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Bleeding disorders

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https://www.readbyqxmd.com/read/28811987/early-complications-and-outcomes-in-adult-spinal-deformity-surgery-an-nsqip-study-based-on-5803-patients
#1
Nathan J Lee, Parth Kothari, Jun S Kim, John I Shin, Kevin Phan, John Di Capua, Sulaiman Somani, Dante M Leven, Javier Z Guzman, Samuel K Cho
STUDY DESIGN: Retrospective analysis. OBJECTIVE: The purpose of this study is to determine the incidence, impact, and risk factors for short-term postoperative complications following elective adult spinal deformity (ASD) surgery. METHODS: Current Procedural Terminology codes were used to query the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) for adults who underwent spinal deformity surgery from 2010 to 2014...
August 2017: Global Spine Journal
https://www.readbyqxmd.com/read/28810430/exploring-changes-in-distress-among-individuals-with-bleeding-disorders-what-is-linked-to-improvements-in-distress
#2
Vaughn Barry, Mary Ellen Lynch, Duc Q Tran, Ana Antun, Anthony DeBalsi, Denise Hicks, Francie Lasseter, Shanna Mattis, Maria Ja Ribeiro, Sidney F Stein, Christine L Kempton
Adults with chronic bleeding disorders report high distress, but it is unclear how distress varies over time. Patients rated their distress on a 10-point scale at two clinic visits. Of 83 patients, roughly one-quarter reported consistent no/low distress (29%), one-quarter reported consistent distress (22%), and half (49%) reported a change in distress of at least two points. Overall activity levels, depressive symptoms, and non-White race were significantly associated with worsening and consistent distress in adjusted analyses while improvements in activity levels and depressive symptoms during the study period were associated with distress improvement...
March 1, 2017: Journal of Health Psychology
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
#3
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/28806860/patient-caregiver-and-provider-perceptions-of-pain-and-pain-management-in-adolescents-and-young-adults-with-bleeding-disorders
#4
Angela Lambing, Cynthia D Nichols, James E Munn, Terry L Anderson, Bartholomew J Tortella, Michelle L Witkop
INTRODUCTION: Recurrent bleeding and associated pain are critical components in the management of bleeding disorders, yet scant data describe perceptions of pain in this patient population. OBJECTIVE: This study assessed perceptions of pain and pain management in adolescents and young adults (AYAs) with haemophilia or von Willebrand disease (VWD) to determine agreement/disagreement between patients, caregivers and health care providers. METHODS: Using an online questionnaire, AYA patients (N=89), their caregivers (N=77), and providers (N=54) reported on pain perception, pain treatment and pain control...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28806858/the-haemtrack-home-therapy-reporting-system-design-implementation-strengths-and-weaknesses-a-report-from-uk-haemophilia-centre-doctors-organisation
#5
C R M Hay, H Xiang, M Scott, P W Collins, R Liesner, G Dolan, R Hollingsworth
INTRODUCTION: Haemtrack is an electronic home treatment diary for patients with inherited bleeding disorders, introduced in 2008. It aimed to improve the timeliness and completeness of patient-reported treatment records, to facilitate analysis of treatment and outcome trends. The system is easy to use, responsive and accessible. METHODS: The software uses Microsoft technologies with a SQL Server database and an ASP.net website front-end, running on personal computers, android and I-phones...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28804850/laboratory-testing-for-von-willebrand-factor-multimers
#6
Susan Oliver, Kun Kan Edwin Lau, Kent Chapman, 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 the 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. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804848/laboratory-testing-for-von-willebrand-factor-factor-viii-binding-for-2n-vwd
#7
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 the 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 a protocol for assessment of VWF activity by means of VWF: factor VIII binding (VWF:FVIIIB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804846/laboratory-testing-for-von-willebrand-factor-ristocetin-cofactor-vwf-rco
#8
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/28804845/laboratory-testing-for-von-willebrand-factor-collagen-binding-vwf-cb
#9
Emmanuel J Favaloro, Soma Mohammed
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 the 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. This chapter describes several protocols for assessment of VWF activity by means of VWF collagen binding (VWF:CB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804844/laboratory-testing-for-von-willebrand-factor-antigen-vwf-ag
#10
Emmanuel J Favaloro, Soma Mohammed, Jürgen Patzke
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders arise due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the 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 protocols for assessment of VWF level by means of VWF antigen (VWF:Ag)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804843/diagnosis-or-exclusion-of-von-willebrand-disease-using-laboratory-testing
#11
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
#12
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
#13
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
#14
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/28804835/chromogenic-factor-viii-assays-for-improved-diagnosis-of-hemophilia-a
#15
Susan Rodgers, Elizabeth Duncan
Hemophilia A is an inherited bleeding disorder caused by a reduced level of factor VIII coagulant activity (FVIII:C) in blood. Bleeding episodes may occur spontaneously in the severe form of hemophilia or after trauma in the milder forms. It is important that patients are diagnosed correctly, which includes placing them into the correct severity category of the disorder so that appropriate treatment can be given. Diagnosis is made by determination of the amount of FVIII:C in the blood, usually using a one-stage factor VIII:C assay...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804822/determination-of-fibrinogen-and-thrombin-time-tt
#16
Anetta Undas
Fibrinogen is measured in plasma most commonly using the Clauss method, based on the comparison of thrombin clotting times of dilutions of plasma against a plasma standard. Thrombin time (TT) is a coagulation assay, which reflects the conversion of fibrinogen to fibrin after addition of thrombin reagent. Measurement of clottable fibrinogen and TT allows detecting inborn (congenital) and acquired qualitative and quantitative disorders of fibrinogen that can lead to thrombotic or bleeding events.
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804815/overview-of-hemostasis-and-thrombosis-and-contribution-of-laboratory-testing-to-diagnosis-and-management-of-hemostasis-and-thrombosis-disorders
#17
Roslyn A Bonar, Giuseppe Lippi, Emmanuel J Favaloro
Hemostasis is a complex and tightly regulated process whereby the body attempts to maintain a homeostatic balance to permit normal blood flow, without bleeding or thrombosis. When this balance is disrupted, due to trauma or underlying congenital bleeding or thrombotic disorders, clinical intervention may be required. To assist clinicians in diagnosing and managing affected patients, hemostasis laboratories offer an arsenal of tests, both routine (screening) and more specialized (diagnostic). In general, screening assays are used to screen for hemostasis-related disease or to monitor or measure the effect of anticoagulant therapy, which may be applied to treat patients with recent thrombosis or at risk of thrombosis...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28802751/acute-management-of-hemostasis-in-patients-with-neurological-injury
#18
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
#19
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/28801214/lobar-hemorrhage-induced-by-acquired-factor-xiii-deficiency-in-a-patient-with-cerebral-amyloid-angiopathy
#20
Hidetaka Arishima, Hiroyuki Neishi, Ken-Ichiro Kikuta, Mihoko Morita, Naoko Hosono, Takahiro Yamauchi, Masayoshi Souri, Akitada Ichinose
A 68-year-old man presented with intracranial hemorrhage in the right frontal lobe, which rapidly increased the day after admission. We performed hematoma removal with a biopsy of the cortex around the hematoma. The day after the operation, a subcutaneous hematoma over the craniotomy appeared, and the computed tomography showed a recurrent hemorrhage with an acute subdural hematoma. We were aware of a bleeding tendency, and a detailed hematologic examination by hematologists revealed autoimmune acquired factor XIII deficiency due to an antifactor XIII antibody...
August 8, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
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