemostasis
Background: The SIPPET randomized clinical trial showed that in previously untreated patients (PUPs) with severe hemophilia A, treatment with plasma-derived factor (F)VIII (pdFVIII) within the first 50 exposure days (EDs) was associated with a lower ...
Syna Miri, Frits R. Rosendaal, Kaan Kavakli, Peyman Eshghi, Soha Mohammadi Moghaddam, Sara Scardo, Behnaz Habibpanah, Mohsen Elalfy, Susan Halimeh, Gabriella Nicolò, Dilek Gökçebay, Namık Özbek, Tiraje Celkan, Ahmad Mohammadi, Mehran Karimi, Amin Shahsavani, Bariş Yılmaz, Canan Albayrak, Burcak Gunes, Zühre Kaya, Yilmaz Ay, Sinan Akbayram, Nazan Sarper, Pier Mannuccio Mannucci, Flora Peyvandi   +24 more
doaj   +1 more source

N-linked glycosylation modulates the immunogenicity of recombinant human factor VIII in hemophilia A mice

Haematologica, 2018
Immune responses to factor VIII remain the greatest complication in the treatment of severe hemophilia A. Recent epidemiological evidence has highlighted that recombinant factor VIII produced in baby hamster kidney cells is more immunogenic than factor ...
Jesse D. Lai, Laura L. Swystun, Dominique Cartier, Kate Nesbitt, Cunjie Zhang, Christine Hough, James W. Dennis, David Lillicrap   +7 more
doaj   +1 more source

The 1.7 Å X-ray crystal structure of the porcine factor VIII C2 domain and binding analysis to anti-human C2 domain antibodies and phospholipid surfaces. [PDF]

PLoS ONE, 2015
The factor VIII C2 domain is essential for binding to activated platelet surfaces as well as the cofactor activity of factor VIII in blood coagulation. Inhibitory antibodies against the C2 domain commonly develop following factor VIII replacement therapy
Caileen M Brison, Steven M Mullen, Michelle E Wuerth, Kira Podolsky, Matthew Cook, Jacob A Herman, Justin D Walter, Shannon L Meeks, P Clint Spiegel   +8 more
doaj   +1 more source

Desmopressin in moderate hemophilia A patients: a treatment worth considering

Haematologica, 2018
Desmopressin increases endogenous factor VIII levels in hemophilia A. Large inter-individual variation in the response to desmopressin is observed.
Janneke I. Loomans, Marieke J.H.A. Kruip, Manuel Carcao, Shannon Jackson, Alice S. van Velzen, Marjolein Peters, Elena Santagostino, Helen Platokouki, Erik Beckers, Jan Voorberg, Johanna G. van der Bom, Karin Fijnvandraat, for the RISE consortium   +12 more
doaj   +1 more source

Rational Use of Herbal Products in Pediatric Patients Treated With Anticancer Drugs in the European Union

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Introduction The use of herbal medical preparation (HMP) is rising among pediatric oncology patients, often to manage treatment‐related symptoms. Their effectiveness remains uncertain, and the risk of herb–drug interactions is underestimated.
Orianne Mahot, Mathieu Corvaisier, Laurence Spiesser‐Robelet, Mylène Duplan, Isabelle Pellier, Delphine Bourneau‐Martin, Séverine Derbré   +6 more
wiley   +1 more source

Ironic Medications: A Narrative‐Based Psycho‐Educational Intervention to Explore Patient–Provider Communication in Adolescents With Haematological Cancer

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Background Adolescents with haematological malignancies face significant emotional and relational challenges, often accompanied by difficulties in communicating their needs within the healthcare context. To address these issues, a narrative‐based psycho‐educational intervention based on the creation and prescription of Ironic Medications was ...
Marta Stoppa, Elena Pagani Bagliacca, Selena Russo, Giulia Rampoldi, Maria Grazia Strepparava, Adriana Cristina Balduzzi, Andrea Biondi, Marco Spinelli   +7 more
wiley   +1 more source

Factor VIII activity and bleeding risk during prophylaxis for severe hemophilia A: a population pharmacokinetic model

Haematologica, 2020
During factor VIII prophylaxis for severe hemophilia A, bleeding risk increases with time when factor VIII activity is below 1%. Maintaining trough activity above 1% does not protect all patients from bleeding.
Andreas Tiede, Faraizah Abdul Karim, Victor Jiménez-Yuste, Robert Klamroth, Sandra Lejniece, Takashi Suzuki, Andreas Groth, Elena Santagostino   +7 more
doaj   +1 more source

Incidence and Severity of Carboplatin‐Associated Hearing Loss in Children With Cancer Assessed by the SIOP Boston 2012 Ototoxicity Criteria

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Background Platinum‐based chemotherapy is known to cause severe and debilitating hearing loss, but unlike cisplatin, the true incidence of carboplatin‐induced hearing loss remains unclear. We evaluated functional hearing outcomes in children receiving carboplatin to determine the incidence and severity of ototoxicity. Procedure We identified a
Aniket Chawla, Sean Carter, Andrew Wood, Sandra Staffieri, Andrew Dodgshun, David D. Eisenstat, Michael Sullivan   +6 more
wiley   +1 more source

Re‐Irradiation in Pediatric Diffuse Midline Glioma: A Multi‐Institutional Retrospective Study

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Background Children with recurrent diffuse midline gliomas (DMGs) have limited therapeutic options at recurrence. Re‐irradiation (RT2) may be used at progression, but with uncertainty about the benefit. Methods We conducted a multi‐institutional retrospective study of children aged < 18 with DMG treated at three centers (Toronto, Canada ...
Ajay Thomas Alex, Sean P. Hassan, Helen Benghiat, Liam Herbert, Jennifer Adamski, Shivani Bailey, Claire Esler, Louise Brookes, Molly Bamber, Grace Koudou, Karen Waite, Julie Bennett, Anirban Das, Derek S. Tsang   +13 more
wiley   +1 more source