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https://www.readbyqxmd.com/read/27929201/the-importance-of-genetic-factors-for-the-development-of-arthropathy-a-longitudinal-study-of-children-and-adolescents-with-haemophilia-a
#1
Edward D Gomperts, John Schwarz, Sharyne M Donfield, Alice E Lail, Jan Astermark, W Keith Hoots, Cheryl A Winkler, Erik Berntorp
Haemophilia A is a congenital bleeding disorder characterised by recurrent haemorrhages into the major joints. Haemophilic arthropathy is a well-established outcome of recurrent joint bleeding; however, it is clear that multiple factors determine the extent and severity of its occurrence. We sought to identify genetic factors related to abnormalities in range of motion (ROM) in the knees, ankles and elbows in a cohort of children and adolescents with haemophilia A not treated primarily with regular prophylaxis...
December 8, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27928886/f376a-m388a-solulin-a-new-promising-antifibrinolytic-for-severe-haemophilia-a
#2
J Parcq, K U Petersen, A Borel-Derlon, P Gautier, M Ebel, D Vivien, Y Repessé
INTRODUCTION: Haemophilia is a major bleeding disorder due to a deficiency of procoagulant factor VIII (type A) or IX (type B). The treatment is substitutive and based on infusion of factor concentrates. Main limitations of this therapy are cost, short factor half-life and the development of inhibitors (up to 30% of severe HA patients). An important aggravating factor of haemophilia is due to a premature fibrinolysis, directing attention to the therapeutic potential of suitable antifibrinolytics...
December 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27927609/data-analysis-protocol-for-the-development-and-evaluation-of-population-pharmacokinetic-models-for-incorporation-into-the-web-accessible-population-pharmacokinetic-service-hemophilia-wapps-hemo
#3
Alanna McEneny-King, Gary Foster, Alfonso Iorio, Andrea N Edginton
BACKGROUND: Hemophilia is an inherited bleeding disorder caused by a deficiency in a specific clotting factor. This results in spontaneous bleeding episodes and eventual arthropathy. The mainstay of hemophilia treatment is prophylactic replacement of the missing factor, but an optimal regimen remains to be determined. Rather, individualized prophylaxis has been suggested to improve both patient safety and resource utilization. However, uptake of this approach has been hampered by the demanding sampling schedules and complex calculations required to obtain individual estimates of pharmacokinetic (PK) parameters...
December 7, 2016: JMIR Research Protocols
https://www.readbyqxmd.com/read/27927518/removal-of-infected-posterior-spinal-implants-be-prepared-to-transfuse
#4
Lindsay M Andras, Elizabeth R A Joiner, Kira Skaggs, Liam R Harris, Alexander M Broom, Amy Williams, David L Skaggs
STUDY DESIGN: Single-center retrospective review of spinal deformity patients undergoing removal of infected posterior spinal fusion implants over a 10-year period. OBJECTIVE: To evaluate the intraoperative blood loss and perioperative complications of implant removal in posterior spinal fusions. SUMMARY OF BACKGROUND DATA: To our knowledge, no studies examine blood loss or complications associated with removal of infected spinal implants in spinal deformity...
July 2016: Spine Deformity
https://www.readbyqxmd.com/read/27926465/freestyle-stentless-bioprosthesis-for-aortic-valve-therapy-17-year-clinical-results
#5
Juergen Ennker, Markus Meilwes, Joern Pons-Kuehnemann, Bernd Niemann, Philippe Grieshaber, Ina C Ennker, Andreas Boening
BACKGROUND: Aortic valve replacement with stentless bioprostheses has been shown to produce lower aortic gradients than stented bioprostheses, thus facilitating left ventricular mass regression and preventing heart failure. We sought to determine the long-term results of stentless biological aortic valve replacement over a 17-year follow-up. METHODS: Between 1996 and 2012, 2551 patients underwent isolated aortic valve replacement with a stentless prosthesis (Medtronic Freestyle) at a single center...
November 2016: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/27924713/acetylcholinesterase-and-neuropathy-target-esterase-activities-in-11-cases-of-symptomatic-flight-crew-members-after-fume-events
#6
Astrid R R Heutelbeck, Catherine Bornemann, Martina Lange, Anke Seeckts, Michael M Müller
In modern aviation, so-called fume events such as exposure to an unknown mixture of chemicals introduced into the aircraft cabin with bleed air drawn off at the engines may occur. Human exposure may result in (neuro)toxic symptoms described as so-called "aerotoxic syndrome." Currently, among other agents organophosphates (OP) are regarded as a likely cause of the observed adverse effects. After fume events 11 flight crew members (9 female/2 male; ages 23-58 yr) were admitted for a medical examination within 5 d post exposure...
2016: Journal of Toxicology and Environmental Health. Part A
https://www.readbyqxmd.com/read/27919671/supracondylar-femoral-osteotomy-and-knee-joint-replacement-during-the-same-surgical-procedure-in-a-type-a-haemophiliac-patient-with-knee-flexion-deformity-and-ankylosis
#7
Jose Luis Osma Rueda, Alejandra Oliveros Vargas, Cristian David Sosa
BACKGROUND: Haemophilia A is the cause of diverse musculoskeletal disorders such as ankylosis, arthritis and associated angular deformity. There are few reported cases in patients with haemophilia A in which simultaneous supracondylar femoral osteotomy and knee joint replacement has been performed to treat knee angular deformity and ankylosis. Here we present the case of an 18year old male patient, with an evolution of two years, who was unable to walk due to the presence of an untreated supracondylar fracture in the left femur and ipsilateral haemophilic arthropathy which led him to develop an ankylosis in flexion close to 70°...
December 2, 2016: Knee
https://www.readbyqxmd.com/read/27919133/-significance-of-urological-surgical-treatment-for-viral-hemorrhagic-cystitis-after-allogeneic-hematopoietic-stem-cell-transplantation
#8
Kazuhiro Kurosawa, Shinji Urakami, Kazuya Ishiwata, Jinpei Miyagawa, Kazushige Sakaguchi, Masashi Fujioka, Hirokatsu Murata, Naoko Inoshita, Shuichi Taniguchi, Toshikazu Okaneya
This study investigated the significance of urological surgical intervention for viral hemorrhagic cystitis (HC) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). A total of 1, 024 patients underwent allo-HSCT at our medical center between January 2006 and July 2014. In the 6 patients (0.58%) who required urological surgical treatment for viral HC, we retrospectively analyzed patient characteristics and outcomes. Two patients underwent nephrostomy for bilateral hydronephrosis due to bladder tamponade...
November 2016: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/27918165/-sarcoidosis-of-the-female-genital-tract
#9
A Šefčíková, M Turková, M Žurková
OBJECTIVE: To present the findings of sarcoidosis on female genital tract. DESIGN: Review. SETTING: Department of Obstetric and Gynecology, Silesian Hospital Opava. METHODS: Overview of published findings from case studies. CONCLUSION: Sarcoidosis is a multisystem granulomatous disorder of unclear cause. It typically involves the lymph nodes of mediastinum, predominantly billateral and/or pulmonary infiltrates...
2016: Ceská Gynekologie
https://www.readbyqxmd.com/read/27918159/-pregnancy-in-women-with-congenital-bleeding-disorder
#10
T Binder, P Salaj, V Komrska
THE AIM OF THE STUDY: To highlight the risks associated with pregnancy at women with von Willebrand´s disease or hemophilia. Introduce the rules of multidisciplinary prenatal and peripartal care to minimalize these risks. The article is accompanied by case report where maladministration led to fatal consequences for the newborn. DESIGN: Review and case report.Seatings: Department Obstetric and Gynecology UJEP and Masaryk´s Hospital Ústí n/Labem, Institute Haematology and Blood Transfer Prague, Children´s Haemato-onkology Clinic University Hospital Prague Motol...
2016: Ceská Gynekologie
https://www.readbyqxmd.com/read/27917714/antiplatelet-and-anticoagulation-treatment-in-patients-with-thrombocytopenia
#11
Nuccia Morici, Silvia Cantoni, Paola Vallerio, Marco Cattaneo, Stefano Savonitto
Thrombocytopenia (TP) is a common finding in patients hospitalized for cardiovascular causes and needing antiplatelet and anticoagulant therapies. However, TP is not only a numeric parameter, but mostly a dynamic condition affected by the patients' underlying disorders and concomitant treatments. Platelets are important players in the hemostatic process, taking part to both primary and secondary hemostasis. Although both TP and antithrombotic treatment contribute to the risk of bleeding, the complexity of the pathogenesis of bleeding events makes it difficult to predict them accurately simply based on these two parameters...
December 5, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27917497/acquired-factor-xiii-inhibitor-associated-with-mantle-cell-lymphoma
#12
Christian P Nixon, Elizabeth H Prsic, Christine A Guertin, Ryan L Stevenson, Joseph D Sweeney
BACKGROUND: Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management. CASE REPORT: A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma...
December 4, 2016: Transfusion
https://www.readbyqxmd.com/read/27913547/new-treatment-approaches-to-von-willebrand-disease
#13
Michelle Lavin, James S O'Donnell
von Willebrand disease (VWD) is the commonest inherited bleeding disorder and results from either a quantitative or qualitative deficiency in the plasma glycoprotein von Willebrand factor (VWF). Recent large cohort studies have significantly enhanced our understanding of the molecular mechanisms involved in the pathogenesis of VWD. In contrast, however, there have been relatively few advances in the therapeutic options available for the treatment of bleeding in patients with VWD. Established treatment options include tranexamic acid, 1-deamino-8-d-arginine vasopressin (DDAVP), and plasma-derived VWF concentrates...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#14
Flora Peyvandi, Marzia Menegatti
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K-dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913535/reversing-vitamin-k-antagonists-making-the-old-new-again
#15
Sabine Eichinger
Vitamin K antagonists (VKAs) are commonly used for the prevention and treatment of thrombotic disorders. The response to VKAs is highly variable due to their specific interaction with the vitamin K cycle, and hence interference with hepatic synthesis of vitamin K-dependent coagulation factors. Monitoring the anticoagulant effect of VKAs by assessing the patient's international normalized ratio (INR) is essential because complications are closely related to the intensity of anticoagulation. Treatment with VKAs contains a substantial risk of bleeding with a high case fatality rate...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913488/platelets-in-liver-and-renal-disease
#16
Michele P Lambert
This review will discuss how 2 common and morbid conditions, renal disease and liver disease, alter platelet number and function. It will review the impact of thrombocytopenia on bleeding complications in patients with these disorders and whether the low platelet count actually correlates with bleeding risk. Emerging data also suggest that platelets are much more than bystanders in both renal and liver disease, but instead play an active role in the pathobiology of these disorders. This review will briefly cover the emerging information on novel roles of platelets in the biology of renal and liver disease...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913486/heavy-menstrual-bleeding-work-up-and-management
#17
Andra H James
Heavy menstrual bleeding (HMB), which is the preferred term for menorrhagia, affects ∼90% of women with an underlying bleeding disorder and ∼70% of women on anticoagulation. HMB can be predicted on the basis of clots of ≥1 inch diameter, low ferritin, and "flooding" (a change of pad or tampon more frequently than hourly). The goal of the work-up is to determine whether there is a uterine/endometrial cause, a disorder of ovulation, or a disorder of coagulation. HMB manifest by flooding and/or prolonged menses, or HMB accompanied by a personal or family history of bleeding is very suggestive of a bleeding disorder and should prompt a referral to a hematologist...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913485/congenital-and-acquired-bleeding-disorders-in-pregnancy
#18
Terry B Gernsheimer
Obstetrical hematology represents challenges not only for the patient, but also for her progeny. In particular, bleeding disorders, both congenital and acquired, not only present problems both for delivery and in the immediate postpartum period, but also may have significant implications for the fetus and neonate. Women with congenital bleeding disorders or who are carriers of X-linked or autosomal disorders should be counseled prior to conception so that pregnancy can be safely undertaken with careful preparation...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913478/thrombosis-in-the-setting-of-obesity-or-inflammatory-bowel-disease
#19
Steven R Lentz
Obesity and inflammatory bowel disease (IBD) are systemic inflammatory disorders that predispose to arterial and venous thrombosis through similar prothrombotic mechanisms. Obesity and IBD are chronic risk factors that lead to a persistently elevated risk of thrombosis, although the thrombotic risk with IBD appears to wax and wane with disease severity. Because of the lack of high-quality evidence to guide management decisions, approaches to the prevention and treatment of thrombosis in patients with obesity or IBD are based on extrapolation from general guidelines for antithrombotic therapy...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27908513/small-bowel-imaging-computed-tomography-enterography-magnetic-resonance-enterography-angiography-and-nuclear-medicine
#20
REVIEW
Jeff L Fidler, Ajit H Goenka, Chad J Fleming, James C Andrews
Radiology examinations play a major role in the diagnosis, management, and surveillance of small bowel diseases and are complementary to endoscopic techniques. Computed tomography enterography and magnetic resonance enterography are the cross-sectional imaging studies of choice for many small bowel diseases. Angiography still plays an important role for catheter-directed therapies. With the emergence of hybrid imaging techniques, radionuclide imaging has shown promise for the evaluation of small bowel bleeding and Crohn disease and may play a larger role in the future...
January 2017: Gastrointestinal Endoscopy Clinics of North America
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