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Platelet disorders

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https://www.readbyqxmd.com/read/28550758/platelets-and-hemophilia-a-review-of-the-literature
#1
REVIEW
Julia Riedl, Cihan Ay, Ingrid Pabinger
Hemophilia A and B are inherited bleeding disorders due to deficiencies of the clotting factors VIII and IX, respectively. The severity of the disease correlates with remaining factor levels, although individual differences in bleeding tendency are seen despite similar factor levels. While thrombin generation is severely impaired in persons with hemophilia, primary hemostasis, i.e. platelet function, has been generally considered to be normal. However, some studies reported prolonged bleeding times in hemophilia, suggesting that also primary hemostasis is affected...
May 13, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28541681/platelet-microcapsule-hybrids-leverage-contractile-force-for-targeted-delivery-of-hemostatic-agents
#2
Caroline E Hansen, David R Myers, Wallace Hunter Baldwin, Yumiko Sakurai, Shannon L Meeks, L Andrew Lyon, Wilbur A Lam
We report a cell-mediated, targeted drug delivery system utilizing polyelectrolyte multilayer capsules that hybridize with the patient's own platelets upon intravenous administration. The hybridized platelets function as the sensor and actuator for targeted drug delivery and controlled release in our system. These capsules are biochemically and mechanically tuned to enable platelet adhesion and capsule rupture upon platelet activation and contraction, enabling the targeted and controlled "burst" release of an encapsulated biotherapeutic...
May 25, 2017: ACS Nano
https://www.readbyqxmd.com/read/28540852/association-of-polyunsaturated-saturated-fatty-acids-to-metabolic-syndrome-cardiovascular-risk-factors-and-lipoprotein-a-in-hypertensive-type-2-diabetic-patients
#3
Amira Fatma Bennacer, Elmahdi Haffaf, Ghouti Kacimi, Brahim Oudjit, Elhadj-Ahmed Koceir
The interactions between fatty acids (FA) classes: polyunsaturated (PUFA-ω6, PUFA-ω3), saturated (SFA), EPA (eicosapentaenoic acid-ω3), DHA (docosahexaenoic-ω3) and cardiometabolic syndrome (CMS) clusters, thrombosis development and vascular inflammation are subtle. This relationship is mediated by insulin resistance (IR), endothelial dysfunction, platelet aggregation disorder and atherosclerosis. OBJECTIVES: We investigated whether PUFA/SFA - PUFA-ω6/PUFA-ω3 ratios and EPA + DHA can be associated with predictive atherothrombogenic biomarkers status in type 2 diabetes (T2D) patients with or without hypertension (HT)...
June 1, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/28540073/viscoelastic-testing-inside-and-beyond-the-operating-room
#4
REVIEW
Liang Shen, Sheida Tabaie, Natalia Ivascu
Hemorrhage is a major contributor to morbidity and mortality during the perioperative period. Current methods of diagnosing coagulopathy have various limitations including long laboratory runtimes, lack of information on specific abnormalities of the coagulation cascade, lack of in vivo applicability, and lack of ability to guide the transfusion of blood products. Viscoelastic testing offers a promising solution to many of these problems. The two most-studied systems, thromboelastography (TEG) and rotational thromboelastometry (ROTEM), offer similar graphical and numerical representations of the initiation, formation, and lysis of clot...
April 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28534116/myeloid-neoplasms-with-germ-line-runx1-mutation
#5
REVIEW
Yoshihiro Hayashi, Yuka Harada, Gang Huang, Hironori Harada
Familial platelet disorder with propensity to myeloid malignancies (FPD/AML) is an autosomal dominant disorder characterized by quantitative and/or qualitative platelet defects with a tendency to develop a variety of hematological malignancies. Heterozygous germ line mutations in the RUNX1 gene are responsible genetic events for FPD/AML. Notably, about half of individuals in the family with germ line mutations in RUNX1 develop overt hematological malignancies. The latency is also relatively long as an average age at diagnosis is more than 30 years...
May 22, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28533135/enhanced-local-disorder-in-a-clinically-elusive-von-willebrand-factor-provokes-high-affinity-platelet-clumping
#6
Alexander Tischer, Venkata R Machha, Juan P Frontroth, Maria A Brehm, Tobias Obser, Reinhard Schneppenheim, Leland Mayne, S Walter Englander, Matthew Auton
Mutation of the cysteines forming the disulfide loop of the platelet GPIbα adhesive A1 domain of von Willebrand factor causes quantitative VWF deficiencies in the blood and von Willebrand disease. We report two cases of transient severe thrombocytopenia induced by DDAVP-treatment. Cys1272Trp and Cys1458Tyr mutations identified by genetic sequencing implicate an abnormal gain-of-function phenotype, evidenced by thrombocytopenia, that quickly relapses back to normal platelet counts and deficient plasma VWF. Using surface plasmon resonance, analytical rheology, and hydrogen-deuterium exchange mass spectrometry (HXMS), we decipher mechanisms of A1-GPIbα mediated platelet adhesion and resolve dynamic secondary structure elements that regulate the binding pathway...
May 19, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/28532218/flow-chamber-and-microfluidic-approaches-for-measuring-thrombus-formation-in-genetic-bleeding-disorders
#7
Rogier M Schoeman, Marcus Lehmann, Keith B Neeves
Platelet adhesion and aggregation, coagulation, fibrin formation, and fibrinolysis are regulated by the forces and flows imposed by blood at the site of a vascular injury. Flow chambers designed to observe these events are an indispensable part of doing hemostasis and thrombosis research, especially with human blood. Microfluidic methods have provided the flexibility to design flow chambers with complex geometries and features that more closely mimic the anatomy and physiology of blood vessels. Additionally, microfluidic systems with integrated optics and/or pressure sensors and on-board signal processing could transform what have been primarily research tools into clinical assays...
May 22, 2017: Platelets
https://www.readbyqxmd.com/read/28532133/neutrophil-lymphocyte-ratio-in-different-stages-of-breast-cancer
#8
Fezzeh Elyasinia, Mohammad Reza Keramati, Farham Ahmadi, Susan Rezaei, Mohammad Ashouri, Reza Parsaei, Maryam Yaghoubi, Fahimeh Elyasinia, Armita Aboutorabi, Ahmad Kaviani
Despite many advances in the treatment of breast cancer, it is still the second most common cause of death in women in the United States. It has been shown that inflammation plays a major role in the treatment of these cancers and inflammatory factors enhance tumor growth, invasion, metastasis, and vascularization. In this study, we would like to analyze peripheral blood neutrophil-lymphocyte ratio (NLR) in breast cancer patients and its correlation with disease staging. This cross-sectional analytic study was conducted in Imam Hospital, affiliated with Tehran University of Medical Sciences; a total of 195 female patients with breast cancer met the inclusion criteria...
April 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/28530237/anti-platelet-factor-4-polyanion-antibodies-mediate-a-new-mechanism-of-autoimmunity
#9
Thi-Huong Nguyen, Nikolay Medvedev, Mihaela Delcea, Andreas Greinacher
Antibodies recognizing complexes of the chemokine platelet factor 4 (PF4/CXCL4) and polyanions (P) opsonize PF4-coated bacteria hereby mediating bacterial host defense. A subset of these antibodies may activate platelets after binding to PF4/heparin complexes, causing the prothrombotic adverse drug reaction heparin-induced thrombocytopenia (HIT). In autoimmune-HIT, anti-PF4/P-antibodies activate platelets in the absence of heparin. Here we show that antibodies with binding forces of approximately 60-100 pN activate platelets in the presence of polyanions, while a subset of antibodies from autoimmune-HIT patients with binding forces ≥100 pN binds to PF4 alone in the absence of polyanions...
May 22, 2017: Nature Communications
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#10
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28527173/gaucher-disease-presenting-in-an-adult-with-intracerebral-bleed
#11
Sandeep Nemani, Bhumi Agrawal, Sumita Danda, Biju George
Gaucher disease (GD) is the most common lysosomal storage disorder, caused by deficiency of acid beta glucosidase. GD usually presents in children but occasional cases can present in adulthood. Here we report a case of type I GD in a 37 year old female who presented with intracerebral bleed due to long standing thrombocytopenia. She underwent splenectomy in view of limited resources for enzyme replacement therapy. With splenectomy her platelet counts normalised and neurological status also improved.
April 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28523956/pathogen-reduction-technologies-the-pros-and-cons-for-platelet-transfusion
#12
Audrey Magron, Jonathan Laugier, Patrick Provost, Eric Boilard
The transfusion of platelets is essential for diverse pathological conditions associated with thrombocytopenia or platelet disorders. To maintain optimal platelet quality and functions, platelets are stored as platelet concentrates (PCs) at room temperature under continuous agitation-conditions that are permissive for microbial proliferation. In order to reduce these contaminants, pathogen reduction technologies (PRTs) were developed by the pharmaceutical industry and subsequently implemented by blood banks...
May 19, 2017: Platelets
https://www.readbyqxmd.com/read/28522826/disruption-of-melatonin-synthesis-is-associated-with-impaired-14-3-3-and-mir-451-levels-in-patients-with-autism-spectrum-disorders
#13
Cécile Pagan, Hany Goubran-Botros, Richard Delorme, Marion Benabou, Nathalie Lemière, Kerren Murray, Frédérique Amsellem, Jacques Callebert, Pauline Chaste, Stéphane Jamain, Fabien Fauchereau, Guillaume Huguet, Erik Maronde, Marion Leboyer, Jean-Marie Launay, Thomas Bourgeron
Autism spectrum disorders (ASD) are characterized by a wide genetic and clinical heterogeneity. However, some biochemical impairments, including decreased melatonin (crucial for circadian regulation) and elevated platelet N-acetylserotonin (the precursor of melatonin) have been reported as very frequent features in individuals with ASD. To address the mechanisms of these dysfunctions, we investigated melatonin synthesis in post-mortem pineal glands - the main source of melatonin (9 patients and 22 controls) - and gut samples - the main source of serotonin (11 patients and 13 controls), and in blood platelets from 239 individuals with ASD, their first-degree relatives and 278 controls...
May 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28515471/clinical-and-molecular-investigation-of-14-japanese-patients-with-complete-tfp-deficiency-a-comparison-with-caucasian-cases
#14
Ryosuke Bo, Kenji Yamada, Hironori Kobayashi, Purevsuren Jamiyan, Yuki Hasegawa, Takeshi Taketani, Seiji Fukuda, Ikue Hata, Yo Niida, Yosuke Shigematsu, Kazumoto Iijima, Seiji Yamaguchi
Mitochondrial trifunctional protein (TFP) deficiency is an inherited metabolic disorder of mitochondrial fatty-acid oxidation. Isolated long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is often reported in Caucasian countries due to a common mutation. However, the molecular and clinical basis of complete TFP deficiency has not been extensively reported. In this study, 14 Japanese cases (13 families) with complete TFP deficiency, including 9 previously reported cases, were analyzed to clarify the clinical and molecular characteristics of TFP deficiency...
May 18, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28514344/-erbium-laser-application-for-oral-surgery-in-patients-with-platelet-hemostatic-disorders
#15
E V Makarova, S V Tarasenko, A L Melikyan, A V Ponomarenko
Hemostatic disorders are typically associated with prolonged bleeding after or during surgical procedures. The aim of the study was to increase the efficiency of oral surgery in these patients using erbium laser. Selected 46 patients receiving oral surgery were randomly divided in 2 groups: 43 patients with thrombocytopenia, trombocytemia and other platelet disorders treated with erbium laser and a control group of 43 patients without concomitant pathology determined for conventional surgical treatment. No postoperative bleeding was seen in group 1...
2017: Stomatologii︠a︡
https://www.readbyqxmd.com/read/28513614/recurrent-somatic-jak-stat-pathway-variants-within-a-runx1-mutated-pedigree
#16
Kiran Tawana, Jun Wang, Péter A Király, Krisztián Kállay, Gábor Benyó, Marianna Zombori, Judit Csomor, Ahad Al Seraihi, Ana Rio-Machin, András Matolcsy, Claude Chelala, Jamie Cavenagh, Jude Fitzgibbon, Csaba Bödör
Germline variants within the transcription factor RUNX1 are associated with familial platelet disorder and acute leukemia in over 40% of carriers. At present, the somatic events triggering leukemic transformation appear heterogeneous and profiles of leukemia initiation across family members are poorly defined. We report a new RUNX1 family where three sisters harboring a germline nonsense RUNX1 variant, c.601C>T (p.(Arg201*)), developed acute myelomonocytic leukemia (AML) at 5 years of age. Whole-exome sequencing of tumor samples revealed all three siblings independently acquired variants within the JAK-STAT pathway, specifically targeting JAK2 and SH2B3 (a negative regulator of JAK2), while also sharing the 46/1 haplotype linked with sporadic JAK2-positive myeloproliferative neoplasms...
May 17, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28506946/maternal-mortality-due-to-hypertensive-disorders-in-pregnancy-childbirth-and-the-puerperium-between-2012-and-2015-in-turkey-a-nation-based-study
#17
Bekir Keskinkılıç, Yaprak Engin-Üstün, Sema Sanisoğlu, Dilek Şahin Uygur, Hüseyin Levent Keskin, Selma Karaahmetoğlu, Ayşe Özcan, Meral Esen, Afra Alkan, Aysun Kabasakal, İrfan Şencan
OBJECTIVE: To analyze maternal deaths in Turkey due to hypertensive disorders. MATERIAL AND METHODS: In this retrospective study 812 maternal deaths were analyzed. Maternal demographic features, presence of antenatal care, medical and obstetric history, mode of delivery, and use emergency antihypertensive therapy were recorded. The delay model for each case was investigated. RESULTS: Hypertensive disorders accounted for 15.5% (n=126) out of all maternal mortality...
March 15, 2017: Journal of the Turkish German Gynecological Association
https://www.readbyqxmd.com/read/28504079/a-novel-nonsense-nbeal2-gene-mutation-causing-severe-bleeding-in-a-patient-with-gray-platelet-syndrome
#18
Lijuan Cao, Jian Su, Jiaming Li, Ziqiang Yu, Xia Bai, Zhaoyue Wang, Lijun Xia, Changgeng Ruan
Gray platelet syndrome (GPS) is a rare, inherited bleeding disorder characterized by the defect of platelet α-granule. Up to date, these are only four studies identifying NBEAL2 gene correlated with GPS. In the current report, we present a Chinese GPS patient who had severe bleeding tendency, abnormalities of platelet functions, and absence of platelet α-granules. Genomic DNA sequencing for the patient identified a nonsense mutation (g.27713C>A) of NBEAL2 gene (g.NG_031914.1) resulting in a premature protein (p...
May 15, 2017: Platelets
https://www.readbyqxmd.com/read/28501326/management-of-platelet-disorders-and-platelet-transfusions-in-icu-patients
#19
REVIEW
Neil Blumberg, Jill M Cholette, Amy E Schmidt, Richard P Phipps, Sherry L Spinelli, Joanna M Heal, Anthony P Pietropaoli, Majed A Refaai, Patricia J Sime
Thrombocytopenia or receipt of antiplatelet drugs, with or without bleeding, is a common indication for platelet transfusions in the ICU. However, there is almost no evidence base for these practices other than expert opinion. Also common is use of platelet transfusions prior to invasive procedures or surgery in patients with thrombocytopenia. Likewise, there is no high-quality evidence that such practices are efficacious or safe. Recently, it has become clear that, whether causal or not, patients receiving prophylactic platelet transfusions experience high rates of nosocomial infection, thrombosis, organ failure, and mortality, which increase the urgency and need for randomized trials to assess these practices...
May 4, 2017: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/28500685/analysis-of-serum-markers-of-cellular-immune-activation-in-patients-with-bullous-pemphigoid
#20
Katja Bieber, Anna Lara Ernst, Stefan Tukaj, Maike M Holtsche, Enno Schmidt, Detlef Zillikens, Ralf J Ludwig, Michael Kasperkiewicz
Experimental models of bullous pemphigoid (BP), the most frequent subepidermal autoimmune bullous disease, revealed that the immune response leading to blister formation represents an incompletely understood complex process involving different inflammatory cells. In contrast to previous reports commonly focusing on limited molecular and cellular phenotypes of the disease, the aim of this study was to investigate a broad spectrum of markers of cellular immune activation in patients with BP. We found that serum levels of soluble CD4, myeloperoxidase, S100A12, eosinophil cationic protein, and soluble P-selectin were significantly elevated in patients with active BP compared with normal controls...
May 13, 2017: Experimental Dermatology
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