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Platelet disorder

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https://www.readbyqxmd.com/read/28811646/pafah1b1-haploinsufficiency-disrupts-gaba-neurons-and-synaptic-e-i-balance-in-the-dentate-gyrus
#1
Matthew T Dinday, Kelly M Girskis, Sunyoung Lee, Scott C Baraban, Robert F Hunt
Hemizygous mutations in the human gene encoding platelet-activating factor acetylhydrolase IB subunit alpha (Pafah1b1), also called Lissencephaly-1, can cause classical lissencephaly, a severe malformation of cortical development. Children with this disorder suffer from deficits in neuronal migration, severe intellectual disability, intractable epilepsy and early death. While many of these features can be reproduced in Pafah1b1(+/-) mice, the impact of Pafah1b1(+/-) on the function of individual subpopulations of neurons and ultimately brain circuits is largely unknown...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28808266/in-silico-analysis-of-glanzmann-variants-of-calf-1-domain-of-%C3%AE-iib%C3%AE-3-integrin-revealed-dynamic-allosteric-effect
#2
Matthieu Goguet, Tarun Jairaj Narwani, Rachel Petermann, Vincent Jallu, Alexandre G de Brevern
Integrin αIIbβ3 mediates platelet aggregation and thrombus formation. In a rare hereditary bleeding disorder, Glanzmann thrombasthenia (GT), αIIbβ3 expression / function are impaired. The impact of deleterious missense mutations on the complex structure remains unclear. Long independent molecular dynamics (MD) simulations were performed for 7 GT variants and reference structure of the Calf-1 domain of αIIb. Simulations were analysed using a structural alphabet to describe local protein conformations. Common and flexible regions as well as deformable zones were observed in all the structures...
August 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28804921/serum-thymidine-kinase-1-canine-c-reactive-protein-haptoglobin-and-vitamin-d-concentrations-in-dogs-with-immune-mediated-hemolytic-anemia-thrombocytopenia-and-polyarthropathy
#3
M Grobman, H Outi, H Rindt, C Reinero
BACKGROUND: Relapses of immune-mediated hemolytic anemia (IMHA), thrombocytopenia (ITP), or polyarthropathy (IMPA) occur despite normal hematologic and cytologic parameters. Thymidine kinase 1 (TK1), canine C-reactive protein (c-CRP), haptoglobin (HPT), and 25-Hydroxyvitamin-D (25(OH)D) might be adjunct to current monitoring strategies. HYPOTHESIS/OBJECTIVES: Compare serum concentrations of TK1, c-CRP, HPT, and 25(OH)D in dogs with well- and poorly controlled primary IMHA, ITP, or IMPA...
August 14, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28804846/laboratory-testing-for-von-willebrand-factor-ristocetin-cofactor-vwf-rco
#4
Soma Mohammed, Emmanuel J Favaloro
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for these VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes several protocols for assessment of VWF activity by means of VWF ristocetin cofactor (VWF:RCo)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804843/diagnosis-or-exclusion-of-von-willebrand-disease-using-laboratory-testing
#5
Emmanuel J Favaloro
von Willebrand disease (VWD) is a common bleeding disorder diagnosed based on clinical features and following laboratory testing. VWD is due to deficiencies or defects in the plasma protein von Willebrand factor (VWF), a large adhesive protein with multiple activities. Laboratory testing therefore centers on assessment of VWF protein level using VWF antigen (VWF:Ag), as well as assays that measure VWF activity, most notably platelet glycoprotein (GP) Ib and collagen binding (VWF:CB) activities. Decreases in VWF:Ag and VWF activities, as well as the pattern of such changes, help define VWD and its type...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804842/flow-cytometry-protocols-for-assessment-of-platelet-function-in-whole-blood
#6
Leonardo Pasalic, Gabrielle J Pennings, David Connor, Heather Campbell, Leonard Kritharides, Vivien M Chen
Flow cytometry is a powerful tool for rapid evaluation of multiple functional properties of large numbers of platelets in whole blood. In the following chapter, we provide a number of flow cytometry-based protocols broadly aimed at (1) assessment of constitutively expressed platelet membrane receptors to diagnose inherited platelet bleeding disorders and (2) investigation of basal and agonist-induced platelet functional responses including generation of platelet-leukocyte aggregates, alpha and dense granule release, calcium flux, and phosphatidylserine exposure...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804841/assessment-of-platelet-function-in-whole-blood-by-flow-cytometry
#7
Leonardo Pasalic
Evaluation of platelet function is important for understanding the physiology of hemostasis and thrombosis and is utilized in clinical practice to diagnose inherited and acquired platelet bleeding disorders. Flow cytometry is a powerful tool for rapid evaluation of multiple functional properties of large number of platelets in whole blood and offers many advantages over other traditional methods. Attention to pre-analytical factors is required to ensure biologically valid and robust results.
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804838/platelet-function-tests-preanalytical-variables-clinical-utility-advantages-and-disadvantages
#8
Anne-Mette Hvas, Erik Lerkevang Grove
Platelet function tests are mainly used in the diagnostic work-up of platelet disorders. During the last decade, the additional use of platelet function tests to evaluate the effect of antiplatelet therapy has also emerged in an attempt to identify patients with an increased risk of arterial thrombosis. Furthermore, platelet function tests are increasingly used to measure residual effect of antiplatelet therapy prior to surgery with the aim of reducing the risk of bleeding. To a limited extend, platelet function tests are also used to evaluate hyperaggregability as a potential marker of a prothrombotic state outside the setting of antiplatelet therapy...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28802751/acute-management-of-hemostasis-in-patients-with-neurological-injury
#9
REVIEW
M Irem Baharoglu, Anneke Brand, Maria M Koopman, Marinus Vermeulen, Yvo B W E M Roos
Neurological injuries can be divided into those with traumatic and nontraumatic causes. The largest groups are traumatic brain injury (TBI) and nontraumatic stroke. TBI patients may present with intracranial hemorrhages (contusions, or subdural or epidural hematomas). Strokes are ischemic or hemorrhagic. In all these disorders, thrombosis and hemostasis play a major role. Treatment aims to either cease bleeding and/or restore perfusion. We reviewed hemostatic and thrombolytic therapies in patients with neurological injuries by MEDLINE and EMBASE search using various key words for neurological disorders and hemostatic therapies restricted to English language and human adults...
July 13, 2017: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/28802375/post-tonsillectomy-hemorrhagic-outcomes-in-children-with-bleeding-disorders-at-a-single-institution
#10
Priyesh N Patel, Alexandra M Arambula, Allison P Wheeler, Edward B Penn
OBJECTIVE: To report on the post-tonsillectomy bleeding outcomes and factors associated with hemorrhage among children with pre- or post-operatively diagnosed bleeding disorders treated with an institutional protocol. METHODS: Retrospective cohort study of patients with hematologic disorders who underwent tonsillectomy between 2003 and 2016 and were treated with perioperative desmopressin or factor replacement and/or aminocaproic acid. Postoperative outcomes were compared to controls matched for age, sex, and indication for surgery...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28796901/from-peroxisomal-disorders-to-common-neurodegenerative-diseases-the-role-of-ether-phospholipids-in-the-nervous-system
#11
REVIEW
Fabian Dorninger, Sonja Forss-Petter, Johannes Berger
The emerging diverse roles of ether (phospho)lipids in nervous system development and function in health and disease are currently attracting growing interest. Plasmalogens, a subgroup of ether lipids, are important membrane components involved in vesicle fusion and membrane raft composition. They store polyunsaturated fatty acids and may serve as antioxidants. Ether lipid metabolites act as precursors for the formation of glycosyl-phosphatidyl-inositol (GPI) anchors; others, like platelet-activating factor, are implicated in signaling functions...
August 10, 2017: FEBS Letters
https://www.readbyqxmd.com/read/28791287/bidirectional-mast-cell-eosinophil-interactions-in-inflammatory-disorders-and-cancer
#12
REVIEW
Maria Rosaria Galdiero, Gilda Varricchi, Mansour Seaf, Giancarlo Marone, Francesca Levi-Schaffer, Gianni Marone
Human mast cells (MCs) and eosinophils were first described and named by Paul Ehrlich. These cells have distinct myeloid progenitors and differ morphologically, ultrastructurally, immunologically, biochemically, and pharmacologically. However, MCs and eosinophils play a pivotal role in several allergic disorders. In addition, these cells are involved in autoimmune disorders, cardiovascular diseases, and cancer. MCs are distributed throughout all normal human tissues, whereas eosinophils are present only in gastrointestinal tract, secondary lymphoid tissues, and adipose tissue, thymus, mammary gland, and uterus...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28783682/myosin-light-chains-9-and-12-are-functional-ligands-for-cd69-that-regulate-airway-inflammation
#13
Koji Hayashizaki, Motoko Y Kimura, Koji Tokoyoda, Hiroyuki Hosokawa, Kenta Shinoda, Kiyoshi Hirahara, Tomomi Ichikawa, Atsushi Onodera, Asami Hanazawa, Chiaki Iwamura, Jungo Kakuta, Kenzo Muramoto, Shinichiro Motohashi, Damon J Tumes, Tomohisa Iinuma, Heizaburo Yamamoto, Yuzuru Ikehara, Yoshitaka Okamoto, Toshinori Nakayama
Recent decades have witnessed a rapid worldwide increase in chronic inflammatory disorders such as asthma. CD4(+) T helper 2 cells play critical roles in the pathogenesis of allergic airway inflammation, and CD69 expression on activated CD4 T cells is required to induce allergic inflammation in tissues. However, how CD69 mechanistically controls allergic inflammation remains poorly defined. In lymphoid tissues, CD69 regulates cellular retention through inhibition of S1P1 expression and requires no specific ligands to function...
September 16, 2016: Science Immunology
https://www.readbyqxmd.com/read/28780512/repeated-administration-of-dapirolizumab-pegol-in-a-randomised-phase-i-study-is-well-tolerated-and-accompanied-by-improvements-in-several-composite-measures-of-systemic-lupus-erythematosus-disease-activity-and-changes-in-whole-blood-transcriptomic-profiles
#14
Chris Chamberlain, Peter J Colman, Ann M Ranger, Linda C Burkly, Geoffrey I Johnston, Christian Otoul, Christian Stach, Miren Zamacona, Thomas Dörner, Murray Urowitz, Falk Hiepe
OBJECTIVES: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease associated with diffuse immune cell dysfunction. CD40-CD40 ligand (CD40L) interaction activates B cells, antigen-presenting cells and platelets. CD40L blockade might provide an innovative treatment for systemic autoimmune disorders. We investigated the safety and clinical activity of dapirolizumab pegol, a polyethylene glycol conjugated anti-CD40L Fab' fragment, in patients with SLE. METHODS: This 32-week randomised, double-blind, multicentre study (NCT01764594) evaluated repeated intravenous administration of dapirolizumab pegol in patients with SLE who were positive for/had history of antidouble stranded DNA/antinuclear antibodies and were on stable doses of immunomodulatory therapies (if applicable)...
August 5, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28778046/desalted-salicornia-europaea-extract-attenuated-vascular-neointima-formation-by-inhibiting-the-mapk-pathway-mediated-migration-and-proliferation-in-vascular-smooth-muscle-cells
#15
Kyung Jong Won, Kang Pa Lee, Suji Baek, Long Cui, Mee-Hyang Kweon, Seung Hyo Jung, Yun-Kyoung Ryu, Jung Min Hong, Eun-Ah Cho, Hwa-Sup Shin, Bokyung Kim
Salicornia europaea L. (SE) has been used as folk medicine for the treatment of various diseases such as obesity, diabetes, and cancer. However, its effects on atherosclerotic events in vascular smooth muscle cells (VSMCs) remain unknown. The present study explored the effects of the ethyl acetate fraction of desalted SE hot water extract (SEWEAF) on atherosclerotic responses (especially migration and proliferation) in VSMCs and vascular neointima formation. Treatment with the SEWEAF significantly suppressed the platelet-derived growth factor (PDGF)-BB-induced VSMC migration and proliferation as well the phosphorylation of mitogen-activated protein kinases (MAPKs) such as the p38 MAPK and extracellular signal-regulated kinase (ERK) 1/2...
August 1, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28777135/hematologic-disorders-in-children-with-continuous-renal-replacement-therapies
#16
Celia Fabra, Sara Infante, Isabel Miras, Susana Pretus, María José Santiago, Sarah N Fernández, Jesús López-Herce
The objective of this study was to analyze hematologic disorders, coagulation disorders, and transfusion requirements in children with continuous renal replacement therapies (CRRT). This is a retrospective analysis of a prospectively collected database of children receiving CRRT between 2010 and 2015. Patient characteristics, CRRT parameters, hematologic and coagulation parameters, and need for transfusions were recorded and analyzed. We compared patients after heart surgery and noncardiac patients, those requiring extracorporeal membrane oxygenation (ECMO) and those without ECMO, and patients with different anticoagulation therapies: heparin and citrate...
August 1, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28776324/maternal-and-foetal-outcomes-following-natural-vaginal-versus-caesarean-section-c-section-delivery-in-women-with-bleeding-disorders-and-carriers
#17
REVIEW
Laxminarayan Karanth, Sachchithanantham Kanagasabai, Adinegara Bl Abas
BACKGROUND: Bleeding disorders are uncommon but may pose significant bleeding complications during pregnancy, labour and following delivery for both the woman and the foetus. While many bleeding disorders in women tend to improve in pregnancy, thus decreasing the haemorrhagic risk to the mother at the time of delivery, some do not correct or return quite quickly to their pre-pregnancy levels in the postpartum period. Therefore, specific measures to prevent maternal bleeding and foetal complications during childbirth, are required...
August 4, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28774589/biallelic-mutations-in-kdsr-disrupt-ceramide-synthesis-and-result-in-a-spectrum-of-keratinization-disorders-associated-with-thrombocytopenia
#18
Takuya Takeichi, Antonio Torrelo, John Y W Lee, Yusuke Ohno, María Luisa Lozano, Akio Kihara, Lu Liu, Yuka Yasuda, Junko Ishikawa, Takatoshi Murase, Ana Belén Rodrigo, Pablo Fernández-Crehuet, Yoichiro Toi, Jemima Mellerio, José Rivera, Vicente Vicente, David P Kelsell, Yutaka Nishimura, Yusuke Okuno, Daiei Kojima, Yasushi Ogawa, Kazumitsu Sugiura, Michael A Simpson, W H Irwin McLean, Masashi Akiyama, John A McGrath
Mutations in ceramide biosynthesis pathways have been implicated in a few Mendelian disorders of keratinization although ceramides are known to have key roles in several biological processes in skin and other tissues. Using whole-exome sequencing in four probands with undiagnosed skin hyperkeratosis/ichthyosis, we identified compound heterozygosity for mutations in KDSR, encoding an enzyme in the de novo synthesis pathway of ceramides. Two individuals had hyperkeratosis confined to palms and soles as well as anogenital skin, whereas the other two had more severe, generalized harlequin ichthyosis-like skin...
July 31, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28774183/annals-express-altered-mean-platelet-volume-in-children-with-henoch-schonlein-purpura-and-its-association-with-disease-activity
#19
Xiang Shi, Wen Chao Li, Li Jun Mo, Xiao Hong Li, Yu Zhen Luo, Liu Qun Qin, Zheng Yang, Wu Ning Mo
BACKGROUND: Henoch-Schonlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. A review of the literature has suggested a correlation between mean platelet volume (MPV) and several inflammatory disorders. However, to the best of our knowledge, any potential correlation between MPV and HSP has not been reported in the literature. Therefore, our study aimed to evaluate the role of MPV levels in patients with HSP. METHODS: This study included 97 children with HSP and 120 healthy individuals as controls...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28770876/assessment-of-the-underlying-causes-of-the-immune-thrombocytopenia-ten-years-experience
#20
Mehmet Sinan Dal, Abdullah Karakus, Tuba Dal, Berrin Balik Aydin, Elif Hattapoglu, Mehmet Onder Ekmen, Turgay Ulas, Orhan Ayyildiz
OBJECTIVE: Immune thrombocytopenia (ITP) is an immune haematologic disorder causing platelet destruction mediated by anti-platelet antibodies. In this study we aimed to evaluate the clinical and laboratory variables of ITP patients in southeast of Turkey. METHODS: In this retrospective study 167 ITP patients between 2005 and 2015 were evaluated. All patients were screened for immunological parameters including ANA (antinuclear antibodies), anti dsDNA (anti-double-stranded-DNA), ACA(anti-cardiolipin) IgM and IgG, LA (lupus anticoagulants)...
July 2017: JPMA. the Journal of the Pakistan Medical Association
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