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https://www.readbyqxmd.com/read/29159724/turkish-case-of-ethylmalonic-encephalopathy-misdiagnosed-as-short-chain-acyl-coa-dehydrogenase-deficiency
#1
Fatma Derya Bulut, Deniz Kör, Berna Şeker-Yılmaz, Gülen Gül-Mert, Sebile Kılavuz, Neslihan Önenli-Mungan
Ethylmalonic encephalopathy is a very rare autosomal recessively inherited inborn error of metabolism; characterized by encephalopathy, recurrent petechiae without bleeding diathesis, chronic diarrhea, and orthostatic acrocyanosis. Here, we describe a case of ethylmalonic encephalopathy with late onset neurologic symptoms and a confusing family history of two deceased brothers with the wrong suspicion of short chain acyl-CoA dehydrogenase deficiency.
November 20, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/29145678/-thrombocytosis-and-thrombocytopenia-background-and-clinical-relevance
#2
Kai Wille, Parvis Sadjadian, Martin Griesshammer
Due to the central role of platelets in hemostasis, the clinical relevance of quantitative changes in platelet counts (< 150 G/l or > 450 G/l) may be significant. Thrombopoesis (= production of platelets) occurs in the bone marrow, and the hormone thrombopoetin takes control on its regulation.In thrombocytosis, primary causes have to be distinguished from the far more common reactive (= secondary) reasons. The most important form of primary thrombocytosis occurs in myeloproliferative neoplasms especially in essential thrombocythemia (ET)...
November 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29125443/neurosurgical-management-in-children-with-bleeding-diathesis-auditing-neurological-outcome
#3
Zaitun Zakaria, Chandrasekaran Kaliaperumal, Darach Crimmins, John Caird
OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29090612/hermansky-pudlak-syndrome-subtype-5-hps-5-novel-mutation-in-a-65-year-old-with-oculocutaneous-hypopigmentation-and-mild-bleeding-diathesis-the-importance-of-recognizing-a-subtle-phenotype
#4
Juliana Perez Botero, Dong Chen, Julie A Majerus, Lea M Coon, Rong He, Deepti M Warad, Rajiv K Pruthi, William L Nichols
Hermansky-Pudlak syndrome (HPS) - characterized by the distinct clinical phenotypes of both oculocutaneous albinism and mild bleeding diathesis-is caused by mutations in genes that have crucial roles in the assembly of cellular organelles (skin melanosomes, platelet delta [dense] granules, lung lamellar bodies, and cytotoxic T-cell lymphocyte granules). Immunodeficiency, pulmonary fibrosis and granulomatous colitis are associated with some, but not all subtypes of HPS, with varying degrees of clinical severity...
November 1, 2017: Platelets
https://www.readbyqxmd.com/read/29048211/ureteroscopy-in-patients-with-bleeding-diathesis-a-systematic-review-and-meta-analysis-of-the-literature
#5
Ala'a Sharaf, Tarik Amer, Bhaskar Somani, Omar M Aboumarzouk
Ureteroscopy (URS) is the most common surgical treatment of urolithiasis and can be problematic in patients with a bleeding diathesis. The intent of this review is to systematically review the literature to assess the safety and efficacy of ureteroscopic procedures in these groups of patients. Methods The systematic review was performed according to the Cochrane diagnostic accuracy reviews guidelines. The search strategy was conducted to perform a comprehensive database search (1990-2017). A cumulative analysis was done and where applicable a comparative analysis between bleeding diathesis patients and those without...
October 19, 2017: Journal of Endourology
https://www.readbyqxmd.com/read/29044644/hermansky-pudlak-syndrome-report-of-two-patients-with-updated-genetic-classification-and-management-recommendations
#6
REVIEW
Manuela Loredana Asztalos, Kristian T Schafernak, Jayla Gray, Adam Berry, Amy S Paller, Anthony J Mancini
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder caused by mutations in one of nine genes involved in the packaging and formation of specialized lysosomes, including melanosomes and platelet-dense granules. The cardinal features are pigmentary dilution, bleeding diathesis, and accumulation of ceroid-like material in reticuloendothelial cells. Pulmonary fibrosis induced by tissue damage is seen in the most severe forms, and one subtype is characterized by immunodeficiency. We describe two patients with HPS type 1 and review the updated gene-based classification, clinical features, and recommendations for evaluation and follow-up...
October 16, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29027774/rational-approach-to-transfusion-in-liver-transplantation
#7
Fuat H Saner, Lasitha Abeysundara, Matthias Hartmann, Susan V Mallett
For over 50 years patients with liver cirrhosis were considered to be at markedly increased risk of bleeding. This dogma was seemingly supported by abnormalities in standard laboratory tests (SLTs), such as the prothrombin time, that were interpreted as indicating a bleeding diathesis. However, publications from the last decade have revealed SLTs to be poor predictors of bleeding and it is now understood that stable patients with cirrhosis have a rebalanced haemostatic system and preserved thrombin generation...
October 12, 2017: Minerva Anestesiologica
https://www.readbyqxmd.com/read/29026981/a-clinical-scoring-system-to-predict-the-development-of-intraventricular-hemorrhage-ivh-in-premature-infants
#8
Yesim Coskun, Semra Isik, Tevfik Bayram, Kamran Urgun, Sibel Sakarya, Ipek Akman
OBJECTıVE: The aim of this study is to develop a scoring system for the prediction of intraventricular hemorrhage (IVH) in preterm infants in the first 7 days of life. METHODS: A prospective, clinical study was conducted in Bahcesehir University, Medical Park Goztepe Hospital Neonatal Intensive Care Unit, with the enrollment of 144 preterm infants with gestational age between 24 and 34 weeks. All preterms were followed up for IVH after birth until the 4th week of life. The demographic characteristics and clinical risk factors were noted...
October 12, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28951819/bleeding-diathesis-or-fabrication-munchausen-syndrome
#9
Syeda Naqvi, Raad Asadullah Khan, Chintan Rupareliya, Rida Hanif, Zeeshan Ali, Faiza Farooq
A case history of an 18-year-old female with a diagnosis of Munchausen syndrome is presented with a literature review of this rare syndrome. We present this case because of the young age and the patient's overwhelming response to cognitive behavioral therapy. We recommend collateral history taking, exclusion of all possible etiologies and detailed briefing of family members as it plays a vital role to reduce the mental and financial suffering of the patient.
June 12, 2017: Curēus
https://www.readbyqxmd.com/read/28934202/platelet-dysfunction-contributes-to-bleeding-complications-in-patients-with-probable-leptospirosis
#10
Rahajeng N Tunjungputri, Muhammad Hussein Gasem, Willemijn van der Does, Pandu H Sasongko, Bambang Isbandrio, Rolf T Urbanus, Philip G de Groot, Andre van der Ven, Quirijn de Mast
BACKGROUND: Severe leptospirosis is frequently complicated by a hemorrhagic diathesis, of which the pathogenesis is still largely unknown. Thrombocytopenia is common, but often not to the degree that spontaneous bleeding is expected. We hypothesized that the hemorrhagic complications are not only related to thrombocytopenia, but also to platelet dysfunction, and that increased binding of von Willebrand factor (VWF) to platelets is involved in both platelet dysfunction and increased platelet clearance...
September 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28930957/clot-dynamics-and-mortality-the-ma-r-ratio
#11
Stephanie A Savage, Ben L Zarzaur, Timothy H Pohlman, Brian L Brewer, Louis J Magnotti, Martin A Croce, Garrett H Lim, Ali C Martin
INTRODUCTION: The coagulopathy of trauma, illustrated by a short R-time, is common and well understood. The physiology behind this may be early thrombin burst with rapid clot formation. Rapid consumption of fibrinogen, however, may result in weak clot and substrate depletion, resulting in low MA. While these characteristics are interesting, utilizing thromboelastography (TEG) to identify those at risk of subsequent bleeding diathesis, especially in those who do not demonstrate early signs of physiologic derangement, is challenging...
October 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28928091/p2y12-antibody-inhibits-platelet-activity-and-protects-against-thrombogenesis
#12
Nicole R Hensch, Zubair A Karim, Joshua Pineda, Nicole Mercado, Fatima Z Alshbool, Fadi T Khasawneh
Given that platelet hyperactivity is known to give rise to thrombotic disorders, new and/or novel antiplatelet therapies are constantly being developed to add to, or to complement the current arsenal of agents. To this end, adenosine diphosphate (ADP) is an important platelet activator that acts by binding to the G-protein coupled P2Y1 and P2Y12 receptors. Although the contribution of the P2Y12 receptor to the genesis of thrombosis is well established, the parenteral arsenal of drugs targeting this receptor in clinical use is limited to cangrelor...
November 18, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28903884/successful-outcome-of-severe-intra-cerebral-bleeding-associated-with-acquired-factor-v-inhibition-utilization-of-multiple-therapeutic-agents
#13
Panagiotis Andreadis, Katerina Kafantari, Aleka Agapidou, Sofia Vakalopoulou, Efthymia Vlachaki
BACKGROUND: Acquired coagulation Factor inhibitors are antibodies that either inhibit the activity or increase the clearance of a clotting factor leading to an increased risk of bleeding. Most of the times, the disorder is attributed to Factor VIII inhibition (Acquired Hemophilia A), however other coagulation factors could also be implicated. CASE REPORT: We herein would like to report an interesting case of a patient who underwent Coronary Artery Bypass Graft and received antibiotic treatment after surgery with a third-generation cephalosporin...
September 13, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28892997/quantification-of-liver-fat-with-mdixon-magnetic-resonance-imaging-comparison-with-the-computed-tomography-and-the-biopsy
#14
Venkatraman Bhat, Sundararaman Velandai, Vikram Belliappa, Jeyeram Illayraja, Karthik Gadabana Halli, Gayathri Gopalakrishnan
INTRODUCTION: Accurate, non-invasive method of fat estimation is a valuable test for evaluation of diseases with abnormal hepatic fat. AIM: To determine the accuracy of mDixon MR technique in assessment of liver fat over CT and to correlate the CT and MRI findings with biopsy. MATERIALS AND METHODS: A prospective observational study was conducted at Imaging Services of Narayana Multispeciality Hospital between March 2011- December 2012. Thirty patients who attended the clinic for non-hepatic complaints were included in the study...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28861294/retrograde-intrarenal-surgery-for-renal-stones-part-2
#15
REVIEW
Özcan Kılıç, Murat Akand, Ben Van Cleynenbreugel
Retrograde intrarenal surgery (RIRS) has become an effective and safe treatment modality in the management of urinary system stone disease. Recent developments and innovations in the flexible ureterorenoscope and auxiliary equipment have made this procedure easier and more effective with increased success rates. RIRS can be used as a primary treatment in patients with renal stones smaller than 2 cm, prior unsuccessful shock wave lithotripsy, infundibular stenosis, renoureteral malformation, skeletal-muscular deformity, bleeding diathesis and obese patients...
September 2017: Turkish Journal of Urology
https://www.readbyqxmd.com/read/28840030/veno-arterial-extracorporeal-membrane-oxygenation-without-therapeutic-anticoagulation-for-intra-operative-cardiopulmonary-support-during-lung-transplantation
#16
Ankit Bharat, Malcolm M DeCamp
Cardiopulmonary support is frequently required during lung transplantation. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) has been recently shown to be an acceptable and, perhaps, safer alternative to cardiopulmonary bypass machine (CBM) for lung transplantation. However, therapeutic anticoagulation used during both these techniques can cause significant bleeding diathesis, especially in the setting of re-transplantation or when severe adhesions are encountered. Technological advances have reduced the thrombogenic potential of VA-ECMO circuits and unlike CBM, VA-ECMO does not include a reservoir containing a stagnant column of blood...
July 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28821556/dasatinib-reversibly-disrupts-endothelial-vascular-integrity-by-increasing-non-muscle-myosin-ii-contractility-in-a-rock-dependent-manner
#17
Anna Kreutzman, Beatriz Colom-Fernández, Ana Marcos Jiménez, Mette Ilander, Carlos Cuesta-Mateos, Yaiza Pérez-García, Cristina Delgado Arévalo, Oscar Brück, Henna Hakanen, Jani Saarela, Alvaro Ortega-Carrión, Ana de Rosendo, Alba Juanes-García, Juan Luis Steegmann, Satu Mustjoki, Miguel Vicente-Manzanares, Cecilia Muñoz-Calleja
Purpose: Dasatinib is a short-acting dual ABL/SRC family tyrosine kinase inhibitor (TKI), which is frequently used to treat chronic myeloid leukemia. Although very effective, patients taking dasatinib often display severe adverse effects, including pleural effusions and increased risk of bleeding primarily in the gastrointestinal tract. The actual causes of these side effects are currently undetermined. We hypothesize that endothelial cells (ECs) that line the inner walls of blood vessels and control the traffic to the underlying tissues might be involved...
August 18, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28820407/-evaluation-of-parameters-of-hemostasis-in-the-third-trimester-in-pregnant-women-with-congen%C3%A4-tal-hemorrhagic-diathesis
#18
L Rzaguliyeva, G Afandiyeva, N Malikqasımova
The aim is to study the coagulogram indices in the III trimester of pregnancy in women with hemophilia and von Willebrand disease. Twenty-seven pregnant women with hereditary coagulopathies of mild and moderate form (mean age 27.9±3.3 years) in the third trimester of pregnancy were examined. Of the examined 12 women - carriers of hemophilia A and B, 15 - with Willebrand disease (BV). Clinico-laboratory examination, clinical and biochemical blood tests were carried out. Coagulologic examination was performed on 17 parameters...
July 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28804836/assessment-of-factor-xiii
#19
László Muszbek, Éva Katona, Adrienne Kerényi
Blood coagulation factor XIII (FXIII) is essential for maintaining hemostasis. The absence of FXIII results in severe bleeding diathesis, which without prophylaxis frequently leads to fatal bleeding. As the usual hemostasis screening tests remain normal, the diagnosis of FXIII deficiency needs specific tests. Here, we describe FXIII activity determination by the ammonia release assay, which is the first-line test in the diagnostic algorithm for FXIII deficiency. The method for another activity test, the undeservedly rarely used fibrin cross-linking assay, is also presented...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28799202/immune-tolerance-induced-by-platelet-targeted-factor-viii-gene-therapy-in-hemophilia-a-mice-is-cd4-t-cell-mediated
#20
Y Chen, X Luo, J A Schroeder, J Chen, C K Baumgartner, J Hu, Q Shi
Essentials The immune response is a significant concern in gene therapy. Platelet-targeted gene therapy can restore hemostasis and induce immune tolerance. CD4 T cell compartment is tolerized after platelet gene therapy. Preconditioning regimen affects immune tolerance induction in platelet gene therapy. SUMMARY: Background Immune responses are a major concern in gene therapy. Our previous studies demonstrated that platelet-targeted factor VIII (FVIII) (2bF8) gene therapy together with in vivo drug selection of transduced cells can rescue the bleeding diathesis and induce immune tolerance in FVIII(null) mice...
October 2017: Journal of Thrombosis and Haemostasis: JTH
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