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Bleeding disorders

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https://www.readbyqxmd.com/read/28328824/early-use-of-noninvasive-techniques-for-clearing-respiratory-secretions-during-noninvasive-positive-pressure-ventilation-in-patients-with-acute-exacerbation-of-chronic-obstructive-pulmonary-disease-and-hypercapnic-encephalopathy-a-prospective-cohort-study
#1
Jinrong Wang, Zhaobo Cui, Shuhong Liu, Xiuling Gao, Pan Gao, Yi Shi, Shufen Guo, Peipei Li
Noninvasive positive-pressure ventilation (NPPV) might be superior to conventional mechanical ventilation (CMV) in patients with acute exacerbations of chronic obstructive pulmonary disease (AECOPDs). Inefficient clearance of respiratory secretions provokes NPPV failure in patients with hypercapnic encephalopathy (HE). This study compared CMV and NPPV combined with a noninvasive strategy for clearing secretions in HE and AECOPD patients.The present study is a prospective cohort study of AECOPD and HE patients enrolled between October 2013 and August 2015 in a critical care unit of a major university teaching hospital in China...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28328595/hemorrhage-and-infarction-of-the-conjunctiva-and-orbit-in-essential-thrombocythemia
#2
Jaehoon Na, Soo Youn Choi, Sehyun Baek, Hwa Lee
An 82-year-old woman who had a history of essential thrombocythemia presented with ocular pain, bleeding, and decreased visual acuity of the left eye. Orbital computed tomography revealed a relatively well-defined homogenous mass-like lesion in the left subconjunctival and intraconal space. Conjunctival biopsy showed acute inflammation with necrosis, vascular ectasia with thrombosis and hemorrhage. After the treatment with hydroxyurea and anagrelide, her symptom and lesion were markedly improved. Hematologic disorders like essential thrombocythemia should be considered in patients with severe spontaneous bleeding around the eye...
March 21, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28327855/-hemorrhagic-erucism-due-to-lonomia-spp-in-a-girl
#3
Walter Peña-Vásquez, Héctor Vásquez-Paz, Rubén Vásquez-Becerra, Alfredo Chiappe-Gonzalez, Marcos Ñavincopa-Flores, Eduardo Ticona-Chávez
Accidents caused by urticating or poisonous setae from lepidoptera caterpillars are known as erucism. These accidents are produced by accidental contact, especially in children, with bristles on the insect's body surface, connected to venom glands. Symptoms may be local or systemic, with deadly clinical presentations. The accident caused by Lonomia spp. caterpillars can trigger bleeding disorders, which is considered the most severe type of erucism. The case of a 5-year-old girl is reported. She was from the town of Villarondos, in the Peruvian Amazon, department of Huánuco, who accidentally knelt down on caterpillar bristles, and subsequently experienced hemolytic anemia, plateletopenia, and coagulation disorder...
October 2016: Revista Peruana de Medicina Experimental y Salud Pública
https://www.readbyqxmd.com/read/28326424/total-knee-arthroplasty-in-a-patient-with-hypofibrinogenemia
#4
Christopher R Nacca, Kalpit N Shah, Jeremy N Truntzer, Lee E Rubin
Patients with afibrinogenemia or hypofibrinogenemia present a unique challenge to the arthroplasty surgeon as fibrinogen is a key contributor to hemostasis. Patients with these disorders are known to have a higher risk for postsurgical bleeding complications. We present the case of a patient with hypofibrinogenemia who underwent an elective total knee arthroplasty. Our colleagues in hematology-oncology guided us initially to achieve and maintain appropriate fibrinogen levels in the early perioperative period...
December 2016: Arthroplasty Today
https://www.readbyqxmd.com/read/28318926/effectiveness-of-tranexamic-acid-for-reducing-intraoperative-bleeding-in-palatoplasties-a-randomized-clinical-trial
#5
Guilherme C Arantes, Rui Manoel R Pereira, Daniela B de Melo, Nivaldo Alonso, Maria do Carmo M B Duarte
BACKGROUND: Few data are available regarding blood loss during cleft palate surgery. This study assessed the effect of using tranexamic acid in the reduction of intraoperative bleeding. METHODS: A double-blind, randomized clinical trial compared intraoperative bleeding and the incidence of oronasal fistulas after palatoplasties in a control group that was given placebo and an intervention group that was given 10 mg/kg tranexamic acid followed by a continuous infusion of 1 mg/kg/h of the same until the end of surgery...
December 30, 2016: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/28317488/association-between-periodontal-health-status-and-cognitive-abilities-the-role-of-cytokine-profile-and-systemic-inflammation
#6
Marta Sochocka, Maciej Sobczyński, Aleksandra Sender-Janeczek, Katarzyna Zwolińska, Olga Błachowicz, Tomasz Tomczyk, Marek Ziętek, Jerzy Leszek
BACKGROUND: Contemporary neurobiology, periodontal medicine, and immunology are now focusing on the relationship between chronic periodontitis and systemic diseases, that also include Alzheimer's disease (AD). However a causative relationship between dementia and periodontitis has yet to be confirmed. OBJECTIVE: The aim of the study was to determine whether periodontal health status and cognitive abilities are correlated with the relative changes in systemic measures of pro- and anti-inflammatory cytokines as a reflection of systemic inflammation...
March 16, 2017: Current Alzheimer Research
https://www.readbyqxmd.com/read/28316858/spontaneous-intramural-oesophageal-haematoma-in-a-patient-with-uncontrolled-hypertension-an-unusual-chest-pain-aetiology
#7
Samantha Cooray, Dionysios Dellaportas, Clifford Caruana, Andrew R Davies
Introduction. Spontaneous intramural oesophageal haematoma is a rare condition that usually occurs secondary to an acute or chronic coagulation disorder. The presenting complaint is often with retrosternal chest pain and most patients are initially investigated to exclude more common causes in the differential diagnosis, such as acute coronary syndromes. Severe life-threatening bleeding or perforation seldom, if ever, arises. Case Presentation. We present a case of spontaneous oesophageal haematoma which appears to have developed gradually in a 69-year-old female with uncontrolled hypertension and antiplatelet medication use...
2017: Case Reports in Surgery
https://www.readbyqxmd.com/read/28316091/2b-or-not-2b-a-prothrombotic-tendency-masquerading-as-a-bleeding-disorder
#8
Yvonne Brennan, Jennifer Curnow, Emmanuel J Favaloro
No abstract text is available yet for this article.
March 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28314138/depression-and-post-traumatic-stress-disorder-in-individuals-with-hereditary-hemorrhagic-telangiectasia-a-cross-sectional-survey
#9
Shruti Chaturvedi, Marianne Clancy, Nicole Schaefer, Olalekan Oluwole, Keith R McCrae
INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is characterized by frequent severe bleeding, particularly epistaxis, and life-threatening complications including stroke, brain abscess and heart failure. The psychological impact of HHT is not known. We conducted this cross sectional study to determine the prevalence of depression and post-traumatic stress disorder (PTSD) related to HHT. METHODS: A survey tool comprising demographic and clinical information and two validated self-administered questionnaires, the PTSD checklist for DSM-5 (PCL-5) and Beck Depression Inventory-II (BDI-II), was distributed to individuals with HHT...
March 9, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28306198/plasma-levels-of-plasminogen-activator-inhibitor-1-and-bleeding-phenotype-in-patients-with-von-willebrand-disease
#10
S Abdul, J Boender, J J M C Malfliet, J Eikenboom, K Fijn van Draat, E P Mauser-Bunschoten, K Meijer, J de Meris, B A P Laros-van Gorkom, J G van der Bom, F W G Leebeek, D C Rijken, S Uitte de Willige
INTRODUCTION: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients. AIM: To investigate whether plasminogen activator inhibitor-1 (PAI-1) level influences the variation in bleeding tendency in VWD patients...
March 17, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28303335/treatment-selection-for-urolithiasis-percutaneous-nephrolithomy-ureteroscopy-shock-wave-lithotripsy-and-active-monitoring
#11
Mahesh Desai, Yinghao Sun, Noor Buchholz, Andrew Fuller, Tadashi Matsuda, Brian Matlaga, Nicole Miller, Damien Bolton, Mohammad Alomar, Arvind Ganpule
Urolithiasis is a significant worldwide source of morbidity, constituting a common urological disease that affects between 10 and 15% of the world population. Recent technological and surgical advances have replaced the need for open surgery with less invasive procedures. The factors which determine the indications for percutaneous nephrolithotomy include stone factors (stone size, stone composition, and stone location), patient factors (habitus and renal anomalies), and failure of other treatment modalities (ESWL and flexible ureteroscopy)...
March 16, 2017: World Journal of Urology
https://www.readbyqxmd.com/read/28301587/the-duplication-mutation-of-quebec-platelet-disorder-dysregulates-plau-but-not-c10orf55-selectively-increasing-production-of-normal-plau-transcripts-by-megakaryocytes-but-not-granulocytes
#12
Catherine P M Hayward, Minggao Liang, Subia Tasneem, Asim Soomro, John S Waye, Andrew D Paterson, Georges E Rivard, Michael D Wilson
Quebec Platelet disorder (QPD) is a unique bleeding disorder that markedly increases urokinase plasminogen activator (uPA) in megakaryocytes and platelets but not in plasma or urine. The cause is tandem duplication of a 78 kb region of chromosome 10 containing PLAU (the uPA gene) and C10orf55, a gene of unknown function. QPD increases uPA in platelets and megakaryocytes >100 fold, far more than expected for a gene duplication. To investigate the tissue-specific effect that PLAU duplication has on gene expression and transcript structure in QPD, we tested if QPD leads to: 1) overexpression of normal or unique PLAU transcripts; 2) increased uPA in leukocytes; 3) altered levels of C10orf55 mRNA and/or protein in megakaryocytes and leukocytes; and 4) global changes in megakaryocyte gene expression...
2017: PloS One
https://www.readbyqxmd.com/read/28300361/zika-virus-and-inherited-bleeding-disorders
#13
EDITORIAL
S S Medina, M C Ozelo
No abstract text is available yet for this article.
March 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28299631/clinical-characteristics-and-outcomes-of-acquired-hemophilia-a-experience-at-a-single-center-in-japan
#14
Yoshiyuki Ogawa, Kunio Yanagisawa, Hideki Uchiumi, Takuma Ishizaki, Takeki Mitsui, Fumito Gouda, Masahiro Ieko, Akitada Ichinose, Yoshihisa Nojima, Hiroshi Handa
Acquired hemophilia A (AHA), which is caused by autoantibodies against coagulation factor VIII (FVIII) is a rare, life-threatening bleeding disorder, the incidence of which appears to be increasing in Japan as the population ages. However, the clinical characteristics, treatment, and outcomes of AHA remain difficult to establish due to the rarity of this disease. We retrospectively analyzed data from 25 patients (median age 73 years; range 24-92 years; male n = 15) diagnosed with AHA between 1999 and 2015 at Gunma University Hospital...
March 15, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28299211/epistaxis-as-a-common-presenting-symptom-of-glanzmann-s-thrombasthenia-a-rare-qualitative-platelet-disorder-illustrative-case-examples
#15
Michael Recht, Meera Chitlur, Derek Lam, Syana Sarnaik, Madhvi Rajpurkar, David L Cooper, Sriya Gunawardena
Children often present to emergency departments (EDs) with uncontrollable nose bleeding. Although usually due to benign etiologies, epistaxis may be the presenting symptom of an inherited bleeding disorder. Whereas most bleeding disorders are detected through standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. Here we present two case reports and review diagnostic and management challenges of platelet function disorders with a focus on Glanzmann's thrombasthenia (GT)...
2017: Case Reports in Emergency Medicine
https://www.readbyqxmd.com/read/28298971/oral-health-and-medical-conditions-among-amish-children
#16
Masahiro Heima, Marc-Allen Harrison, Peter Milgrom
BACKGROUND: The Amish are a growing population who live a traditional, rural way of life, which makes them less accepting of modernism. Most Amish live in poverty and are detached from modern health care. In addition, the recent change of their lifestyle has been reported, such as consuming a nontraditional diet and the usage of electronic devices. As a result, their lifestyle change may have impacted their oral health. However, since only a single report about oral health among Amish children has been published approximately three decades ago it has not yet been updated...
March 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28296950/cellular-and-molecular-defects-in-a-patient-with-hermansky-pudlak-syndrome-type-5
#17
Joshi Stephen, Tadafumi Yokoyama, Nathanial J Tolman, Kevin J O'Brien, Elena-Raluca Nicoli, Brian P Brooks, Laryssa Huryn, Steven A Titus, David R Adams, Dong Chen, William A Gahl, Bernadette R Gochuico, May Christine V Malicdan
Hermansky-Pudlak syndrome (HPS) is a heterogeneous group of genetic disorders typically manifesting with tyrosinase-positive oculocutaneous albinism, bleeding diathesis, and pulmonary fibrosis, in some subtypes. Most HPS subtypes are associated with defects in Biogenesis of Lysosome-related Organelle Complexes (BLOCs), which are groups of proteins that function together in the formation and/or trafficking of lysosomal-related endosomal compartments. BLOC-2, for example, consists of the proteins HPS3, HPS5, and HPS6...
2017: PloS One
https://www.readbyqxmd.com/read/28296755/inflammatory-bowel-diseases-a-burden-in-pediatrics-case-series-and-a-review-of-the-literature
#18
Cristina Oana Mărginean, Lorena Elena Meliţ, Simona Mocanu, Maria Oana Mărginean
INTRODUCTION: Inflammatory bowel disease is a chronic condition of the gastrointestinal tract, comprising mainly Crohn disease (CD) and ulcerative colitis (UC). Both of them are frequently encountered in children, being multifactorial conditions, with an unclear etiology. PATIENTS CONCERNS: We present 4 cases of inflammatory bowel disease (IBD) in children in order to underline the variable evolution depending on the patient's particularities. DIAGNOSIS, INTERVENTIONS AND OUTCOMES: The first case, a 13-year-old male patient, with a history of Henoch-Schonlein purpura, was admitted for rectal bleeding and weight loss, with normal laboratory parameters...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28296243/measurement-of-platelet-aggregation-independent-of-patient-platelet-count-a-flow-cytometric-approach
#19
Pernille Just Vinholt, Henrik Frederiksen, Anne-Mette Hvas, Ulrik Sprogøe, Christian Nielsen
BACKGROUND: Methods for testing platelet aggregation in thrombocytopenia are missing. OBJECTIVE: To establish a flow cytometric test of in vitro platelet aggregation independent of the patient platelet count and examined the association of aggregation with bleeding history in thrombocytopenic patients. PATIENTS/METHODS: We established a flow cytometric assay of platelet aggregation and measured samples from healthy individuals pre-incubated with antiplatelet drugs and samples from two patients with inherited platelet disorders...
March 15, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28295573/therapeutic-plasma-exchange-for-perioperative-management-of-patients-with-congenital-factor-xi-deficiency
#20
Mohamed S Alsammak, Aneel A Ashrani, Jeffrey L Winters, Rajiv K Pruthi
BACKGROUND: Factor XI (FXI) deficiency (hemophilia C [HEM-C]) is a bleeding disorder with unpredictable severity that correlates poorly with FXI coagulation activity (FXI:C). It poses a perioperative hemostatic management challenge. For US patients with severe disease, fresh frozen plasma (FFP) or, in current use, thawed plasma is the most readily available option but comes with risk of volume overload. We report our experience of using therapeutic plasma exchange (TPE) as an alternative perioperative management strategy...
March 15, 2017: Journal of Clinical Apheresis
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