Results 131 to 140 of about 185,950 (370)

Non‐canonical PKG1 regulation in cardiovascular health and disease

British Journal of Pharmacology, EarlyView.
It is well established that the cyclic GMP‐dependent protein kinase I (PKG1) is canonically activated by cyclic guanosine monophosphate (cGMP), enabling its regulation of vascular tone, cardiac function and smooth muscle homeostasis. However, diverse non‐canonical stimuli of PKG1 have also been identified.
Jie Su, Joseph Robert Burgoyne
wiley   +1 more source

Human von Willebrand factor/factor VIII concentrates in the management of pediatric patients with von Willebrand disease/hemophilia A

Therapeutics and Clinical Risk Management, 2016
Giancarlo Castaman, Silvia Linari Department of Oncology, Center for Bleeding Disorders, Careggi University Hospital, Florence, ItalyAbstract: Several plasma-derived intermediate and high-purity concentrates containing von Willebrand factor (VWF) and ...
Castaman G, Linari S
doaj  

von Willebrand's disease characterized by increased ristocetin sensitivity and the presence of all von Willebrand factor multimers in plasma [PDF]

, 1986
Lars Holmberg, Erik Berntorp, Mikael Donnér, Nilsson Im   +3 more
openalex   +1 more source

Avascular Necrosis and Minimal Trauma Fractures in Telomere Biology Disorders

Clinical Genetics, EarlyView.
Avascular Necrosis (AVN) and Minimal Trauma Fractures (MTF) cause significant morbidity in people with Telomere Biology Disorders (TBDs). They occur more frequently in young patients and in those with autosomal recessive (AR) or X‐linked recessive (XLR) than in autosomal dominant (AD) genotypes.
Arman M. Niknafs, Neelam Giri, Marena R. Niewisch, Sharon A. Savage   +3 more
wiley   +1 more source

Identification and Characterization of Novel Variations in Platelet G-Protein Coupled Receptor (GPCR) Genes in Patients Historically Diagnosed with Type 1 von Willebrand Disease.

PLoS ONE, 2015
The clinical expression of type 1 von Willebrand disease may be modified by co-inheritance of other mild bleeding diatheses. We previously showed that mutations in the platelet P2Y12 ADP receptor gene (P2RY12) could contribute to the bleeding phenotype ...
Jacqueline Stockley, Shaista P Nisar, Vincenzo C Leo, Essa Sabi, Margaret R Cunningham, Jeroen C Eikenboom, Stefan Lethagen, Reinhard Schneppenheim, Anne C Goodeve, Steve P Watson, Stuart J Mundell, Martina E Daly, GAPP Study in Collaboration with the MCMDM-1VWD Study Group   +12 more
doaj   +1 more source

Factor VIII/von Willebrand factor binding to von Willebrand's disease platelets [PDF]

, 1982
HR Gralnick, Williams Sb, BC Shafer, Laurence Corash   +3 more
openalex   +1 more source

Interstitial 11q Deletions and Terminal 11q Duplications Cause a Bleeding Tendency due to Platelet Dysfunction That Is Similar to 11q Deletions Causing Jacobsen Syndrome

European Journal of Haematology, EarlyView.
ABSTRACT Introduction Jacobsen syndrome, resulting from a terminal deletion of chromosome 11 (11q), may lead to an increased bleeding tendency due to low platelet counts or platelet dysfunction. Currently, information on bleeding tendency and platelet function in patients with nonterminal 11q‐aberrations such as larger deletions, interstitial 11q ...
Elise J. Huisman, Virgil A. S. H. Dalm, Marieke Joosten, Frans J. Smiers, Leendert Porcelijn, Arjan Hoogendijk, Hans Janssen, Nicole N. van der van der Wel, Idske C. L. Kremer Hovinga, Masja de Haas, Marjon H. Cnossen   +10 more
wiley   +1 more source

In vivo interaction of von Willebrand factor with platelets following cryoprecipitate transfusion in platelet-type von Willebrand's disease [PDF]

, 1984
JL Miller, BD Boselli, JM Kupinski
openalex   +1 more source

Outcomes and Healthcare Resource Utilisation in Adults With von Willebrand Disease Receiving On‐Demand Recombinant von Willebrand Factor in the United Kingdom

European Journal of Haematology, EarlyView.
ABSTRACT Objectives We describe treatment outcomes and healthcare resource utilisation (HCRU) in adults with von Willebrand disease (VWD) treated on demand with recombinant von Willebrand factor (rVWF) in the United Kingdom. Methods Retrospective chart review of adults (≥ 18 years) with congenital VWD receiving first‐time rVWF for the on‐demand ...
Mike Laffan, Heena Howitt, Cheryl Jones, Sarah Brighton, Rosa Willock, Anna Sanigorska, Oliver Heard   +6 more
wiley   +1 more source

Further specificity characterization of von Willebrand factor inhibitors developed in two patients with severe von Willebrand disease [PDF]

, 1988
MF Lopez-Fernandez, R Martin, C Lopez-Berges, F Ramos, Norma B de Bosch, Javier Batlle   +5 more
openalex   +1 more source