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https://www.readbyqxmd.com/read/28647217/recognition-and-management-of-hemostatic-disorders-in-critically-ill-patients-needing-to-undergo-an-invasive-procedure
#1
REVIEW
Marcella C A Müller, Simon J Stanworth, Michiel Coppens, Nicole P Juffermans
Abnormal laboratory coagulation test results are frequently documented in critically ill patients, and these patients often also need to undergo invasive procedures. Clinicians have an understandable desire to minimize any perceived heightened risk of bleeding complications in those patients who require invasive procedures. In this setting, prophylactic administration of platelets or plasma is commonplace. This review explores the nature of these sequential statements and the degree to which these statements are supported by evidence...
June 15, 2017: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/28645365/complexity-of-immune-responses-to-aav-transgene-products-example-of-factor-ix
#2
REVIEW
Roland W Herzog
After two decades of research, in vivo gene transfer with adeno-associated viral (AAV) vectors has now resulted in successful treatments and even cures for several human diseases. However, the potential for immune responses against the therapeutic gene products remains one of the concerns as this approach is broadened to more patients, diverse diseases, and target organs. Immune responses following gene transfer of coagulation factor IX (FIX) for the treatment of the bleeding disorder hemophilia B has been extensively investigated in multiple animal models...
May 29, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/28645246/igmk-paraprotein-from-gammopathy-patient-can-bind-to-cardiolipin-and-interfere-with-coagulation-assay-a-case-report
#3
Xin-Yao Wu, Yu-Feng Yin, Jia-Lin Teng, Li-Wei Zhang, Cheng-de Yang
BACKGROUND: The monoclonal gammopathies are a group of plasma-cell proliferative disorders characterized by the secretion of monoclonal immunoglobulin (M protein or paraprotein). Some rare cases have revealed the specific affinity of paraprotein as autoantibody. Here we report a patient with monoclonal gammopathy of undetermined significance (MGUS) accompanied by a remarkable increase of anticardiolipin antibody (aCL) and an extensively decreased coagulation factor activity, however, without any clinical signs of antiphospholipid syndrome (APS) and bleeding...
June 23, 2017: BMC Immunology
https://www.readbyqxmd.com/read/28645121/effect-of-obesity-on-gestational-and-perinatal-outcomes
#4
Sônia Regina Cabral Madi, Rosa Maria Rahmi Garcia, Vandrea Carla de Souza, Renato Luís Rombaldi, Breno Fauth de Araújo, José Mauro Madi
Purpose To assess the impact of pre-pregnancy obesity (body mass index [BMI] ≥ 30 kg/m(2)) on the gestational and perinatal outcomes. Methods Retrospective cohort study of 731 pregnant women with a BMI ≥ 30 kg/m(2) at the first prenatal care visit, comparing them with 3,161 women with a BMI between 18.5 kg/m(2) and 24.9 kg/m(2). Maternal and neonatal variables were assessed. Statistical analyses reporting the demographic features of the pregnant women (obese and normal) were performed with descriptive statistics followed by two-sided independent Student's t tests for the continuous variables, and the chi-squared (χ(2)) test, or Fisher's exact test, for the categorical variables...
June 23, 2017: Revista Brasileira de Ginecologia e Obstetrícia
https://www.readbyqxmd.com/read/28644313/unique-clinical-features-of-los-angeles-grade-d-esophagitis-suggest-that-factors-other-than-gastroesophageal-reflux-contribute-to-its-pathogenesis
#5
Anh D Nguyen, Stuart J Spechler, Monique N Shuler, Rhonda F Souza, Kerry B Dunbar
BACKGROUND: The Los Angeles (LA) grade of reflux esophagitis (A to D) is assumed to reflect severity of the underlying gastroesophageal reflux disease (GERD). Thus, LA-D esophagitis patients might be expected to have the most conditions predisposing to GERD (eg, obesity, hiatal hernia), and the highest frequency of GERD symptoms. GOALS: The main goal of this study is to compare clinical features of patients with the most severe (LA-D) and mildest (LA-A) grades of esophagitis...
June 21, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28643773/a-cdc42-rhoa-regulatory-circuit-downstream-of-glycoprotein-ib-guides-transendothelial-platelet-biogenesis
#6
Sebastian Dütting, Frederique Gaits-Iacovoni, David Stegner, Michael Popp, Adrien Antkowiak, Judith M M van Eeuwijk, Paquita Nurden, Simon Stritt, Tobias Heib, Katja Aurbach, Oguzhan Angay, Deya Cherpokova, Niels Heinz, Ayesha A Baig, Maximilian G Gorelashvili, Frank Gerner, Katrin G Heinze, Jerry Ware, Georg Krohne, Zaverio M Ruggeri, Alan T Nurden, Harald Schulze, Ute Modlich, Irina Pleines, Cord Brakebusch, Bernhard Nieswandt
Blood platelets are produced by large bone marrow (BM) precursor cells, megakaryocytes (MKs), which extend cytoplasmic protrusions (proplatelets) into BM sinusoids. The molecular cues that control MK polarization towards sinusoids and limit transendothelial crossing to proplatelets remain unknown. Here, we show that the small GTPases Cdc42 and RhoA act as a regulatory circuit downstream of the MK-specific mechanoreceptor GPIb to coordinate polarized transendothelial platelet biogenesis. Functional deficiency of either GPIb or Cdc42 impairs transendothelial proplatelet formation...
June 15, 2017: Nature Communications
https://www.readbyqxmd.com/read/28643468/eltrombopag-use-in-a-patient-with-wiskott-aldrich-syndrome
#7
Maria Gabelli, Antonio Marzollo, Lucia Dora Notarangelo, Giuseppe Basso, Maria Caterina Putti
Wiskott-Aldrich syndrome (WAS) is an inherited X-linked disorder characterized by microthrombocytopenia, immunodeficiency, and eczema. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice. Eltrombopag, a thrombopoietin receptor agonist, may be useful to prevent bleeding while awaiting HSCT. We present a case of a male with WAS, profound thrombocytopenia, and bleeding diathesis successfully managed with eltrombopag before HSCT. Eltrombopag was given for 32 weeks obtaining a stable platelet count without any platelet transfusion...
June 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28640903/type-2b-von-willebrand-disease-with-or-without-large-multimers-a-distinction-of-the-two-sides-of-the-disorder-is-long-overdue
#8
Alessandra Casonato, Viviana Daidone, Eva Galletta, Antonella Bertomoro
Most, but not all patients with type 2B von Willebrand disease (VWD)-which features gain-of-function mutations in the A1 domain of von Willebrand factor (VWF)-have no circulating large VWF multimers. Similarities and differences were analysed in 33 type 2B patients, 12 with a normal and 21 with an abnormal multimer pattern, to see whether they should be considered separately. The minimum aggregating dose of ristocetin was similarly reduced in both patient groups, and modulated by their underlying VWF mutations...
2017: PloS One
https://www.readbyqxmd.com/read/28640329/association-of-adverse-oral-health-outcomes-with-socioeconomic-inequalities-and-dental-needs-in-brazilian-adolescents
#9
Daniela de Rossi Figueiredo, João Luiz Bastos, Karen Glazer Peres
This study aimed to explore the relations between adverse oral outcomes and socioeconomic, demographic, and self-rated oral health variables and to describe their distribution. Principal component analysis was conducted on data from adolescents in the Brazilian National Oral Health Survey (N = 5,445). Higher loadings were found for crowding (0.6), maxillary and mandibular irregularities (0.5), and count of communitary periodontal index (CPI) sextants with bleeding and dental calculus (0.5). The mean rates for periodontal and occlusal disorders were at least two times higher in adolescents from lower income families and those reporting the need for dental prostheses, as well as those dissatisfied with their dental and overall oral health...
June 5, 2017: Cadernos de Saúde Pública
https://www.readbyqxmd.com/read/28639570/menorrhagia-and-uterine-volume-associated-with-lower-urinary-tract-symptoms-in-patients-with-adenomyosis
#10
Ting Li, Xiao-Xuan Xu, Yi Dai, Jun-Ji Zhang, Jing-He Lang, Jin-Hua Leng
BACKGROUND: Adenomyosis is a gynecological disorder with symptoms most presenting as dysmenorrhea and heavy menstrual bleeding. However, the presence of lower urinary tract symptoms (LUTS) among women with adenomyosis remains unclear. This study was designed to determine the prevalence of LUTS and factors related to the severity of these symptoms in this population. METHODS: From July 2016 to November 2016, a total of 298 untreated symptomatic adenomyosis patients and 280 age-matched controls were enrolled...
July 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28638289/diagnosis-and-treatment-of-hereditary-hemorrhagic-telangiectasia
#11
REVIEW
Cameron Grigg, Daniel Anderson, James Earnshaw
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disorder known for its debilitating symptoms. More than 90% of patients with HHT experience epistaxis, and they average up to 18 bleeds per month. We review the current literature on the pathophysiology, clinical presentation, and management of HHT. METHODS: We searched MEDLINE, EMBASE, and PubMed and identified 19 articles published since 2000 with current information on HHT. RESULTS: HHT is a disease more commonly associated with significant morbidity rather than mortality...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28637664/expanded-repertoire-of-rasgrp2-variants-responsible-for-platelet-dysfunction-and-severe-bleeding
#12
Sarah K Westbury, Matthias Canault, Daniel Greene, Emilse Bermejo, Katharine Hanlon, Michele P Lambert, Carolyn M Millar, Paquita Nurden, Samya G Obaji, Shoshana Revel-Vilk, Chris Van Geet, Kate Downes, Sofia Papadia, Salih Tuna, Christopher Watt, Nihr BioResource-Rare Diseases Consortium, Kathleen Freson, Michael A Laffan, Willem H Ouwehand, Marie-Christine Alessi, Ernest Turro, Andrew D Mumford
Heritable platelet function disorders (PFDs) are genetically heterogeneous and poorly characterised. Pathogenic variants in RASGRP2, which encodes calcium and diacylglycerol-regulated guanine exchange factor I (CalDAG-GEFI), have been reported previously in three pedigrees with bleeding and reduced platelet aggregation responses. To better define the phenotype associated with pathogenic RASGRP2 variants, we compared high-throughput sequencing and phenotype data from 2,042 cases in pedigrees with unexplained bleeding or platelet disorders to data from 5,422 controls...
June 21, 2017: Blood
https://www.readbyqxmd.com/read/28637620/runx1-deficiency-familial-platelet-disorder-with-predisposition-to-myeloid-leukemia-fpdmm
#13
REVIEW
Brigitte Schlegelberger, Paula G Heller
In this review, we discuss disease-causing alterations of RUNT-related transcription factor 1 (RUNX1), a master regulator of hematopoietic differentiation. Familial platelet disorder with predisposition to myeloid leukemia (FPDMM) typically presents with (1) mild to moderate thrombocytopenia with normal-sized platelets; (2) functional platelets defects leading to prolonged bleeding; and (3) an increased risk to develop myelodysplastic syndromes (MDS), acute myeloid leukemia (AML), or T-cell acute lymphoblastic leukemia (T-ALL)...
April 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/28636092/impact-of-diagnosis-of-von-willebrand-disease-on-patient-outcomes-analysis-of-medical-insurance-claims-data
#14
R F Sidonio, K M Haley, D Fallaize
The inherited bleeding disorder von Willebrand disease (VWD) is challenging to diagnose owing to disease heterogeneity, lack of a definitive laboratory test and variations in diagnostic criteria. We evaluated the impact of diagnosis and diagnostic delay on patient outcomes. The PharMetrics Plus Database was interrogated for medical claims for VWD (ICD-9 286.4) and bleeding events between 1 January 2006 and 30 June 2015. Longitudinal analysis was performed of patients newly diagnosed with VWD (≥9 months' continuous enrolment before first VWD claim) through 24 months following diagnosis...
June 21, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28634578/neonatal-venous-thromboembolism
#15
REVIEW
Kristina M Haley
Neonates are the pediatric population at highest risk for development of venous thromboembolism (VTE), and the incidence of VTE in the neonatal population is increasing. This is especially true in the critically ill population. Several large studies indicate that the incidence of neonatal VTE is up almost threefold in the last two decades. Central lines, fluid fluctuations, sepsis, liver dysfunction, and inflammation contribute to the risk profile for VTE development in ill neonates. In addition, the neonatal hemostatic system is different from that of older children and adults...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28634523/mallory-weiss-tear-after-violent-hiccups-a-rare-association
#16
Sankalp Dwivedi, Hussein Al-Hamid, Bradley J Warren
We present an unusual case of a 44-year-old male who developed violent hiccups soon after a ureteroscopy for nephrolithiasis; later, the forceful hiccups were followed by hematemesis. Upper esophagogastroduodenoscopy revealed Mallory-Weiss tears and esophageal erosions in the lower esophagus. Esophageal biopsy was unremarkable. The patient did not have a prior history of the gastrointestinal disorder. Although extracorporeal shock wave lithotripsy has on rare occasion been implicated in the development of gastrointestinal erosions, no such correlation exists for ureteroscopy and upper gastrointestinal bleeding in the management of nephrolithiasis...
January 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28634189/reduced-platelet-count-but-no-major-platelet-function-abnormalities-are-associated-with-loss-of-function-atp-binding-cassette-1-abca1-gene-mutations
#17
Pietro Minuz, Alessandra Meneguzzi, Eti Alessandra Femia, Cristiano Fava, Stefano Calabria, Mariangela Scavone, Donatella Benati, Giovanni Poli, Carlo Zancanaro, Sebastiano Calandra, Tiziano Lucchi, Marco Cattaneo
Loss of function mutations of the the ATP-binding cassette-1 (ABCA1) gene are the cause of Tangier disease (TD) in homozygous subjects and familial HDL deficiency (FHD) in heterozygous subjects. These disorders are characterized by reduced plasma HDL-cholesterol and altered efflux of cholesterol from cells. Previous studies in TD patients and ABCA1(-/-) murine models reported defects in platelet count, morphology and function, but the issue is still controversial. We analysed three subjects with low to very low HDL cholesterol levels due to loss of function mutations of the ABCA1 gene...
June 20, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28629414/fibrinogen-deficiency-in-a-dog-a-case-report
#18
Franck Jolivet, Armelle Diquélou, Catherine Trumel, Simon Privat, Olivier Dossin
BACKGROUND: Among coagulation disorders, primary fibrinogen deficiency is very rare in dogs. It is divided into hypofibrinogenemia, afibrinogenemia and dysfibrinogenemia. Afibrinogenemia has been described in three dogs. There are, however, no published case reports of primary hypofibrinogenemia in dogs. CASE PRESENTATION: A 1.5 year-old male German Pointer dog was evaluated for a locked-jaw syndrome associated with eye protrusion which appeared after a minor head trauma...
June 19, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28625908/-on-the-spot-the-use-of-4d-cta-to-differentiate-a-true-spot-sign-from-a-distal-intracranial-aneurysm
#19
Johanna P de Jong, Leo Kluijtmans, Martinus J van Amerongen, Mathias Prokop, Hieronymus D Boogaarts, Frederick J A Meijer
BACKGROUND: Spontaneous intracerebral hemorrhage may arise from underlying abnormalities including aneurysms. Computed tomography angiography (CTA) is widely used for the detection of possible underlying causes, which is important since it may have immediate therapeutic consequences. In addition, CTA is used to detect the so-called "spot sign", indicating active hemorrhage which carries a worse prognosis. CTA is, however, a snapshot in time. Four-dimensional CTA (4D-CTA) is a dynamic type of imaging and has emerged as a valuable imaging technique for different neurovascular disorders...
June 15, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28624906/pathogen-reduction-inactivation-of-products-for-the-treatment-of-bleeding-disorders-what-are-the-processes-and-what-should-we-say-to-patients
#20
Giovanni Di Minno, David Navarro, Carlo Federico Perno, Mariana Canaro, Lutz Gürtler, James W Ironside, Hermann Eichler, Andreas Tiede
Patients with blood disorders (including leukaemia, platelet function disorders and coagulation factor deficiencies) or acute bleeding receive blood-derived products, such as red blood cells, platelet concentrates and plasma-derived products. Although the risk of pathogen contamination of blood products has fallen considerably over the past three decades, contamination is still a topic of concern. In order to counsel patients and obtain informed consent before transfusion, physicians are required to keep up to date with current knowledge on residual risk of pathogen transmission and methods of pathogen removal/inactivation...
June 18, 2017: Annals of Hematology
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