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https://www.readbyqxmd.com/read/28081351/the-effect-of-methylprednisolone-treatment-on-fibrinolysis-thecoagulation-system-and-blood-loss-in-cardiac-surgery
#1
Leila Ebrahimi, Maryam Kheirandish, Mahnoosh Foroughi
BACKGROUND/AIM: The purpose of this study was to examine steroid pretreatment in order to decrease postoperative coagulopathy disorders and bleeding. MATERIALS AND METHODS: In this randomized double-blinded study, the efficacy of low versus high doses of methylprednisolone on the coagulation system and postoperative bleeding was compared in patients who were undergoing cardiac surgery with cardiopulmonary bypass (CPB). The platelet response to agonists, D-dimer concentration, tissue plasminogen activator (tPA), plasminogen activator inhibitor (PAI-1) antigens, and platelet receptors CD42b, CD62P, and CD41a were evaluated...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28078347/alterations-of-the-platelet-proteome-in-type-i-glanzmann-thrombasthenia-caused-by-different-homozygous-delg-frameshift-mutations-in-itga2b
#2
Stefan Loroch, Katharina Trabold, Stepan Gambaryan, Cora Reiß, Kathrin Schwierczek, Ingrid Fleming, Albert Sickmann, Wolfgang Behnisch, Barbara Zieger, René P Zahedi, Ulrich Walter, Kerstin Jurk
Glanzmann thrombasthenia (GT) is one of the best characterized inherited platelet function disorders but global platelet proteome has not been determined in these patients. We investigated the proteome and function of platelets from two patients with type I GT, caused by different homozygous ITGA2b mutations, from family members and unrelated controls. The global proteome of highly purified washed platelets was quantified by liquid chromatography-mass spectrometry (LC-MS) and targeted MS-methods. Platelet function was analysed by flow cytometry, light transmission aggregometry and flow-based assays...
January 12, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28072956/-clinical-and-immunological-analysis-of-the-patient-with-autoimmunity-due-to-germline-stat3-gain-of-function-mutation
#3
Y Ding, Y Zhang, Y P Wang, H Y Zhao, X M Chen, X H Xue, X M Bai, Y F An, Z Y Zhang, X M Tang, X D Zhao
Objective: To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3. Method: A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children's Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28069912/idiosyncratic-drug-induced-neutropenia-agranulocytosis
#4
Emmanuel Andrès, Rachel Mourot-Cottet, Frédéric Maloisel, François Séverac, Olivier Keller, Thomas Vogel, Martine Tebacher, Jean-Christophe Weber, Georges Kaltenbach, Jacques-Eric Gottenberg, Bernard Goichot, Jean Sibilia, Anne-Sophie Korganow, Raoul Herbrecht
BACKGROUD: Few data is currently available on neutropenia and agranulocytosis related to drug intake. We report here data on 203 patients with established idiosyncratic drug-induced agranulocytosis, followed up in a referral centre within a university hospital. PATIENTS AND METHODS: Data from 203 patients with idiosyncratic drug-induced agranulocytosis were retrospectively reviewed. All cases were extracted from a cohort study on agranulocytosis in the Strasbourg University Hospital (Strasbourg, France) RESULTS: : The mean age was 61...
January 9, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28066748/the-use-of-platelet-rich-plasma-to-augment-conservative-and-surgical-treatment-of-hip-and-pelvic-disorders
#5
REVIEW
Matthew J Kraeutler, Tigran Garabekyan, Omer Mei-Dan
BACKGROUND: In recent years, platelet-rich plasma (PRP) has gained popularity within the orthopaedic community as a treatment modality to enhance tissue healing. PURPOSE: This review aims to concisely present the current indications for PRP injections in the treatment of hip and pelvic pathologies and to describe some novel applications for PRP which have not yet been reported in the literature. METHODS: We reviewed the literature on the non-operative and operative indications for PRP in the treatment of hip and pelvic pathologies...
July 2016: Muscles, Ligaments and Tendons Journal
https://www.readbyqxmd.com/read/28066016/thrombocytopenia-associated-with-olanzapine-a-case-report-and-review-of-literature
#6
Swapnajeet Sahoo, Himanshu Singla, M Spoorty, Pankaj Malhotra, Sandeep Grover
There is limited literature on olanzapine-associated thrombocytopenia. In this report, we present a case of a 32-year-old female, suffering from persistent delusional disorder who had thrombocytopenia (46,000/mm(3)) with the use of olanzapine 25 mg/day, 6 weeks after starting medication. Blood film did not reveal any evidence of any dysplastic cells, disturbance in the count of other cell lines, and autoimmune workup including antinuclear antibodies and anti-neutrophil cytoplasmic antibodies were found to be negative...
July 2016: Indian Journal of Psychiatry
https://www.readbyqxmd.com/read/28064294/primary-versus-secondary-immune-thrombocytopenia-in-adults-a-comparative-analysis-of-clinical-and-laboratory-attributes-in-newly-diagnosed-patients-in-southern-pakistan
#7
S Sultan, S J Ahmed, S Murad, S M Irfan
BACKGROUND: Immune thrombocytopenic purpura (ITP) is a hemorrhagic diathesis, characterized by platelets destruction alongside impaired production. Patients from Asian regions often exhibit distinctive characteristics in comparison to the western patients. We accomplished this study to evaluate the prevalence of primary versus secondary ITP along with the comparative analysis between them. The secondary objective was to determine the etiological spectrum of secondary ITP. METHODS: We illustrate the results of a large cohort of newly diagnosed adults ITP from southern Pakistan...
October 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28064200/rare-variants-in-gp1bb-are-responsible-for-autosomal-dominant-macrothrombocytopenia
#8
Suthesh Sivapalaratnam, Sarah K Westbury, Jonathan C Stephens, Daniel Greene, Kate Downes, Anne M Kelly, Claire Lentaigne, William J Astle, Eric G Huizinga, Paquita Nurden, Sofia Papadia, Kathelijne Peerlinck, Christopher J Penkett, David J Perry, Catherine Roughley, Ilenia Simeoni, Kathleen Stirrups, Daniel P Hart, R Campbell Tait, Andrew D Mumford, Nihr BioResource, Michael A Laffan, Kathleen Freson, Willem H Ouwehand, Shinji Kunishima, Ernest Turro
The von Willebrand receptor complex, which is composed of the glycoproteins Ibα and Ibβ, V and IX, plays an essential role in the earliest steps in hemostasis. Over the last four decades, it has become apparent that loss of function of any one of three of the genes encoding these glycoproteins namely, GP1BA, GP1BB and GP9, leads to autosomal recessive macrothrombocytopenia complicated by bleeding. A small number of variants in GP1BA have been reported to cause a milder and dominant form of macrothrombocytopenia but only two tentative reports exists of such a variant in GP1BB By analyzing data from a collection of over 1,000 genome-sequenced patients with a rare bleeding and/or platelet disorder, we have identified a significant association between rare monoallelic variants in GP1BB and macrothrombocytopenia...
November 14, 2016: Blood
https://www.readbyqxmd.com/read/28062601/bleeding-disorders-in-congenital-syndromes
#9
REVIEW
Susmita N Sarangi, Suchitra S Acharya
Pediatricians provide a medical home for children with congenital syndromes who often need complex multidisciplinary care. There are some syndromes associated with thrombocytopenia, inherited platelet disorders, factor deficiencies, connective tissue disorders, and vascular abnormalities, which pose a real risk of bleeding in affected children associated with trauma or surgeries. The risk of bleeding is not often an obvious feature of the syndrome and not well documented in the literature. This makes it especially hard for pediatricians who may care for a handful of children with these rare congenital syndromes in their lifetime...
January 6, 2017: Pediatrics
https://www.readbyqxmd.com/read/28060550/evaluating-platelet-function-disorders-in-children-with-bleeding-tendency-a-single-center-study
#10
Osama Tanous, Orna Steinberg Shemer, Joanne Yacobovich, Meira Zoldan, Yoseph Horovitz, Isaac Yaniv, Esther Rabizadeh, Hannah Tamary, Sigal Nakav, Judith Lahav
Platelet function disorders (PFDs) are a common cause of mild bleeding tendency. However, they cannot be recognized by standard screening studies. The gold standard test for PFD is platelet aggregation, performed by light transmission aggregometry (LTA). A newer and less validated method is the closure time (CT), performed by the platelet function Analyzer 100 (PFA-100). Data regarding the validity of these tests in children are limited. The aim of this study was to evaluate the usefulness of LTA and PFA-100 for the diagnosis of pediatric patients with bleeding tendency...
January 6, 2017: Platelets
https://www.readbyqxmd.com/read/28059092/bone-marrow-morphology-and-disease-progression-in-congenital-thrombocytopenia-a-detailed-clinicopathologic-and-genetic-study-of-eight-cases
#11
Hamilton C Tsang, James B Bussel, Susan Mathew, Yen-Chun Liu, Allison A Imahiyerobo, Attilio Orazi, Julia T Geyer
Patients with congenital thrombocytopenia have an increased risk of developing myeloid neoplasms. In these cases, the morphologic distinction between disease at baseline and at progression is challenging. This report analyzes clinicopathologic features of congenital thrombocytopenia with long-term follow-up at one referral center. Records from the last 20 years were searched for cases of congenital thrombocytopenia with bone marrow biopsies and peripheral blood smears. The clinical, morphologic, immunophenotypic, and molecular features were analyzed...
January 6, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28057742/dual-role-of-il-21-in-megakaryopoiesis-and-platelet-homeostasis
#12
Salima Benbarche, Catherine Strassel, Catherine Angénieux, Léa Mallo, Monique Freund, Christian Gachet, François Lanza, Henri de la Salle
Gene profiling studies have indicated that in vitro differentiated human megakaryocytes express the receptor for IL-21 (IL-21R), an immunostimulatory cytokine associated with inflammatory disorders and currently under evaluation in cancer therapy. The aim of this study was to investigate whether IL-21 modulates megakaryopoiesis. We first checked the expression of IL-21 receptor on human bone marrow and in vitro differentiated megakaryocytes. Then, we investigated the effect of IL-21 on the in vitro differentiation of human blood CD34+ progenitors into megakaryocytes...
January 5, 2017: Haematologica
https://www.readbyqxmd.com/read/28056758/thrombotic-management-of-antiphospholipid-syndrome-towards-novel-targeted-therapies
#13
Md Asiful Islam, Fahmida Alam, Kah Keng Wong, Mohammad Amjad Kamal, Siew Hua Gan
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and/or pregnancy morbidity with persistent levels of antiphospholipid antibodies (aPLs). The development of thrombosis in APS is mediated by aPLs and contributes to the high mortality rate in APS patients. However, although APS has been reported for more than 30 years, there has been no optimal regimen for its prevention or for the management of thrombosis, mainly because the mainstay treatment strategies for managing APS are not targeted towards aPL-mediated thrombotic pathophysiology...
January 5, 2017: Current Vascular Pharmacology
https://www.readbyqxmd.com/read/28053696/invasive-thymoma-with-pure-red-cell-aplasia-and-amegakaryocytic-thrombocytopenia
#14
Takuya Onuki, Yusuke Kiyoki, Sho Ueda, Masatoshi Yamaoka, Seiich Shimizu, Masaharu Inagaki
We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 10(4)/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal tumor was detected, and bone marrow biopsy revealed few megakaryocytes and severely reduced numbers of erythroid cells. The diagnosis was thymoma with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT)...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/28051053/simultaneous-occurrence-of-intussusception-and-duodenal-haematoma-in-a-patient-with-glanzmann-s-thrombasthenia
#15
Iftikhar Ahmad Jan, Saif Khalfan AlKaabi, Awadh AlMughadam, D Sreenivas Murthy, Basmah AlHamoudi
Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder in which the platelets have anomalies of fibrinogen receptors causing bleeding tendencies. The disease usually presents with mucosal bleeding, petechial haemorrhages or gastrointestinal (GI) bleeding. Only sporadic cases of intussusception or duodenal haematoma have been reported with GT. We present a case of 5-year-old girl admitted with non-bilious vomiting, watery diarrhoea and abdominal pain. She is a known patient of Glanzmann's thrombasthenia...
October 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/28049097/in-vivo-anti-arthritic-efficacy-of-camellia-sinensis-l-in-collagen-induced-arthritis-model
#16
Ankit Tanwar, Raman Chawla, Md Meraj Ansari, Neha, Pallavi Thakur, Ankita Singh Chakotiya, Rajeev Goel, Himanshu Ojha, M Asif, Mitra Basu, Rajesh Arora, Haider Ali Khan
BACKGROUND: Rheumatoid arthritis (RA), an autoimmune inflammatory disorder with synovial hyperplasia, destruction of cartilage, bone damage is often associated with risk of infections. Such risk could be attributed towards usage of immunosuppressive agents. Thus, the present study was undertaken to evaluate the anti-arthritic efficacy of aquo-alcoholic extract of Camellia sinensis (L.). MATERIAL AND METHODS: Dried leaves of Camellia sinensis (L.) or Cs were filtered and extracted in 1:1 aqueous: ethanol by Soxhlet apparatus followed by lyophilization and spray drying to develop amorphous powder...
December 31, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28045096/diabetes-cardiac-disorders-and-asthma-as-risk-factors-for-severe-organ-involvement-among-adult-dengue-patients-a-matched-case-control-study
#17
Junxiong Pang, Jung Pu Hsu, Tsin Wen Yeo, Yee Sin Leo, David C Lye
Progression to severe organ involvement due to dengue infection has been associated with severe dengue disease, intensive care treatment, and mortality. However, there is a lack of understanding of the impact of pre-existing comorbidities and other risk factors of severe organ involvement among dengue adults. The aim of this retrospective case-control study is to characterize and identify risk factors that predispose dengue adults at risk of progression with severe organ involvement. This study involved 174 dengue patients who had progressed with severe organ involvement and 865 dengue patients without severe organ involvement, matched by the year of presentation of the cases, who were admitted to Tan Tock Seng Hospital between year 2005 and 2008...
January 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28043617/bedaquiline-in-the-multidrug-resistant-tuberculosis-treatment-belarus-experience
#18
Alena Skrahina, Hennadz Hurevich, Dennis Falzon, Liudmila Zhilevich, Valiantsin Rusovich, Masoud Dara, Svetlana Setkina
BACKGROUND/OBJECTIVE: Outcomes of treatment for multidrug-resistant tuberculosis (MDR-TB) remain poor worldwide. Among patients with MDR-TB in Belarus who started treatment in 2012, only 54% completed it successfully, with treatment failure reported in 22% of the patients; additionally, 11% died and 13% were lost to follow-up or remained unevaluated. In Belarus, to improve outcomes, bedaquiline was introduced in MDR-TB treatment in June 2015. The national TB program developed measures to monitor safety and effectiveness of bedaquiline-containing regimens in line with the World Health Organization recommendations...
December 2016: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28041820/combined-alpha-delta-platelet-storage-pool-deficiency-is-associated-with-mutations-in-gfi1b
#19
Carlos R Ferreira, Dong Chen, Shirley M Abraham, David R Adams, Karen L Simon, May C Malicdan, Thomas C Markello, Meral Gunay-Aygun, William A Gahl
Combined alpha-delta platelet storage pool deficiency is characterized by the absence or reduction in the number of both alpha granules and dense bodies. This disorder can have variable severity as well as a variable inheritance pattern. We describe two patients from unrelated families with combined alpha-delta storage pool deficiency due to mutations in GFI1B, a zinc finger protein known to act as a transcriptional repressor of various genes. We demonstrate that this disease is associated with either a heterozygous mutation (de novo or familial) abrogating the binding of the zinc fingers with the promoter of its target genes, or by hypomorphic biallelic mutations in GFI1B leading to autosomal recessive inheritance...
December 18, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28039017/development-of-pro-inflammatory-phenotype-in-monocytes-after-engulfing-hb-activated-platelets-in-hemolytic-disorders
#20
Rashi Singhal, Sheetal Chawla, Deepak K Rathore, Angika Bhasym, Gowtham K Annarapu, Vandana Sharma, Tulika Seth, Prasenjit Guchhait
Monocytes and macrophage combat infections and maintain homeostatic balance by engulfing microbes and apoptotic cells, and releasing inflammatory cytokines. Studies have described that these cells develop anti-inflammatory properties upon recycling the free-hemoglobin (Hb) in hemolytic conditions. While investigating the phenotype of monocytes in two hemolytic disorders-paroxysmal nocturnal hemoglobinuria (PNH) and sickle cell disease (SCD), we observed a high number of pro-inflammatory (CD14(+)CD16(hi)) monocytes in these patients...
December 28, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
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