Results 131 to 140 of about 55,967 (276)

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

Polycythemia Vera and Essential Thrombocythemia: A Nationwide Population‐Based Study on Treatment Patterns, Vascular Complications and Survival

European Journal of Haematology, EarlyView.
ABSTRACT Background Polycythemia vera (PV) and essential thrombocythemia (ET) are associated with a well‐recognized increased risk of thrombotic events, bleeding, and all‐cause mortality, but the frequency of these outcomes during treatment has rarely been assessed in large cohorts.
Anneli Enblom Larsson, Henrik Renlund, Björn Andréasson, Henrik Holmberg, Maria Liljeholm, Anders Själander   +5 more
wiley   +1 more source

Clinical Features And Laboratory Patterns In A Cohort Of Consecutive Argentinian Patients With Von Willebrand's Disease [PDF]

, 2001
Background and Objectives. von Willebrand's disease (vWD) is a bleeding disorder with variable clinical expression. Our aim was to classify patients with vWD and to determine the phenotype in their relatives. Design and Methods.
Blanco, A. N., Farias, Cristina Elena, Kempfer, Ana Catalina, Lazzari, María Ángela, Meschengieser, S. S., Salviu, M. J., Woods, Adriana Inés   +6 more
core  

Efficacy and Safety of Prophylaxis With a Plasma‐Derived von Willebrand Factor/Factor VIII Concentrate (Wilate) in Patients With Type 3 von Willebrand Disease—A WIL‐31 Study Sub‐Analysis

European Journal of Haematology, EarlyView.
ABSTRACT Objectives The WIL‐31 study demonstrated efficacy and safety of prophylaxis with the plasma‐derived von Willebrand factor/factor VIII concentrate wilate in von Willebrand disease (VWD) of all types and was the only prospective study with an on‐demand run‐in study as an intra‐individual comparator.
Claudia Djambas Khayat, Leonid Dubey, Adlette Inati, Toshko Lissitchkov, Dzmitry Novik, Elina Peteva, Robert F. Sidonio Jr, Ali T. Taher, Kateryna V. Vilchevska, Vladimir Vdovin, Ana Boban   +10 more
wiley   +1 more source

Motives and predictors of modified Atkins diet discontinuation as treatment of adults with drug‐resistant epilepsy

Epilepsia, EarlyView.
Abstract Objective This study was undertaken to explore the motives and predictors of modified Atkins diet (MAD) discontinuation as a treatment of adults with drug‐resistant epilepsy (DRE). Methods A single‐center observational longitudinal study was made of a cohort of patients treated with MAD during the first 6 years at a multidisciplinary ...
Raquel Samões, Ana Cavalheiro, Maria Manuel Tavares, Catarina Teixeira, Bárbara Leal, João Chaves, Sara Cavaco   +6 more
wiley   +1 more source

Evaluation of the Clinical Status of Patients with Inherited Bleeding Disorders in Diyala-Iraq

Diyala Journal of Medicine, 2019
Background: Inherited bleeding disorders are rare diseases that are both complicated and expensive to manage, they are classified into: coagulation factor disorders, vessel wall defect and platelet disorders.
Imad Ahmed Lateef
doaj   +2 more sources

Long‐term outcomes of stereotactic radiofrequency ablation in hypothalamic hamartomas: A single‐center experience

Epilepsia, EarlyView.
Abstract Objective Hypothalamic hamartomas (HHs) lead to refractory epilepsy, and minimally invasive surgical approaches are standard of care for affected patients. Stereotactic radiofrequency thermocoagulation (SRT) is one of the treatment methods recognized to achieve seizure freedom. This study reports surgical outcome from a single center reporting
Peter Christoph Reinacher, Julia Jacobs, Mukesch Johannes Shah, Theo Demerath, Kathrin Wagner, Victoria San Antonio‐Arce, Horst Urbach, Volker Arnd Coenen, Andreas Schulze‐Bonhage, Kerstin Alexandra Klotz   +9 more
wiley   +1 more source

EHD4 and ASAP2 are critical negative regulators of the claudin‐5‐based endothelial barrier

The FEBS Journal, EarlyView.
Cell‐surface CLDN‐5 protein levels can be evaluated using a probe that can bind to the extracellular domains of CLDN‐5. A probe derived from Clostridium perfringens enterotoxin allows us to isolate cells with high CLDN‐5 protein levels from a knockout cell library.
Yosuke Hashimoto, Gergő Porkoláb, Natalie Hudson, Jeffrey O'Callaghan, Nicole Hanley, Conor Delaney, Mária A. Deli, Peter Westenskow, Matthew Campbell   +8 more
wiley   +1 more source

An update on the landscape of collagen bioactive fragments

The FEBS Journal, EarlyView.
The remodeling of the extracellular matrix releases collagen bioactive fragments, which exert molecular functions and regulate numerous biological processes via several signaling pathways. Here, we summarize the latest findings describing the roles of major bioactive fragments from collagens I, IV, VI, and XVIII in various physiological and ...
Sylvie Ricard‐Blum, Julie Fradette
wiley   +1 more source

Contrasting Approaches in the Implementation of GRADE Methodology in Guidelines for Haemophilia and Von Willebrand Disease

Haemophilia, EarlyView.
ABSTRACT Introduction The 2024 ISTH clinical practice guideline (CPG) for treatment of congenital haemophilia, the NBDF‐McMaster Guideline on Care Models for Haemophilia Management, and ASH ISTH NBDF WFH guidelines on the diagnosis and management of VWD all utilised GRADE methodology.
Mark W. Skinner, Manuela Albisetti, Jesús Ardila, Jan Astermark, Jan Blatny, Manuel Carcao, Pratima Chowdary, Nathan T. Connell, Miguel Crato, Yesim Dargaud, Roseline d'Oiron, Amy L. Dunn, Miguel A. Escobar, Carmen Escuriola‐Ettingshausen, Emna Gouider, Annie Harroche, Cedric Hermans, Victor Jimenez‐Yuste, Radoslaw Kaczmarek, Gili Kenet, Liane Khoo, Robert Klamroth, Florian Langer, David Lillicrap, Johnny Mahlangu, Christoph Male, Tadashi Matsushita, Sandrine Meunier, Wolfgang Miesbach, Beatrice Nolan, Johannes Oldenburg, Brian O'Mahony, Margareth Ozelo, Glenn F. Pierce, Gloria Ramos, Michael Recht, Olivia Romero‐Lux, Dawn Rotellini, Rita C. Santoro, Tammuella C. Singleton, Alok Srivastava, Sophie Susen, Kate Talks, Huyen Tran, Leonard A. Valentino, Jerzy Windyga, Renchi Yang, Maria Elisa Mancuso, also on behalf of AICE, AHAD‐AP, AHCDC, ATHN, CLAHT, EHC, French CRH & MHEMO, HTCCNC, NBDF, PEDNET, UKHCDO, WFH   +59 more
wiley   +1 more source