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https://www.readbyqxmd.com/read/29792748/portal-vein-thrombosis-imaging-the-spectrum-of-disease-with-an-emphasis-on-mri-features
#1
Reena C Jha, Satinderpal Singh Khera, Amit Dinesh Kalaria
OBJECTIVE: The purpose of this article is to review the classic and uncommon imaging findings of portal vein thrombosis (PVT) (acute, chronic, congenital, and septic thrombus) as visualized on multiple modalities, with an emphasis on MRI findings. Additional aims are to understand the imaging of obliterative portal venopathy and its clinical significance, appreciate morphologic changes of the biliary system that may accompany PVT, and recognize changes in liver enhancement patterns seen with PVT related to the hepatic arterial buffer response...
May 24, 2018: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/29790625/managing-paediatric-bleeding-disorder-patients-undergoing-surgical-procedures-leveraging-the-electronic-medical-record
#2
C Tarango, D Dahale, N Dodson, A Otte, P J Schoettker
INTRODUCTION: Individuals with bleeding disorders have a high risk of bleeding complications with surgical procedures. Careful planning and management of peri-operative treatment is vital for their safety. Yet, inter-provider communication and communication between patients/families and providers is not reliable. AIM: Our haemophilia treatment centre (HTC) created a care gap report that used the electronic medical record to inform our team when patients with bleeding disorders were scheduled for procedures...
May 23, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29787683/micrornas-as-potential-regulators-of-platelet-function-and-bleeding-diatheses
#3
Ascensión M De Los Reyes-García, Ana B Arroyo, Raúl Teruel-Montoya, Vicente Vicente, María L Lozano, Rocío González-Conejero, Constantino Martínez
Although a growing number of studies suggest that microRNAs (miRNAs) play a relevant role in platelet biology, their implications in bleeding diatheses are starting to be investigated. Indeed, several studies have shown that alterations in the intracellular levels of highly expressed platelet miRNAs provoke a thrombotic phenotype. On the other hand, primary immune thrombocytopenia (ITP), which is considered the hallmark of acquired bleeding disorders, has been recently associated with altered levels of miRNAs in peripheral blood mononuclear cells, plasma, and platelets...
May 22, 2018: Platelets
https://www.readbyqxmd.com/read/29787319/usefulness-of-multiple-electrode-aggregometry-as-a-screening-tool-for-bleeding-disorders-in-a-pediatric-hospital
#4
Thorsten Haas, Melissa M Cushing, Stephanie Varga, Séverine Gilloz, Markus Schmugge
Platelet function testing is a cornerstone in the diagnostic investigation of patients with a bleeding history. Multiple electrode aggregometry (MEA) has been shown to detect von Willebrand disease (VWD), platelet function disorders, and drug-induced bleeding disorders. However, there are few studies supporting its successful use in children. We have implemented and used MEA over 3 years in our hemostasis laboratory in order to study its usefulness to supplement and expedite diagnosis. This is a retrospective, single-center, cohort study of 109 hospitalized children who underwent a laboratory investigation of hemostasis and either had a reported bleeding history or an abnormal bleeding episode...
May 22, 2018: Platelets
https://www.readbyqxmd.com/read/29786011/nontraumatic-subperiosteal-orbital-hemorrhage-following-upper-gastrointestinal-endoscopy
#5
Swaranjali S Gore, Neha Rathi, Amol Y Ganvir, Nayana A Potdar, Monisha K Apte, Trupti R Marathe, Akshay Gopinathan Nair, Chhaya A Shinde
Subperiosteal hemorrhages are typically the result of blunt orbital or facial trauma. Nontraumatic subperiosteal hemorrhages are uncommon and are usually attributed to increase in central venous pressure and bleeding disorders. Here, we report the case of a 38-year-old female who underwent an upper gastrointestinal (GI) endoscopy and developed bilateral nontraumatic subperiosteal hemorrhages that resolved with conservative treatment. Here, we discuss the source of bleeding and the mechanisms for the occurrence of orbital subperiosteal bleeds...
June 2018: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29785254/low-rates-of-patient-reported-outcome-claims-for-orphan-drugs-approved-by-the-us-food-and-drug-administration
#6
Szymon Jarosławski, Pascal Auquier, Borislav Borissov, Claude Dussart, Mondher Toumi
Background : Claims included in package inserts (PIs) for medicinal products approved by the US Food and Drug Administration (FDA) constitute the regulatory definition of drugs' benefits and risks. Objective : We sought to assess the usage of patient-reported outcome (PRO) claims in a comprehensive set of US FDA orphan drug approvals dated between 1/1/2012 and 31/12/2016, and characterize them. Study design : Orphan drug approval documentation was obtained from the US FDA website. Drug Package Inserts (PI) were analyzed to extract information on PRO-related language...
2018: Journal of Market Access & Health Policy
https://www.readbyqxmd.com/read/29782633/macrothrombocytopenia-renal-dysfunction-and-nephrotic-syndrome-in-a-young-male-patient-a-case-report-of-myh9-related-disease
#7
Gabriela Sevignani, Giovana Memari Pavanelli, Sibele Sauzem Milano, Bianca Ramos Ferronato, Maria Aparecida Pachaly, Hae Ii Cheong, Mauricio de Carvalho, Fellype Carvalho Barreto
MYH9-related disease is an autosomal dominant disorder caused by mutations of the MYH9 gene, which encodes the non-muscle myosin heavy chain IIA on chromosome 22q12. It is characterized by congenital macrothrombocytopenia, bleeding tendency, hearing loss, and cataracts. Nephropathy occurs in approximately 30% of MYH9-related disease in a male patient carrier of a de novo missense mutation in exon 1 of the MYH9 gene [c.287C > T; p.Ser(TCG)96(TTG)Leu]. He presented all phenotypic manifestations of the disease, but cataracts...
May 17, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29780794/pediatric-scurvy-when-contemporary-eating-habits-bring-back-the-past
#8
Alice Brambilla, Cristina Pizza, Donatella Lasagni, Lucia Lachina, Massimo Resti, Sandra Trapani
Vitamin C deficiency is anecdotal in developed countries, mainly associated with underling clinical morbidities as autism or neurological impairment. Chronic insufficient dietary supply is responsible for vascular fragility and impaired bone formation, resulting in gingival bleeding, petechial lesions, articular and bone pain or limb swelling. Children may present anorexia, irritability, failure to thrive, limping or refusal to walk. Accordingly, pediatric scurvy is frequently misdiagnosed with osteomyelitis, septic arthritis, bone and soft tissue tumor, leukemia, bleeding disorders, and rheumatologic conditions...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29776906/blood-induced-bone-loss-in-murine-hemophilic-arthropathy-is-prevented-by-blocking-the-irhom2-adam17-tnf%C3%AE-pathway
#9
Coline Haxaire, Narine Hakobyan, Tania Pannellini, Camila Carballo, David McIlwain, Tak W Mak, Scott Rodeo, Suchitra Acharya, Daniel Li, Jackie Szymonifka, Xiangqian Song, Sébastien Monette, Alok Srivastava, Jane E Salmon, Carl P Blobel
Hemophilic arthropathy (HA) is a debilitating degenerative joint disease that is a major manifestation of the bleeding disorder Hemophilia A. HA typically begins with hemophilic synovitis (HS) that resembles inflammatory arthritides such as rheumatoid arthritis (RA) and frequently results in bone loss in patients. A major cause of RA is inappropriate release of the pro-inflammatory cytokine tumor necrosis factor α (TNFα) by the TNFα convertase (TACE, also referred to as ADAM17) and its regulator, iRhom2...
May 18, 2018: Blood
https://www.readbyqxmd.com/read/29776849/immune-factors-in-deep-vein-thrombosis-initiation
#10
REVIEW
Ivan Budnik, Alexander Brill
Deep vein thrombosis (DVT) is a major origin of morbidity and mortality. While DVT has long been considered as blood coagulation disorder, several recent lines of evidence demonstrate that immune cells and inflammatory processes are involved in DVT initiation. Here, we discuss these mechanisms, in particular, the role of immune cells in endothelial activation, and the immune cascades leading to expression of adhesion receptors on endothelial cells. We analyze the specific recruitment and functional roles of different immune cells, such as mast cells and leukocytes, in DVT...
May 15, 2018: Trends in Immunology
https://www.readbyqxmd.com/read/29773783/cholesteryl-ester-storage-disease-fatal-outcome-without-causal-therapy-in-a-female-patient-with-the-preventable-sequelae-of-progressive-liver-disease-after-many-years-of-mild-symptoms
#11
Ali Canbay, Meike N Müller, Stathis Philippou, Guido Gerken, Andreas Tromm
BACKGROUND Cholesteryl ester storage disease (CESD), also known as lysosomal acid lipase deficiency (LAL-D), is a rare autosomal-recessive inheritable lysosomal storage disease. Since 2015, a causal treatment with sebelipase alfa, which replaces the missing LAL enzyme, has been approved. We report a fatal course of LAL-D in a female patient. CASE REPORT In 1979, CESD was first diagnosed in a 13-year-old female with marked hepatomegaly. At that time, no specific treatment for CESD was available and the spontaneous course of the disease had to be awaited...
May 18, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29772593/-coagulation-monitoring-and-bleeding-management-in-cardiac-surgery
#12
Berthold Bein, Robert Schiewe
The transfusion of allogeneic blood products is associated with increased morbidity and mortality. An impaired hemostasis is frequently found in patients undergoing cardiac surgery and may in turn cause bleeding and transfusions. A goal directed coagulation management addressing the often complex coagulation disorders needs sophisticated diagnostics. This may improve both patients' outcome and costs. Recent data suggest that coagulation management based on a rational algorithm is more effective than traditional therapy based on conventional laboratory variables such as PT and INR...
May 2018: Anästhesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie: AINS
https://www.readbyqxmd.com/read/29770858/-management-of-anticoagulants-in-ophthalmic-surgery-a-survey-among-ophthalmic-surgeons-in-germany
#13
N Feltgen, B Mele, T Dietlein, C Erb, A Eckstein, A Hager, A Heiligenhaus, H Helbig, H Hoerauf, E Hoffmann, D Pauleikhoff, M Schittkowski, B Seitz, C Sucker, S Suffo, U Schaudig, F Tost, S Thurau, P Walter, J Koscielny
INTRODUCTION: As our population ages and comorbidities rise, ophthalmic surgeons are increasingly faced with patients on anticoagulant therapy or with clotting disorders. The ophthalmic surgeon has to weigh the perioperative risk of haemorrhage when anticoagulation continues against the risk of thromboembolism caused by discontinuation or changing the patient's medication (bridging, switching, cessation). There are currently no guidelines or recommendations. METHODS: A survey was sent to the DOG (German Ophthalmologic Society) divisions and associated surgical organizations to determine the status quo...
May 16, 2018: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/29769259/novel-therapeutics-for-hemophilia-and-other-bleeding-disorders
#14
Michael U Callaghan, Robert Sidonio, Steven W Pipe
Hemophilia and von Willebrand disease are the most common congenital bleeding disorders. Treatment for these disorders has focused on replacement of the missing coagulation factor to prevent or treat bleeding. New technologies and insights into hemostasis have driven the development of many promising new therapies for hemophilia and von Willebrand disease. Emerging bypass agents including zymogen-like factor IXa and Xa molecules are in development and a bispecific antibody, emicizumab, demonstrated efficacy in a phase III trial in people with hemophilia A and inhibitors...
May 16, 2018: Blood
https://www.readbyqxmd.com/read/29766740/factors-predictive-of-postoperative-acute-respiratory-failure-following-inpatient-sinus-surgery
#15
Brittany N Burton, Sapideh Gilani, Matthew W Swisher, Richard D Urman, Ulrich H Schmidt, Rodney A Gabriel
OBJECTIVE: The impact of perioperative risk factors on outcomes following outpatient sinus surgery is well defined; however, risk factors and outcomes following inpatient surgery remain poorly understood. We aimed to define risk factors of postoperative acute respiratory failure following inpatient sinus surgery. METHODS: Utilizing data from the Nationwide Inpatient Sample Database from the years 2010 to 2014, we identified patients (≥18 years of age) with an Internal Classification of Disease, Ninth Revision, Clinical Modification (ICD-9-CM) procedure code of sinus surgery...
May 1, 2018: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/29765293/recurrent-bleedings-in-newborn-a-factor-vii-deficiency-case-report
#16
Kim Cattivelli, Cristina Distefano, Lorenza Bonetti, Sophie Testa, Simona Maria Siboni, Alessandro Plebani, Carlo Poggiani
Background: Major hemorrhages in newborns can be caused by several conditions, and knowledge of the differential diagnosis is essential in order to ensure prompt recognition and appropriate treatment. Case Report: We describe the case of a male newborn experiencing recurrent hemorrhages from the first days of life. Laboratory findings showed normal platelet count, hepatic function, and C-reactive protein. Coagulation tests detected an isolated prothrombin time (PT) prolongation and severe factor VII (FVII) deficiency...
April 2018: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/29762905/pharmacokinetics-and-the-transition-to-extended-half-life-factor-concentrates-communication-from-the-ssc-of-the-isth
#17
Margaret V Ragni, Stacy E Croteau, Massimo Morfini, Marjon H Cnossen, Alfonso Iorio
Extended half-life proteins (EHL) are increasingly used in clinical practice, but there is no standardized approach to sampling, interpretation, and implementation of pharmacokinetics (PK) data to maximize treatment benefit. The goal of EHL treatment is to attain a trough level sufficient to protect against spontaneous bleeds and reduce infusion frequency and limitations on individual activity and lifestyle. Performing classical PK assessments requires multiple blood samples which is burdensome for patients and providers...
May 15, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29762845/peritoneal-dialysis-effectively-removes-toxic-substances-and-improves-liver-functions-of-liver-failure-patients
#18
W-X Zhao, X-M Liu, C-M Yu, H Xu, J-R Dai, H-Y Chen, L Li, F Chen, Y-L Ou, Z-K Zhao
OBJECTIVE: Liver failure (LF) is a clinically complex disorder that characterizes with hepatic dysfunction. This study aimed at observing the therapeutic effects of peritoneal dialysis on liver function in LF patients. PATIENTS AND METHODS: This study involves 62 patients diagnosed as LF hospitalized from February 2005 to December 2016. The 62 LF patients were randomly divided into 3 groups, including artificial liver applying plasma exchange group (PE, n = 28), peritoneal dialysis group (PD, n = 22), and conservative treatment group (CT, n=12)...
April 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29760930/prevalence-of-thrombocytopenia-before-and-after-initiation-of-haart-among-hiv-infected-patients-at-black-lion-specialized-hospital-addis-ababa-ethiopia-a-cross-sectional-study
#19
Gashaw Garedew Woldeamanuel, Diresibachew Haile Wondimu
Background: Hematological abnormalities are common in HIV positive patients. Of these, thrombocytopenia is a known complication which has been associated with a variety of bleeding disorders. However, its magnitude and related factors have not been well-characterized in the era of highly active antiretroviral therapy (HAART) in Ethiopia. Therefore, this study aimed to assess the prevalence of thrombocytopenia before and after initiation of HAART among HIV positive patients attending Black Lion Specialized Hospital, Addis Ababa, Ethiopia...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29760702/emerging-concepts-in-immune-thrombocytopenia
#20
REVIEW
Maurice Swinkels, Maaike Rijkers, Jan Voorberg, Gestur Vidarsson, Frank W G Leebeek, A J Gerard Jansen
Immune thrombocytopenia (ITP) is an autoimmune disease defined by low platelet counts which presents with an increased bleeding risk. Several genetic risk factors (e.g., polymorphisms in immunity-related genes) predispose to ITP. Autoantibodies and cytotoxic CD8+ T cells (Tc) mediate the anti-platelet response leading to thrombocytopenia. Both effector arms enhance platelet clearance through phagocytosis by splenic macrophages or dendritic cells and by induction of apoptosis. Meanwhile, platelet production is inhibited by CD8+ Tc targeting megakaryocytes in the bone marrow...
2018: Frontiers in Immunology
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