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treatment bleeding diathesis

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https://www.readbyqxmd.com/read/29320972/rituximab-use-in-pediatric-lupus-anticoagulant-hypoprothrombinemia-syndrome-report-of-three-cases-and-review-of-the-literature
#1
K Cetin Gedik, S Siddique, C L Aguiar
Lupus anticoagulant hypoprothrombinemia syndrome (LA-HPS) is a rare condition that may predispose both to thrombosis and bleeding due to positive lupus anticoagulant (LA) and factor II (FII) deficiency. It can be seen in association with infections or systemic lupus erythematosus (SLE) and may require glucocorticoids (GCs) and/or immunosuppressive medications. Pediatric LA-HPS cases in the literature and three cases that received only rituximab (RTX) for LA-HPS (in addition to GCs) at two institutions between January 2010 and June 2017 were analyzed descriptively...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29317961/successful-treatment-of-rapidly-progressive-life-threatening-esophageal-submucosal-hematoma-in-a-patient-with-van-der-hoeve-syndrome
#2
Yasuhiro Watanabe, Naomi Shimizu, Masahiro Iwakawa, Takashi Yamaguchi, Noriko Ban, Hidetoshi Kawana, Atsuhito Saiki, Emiko Sakaida, Chiaki Nakaseko, Yasuhiro Matsuura, Nobuyuki Aotsuka, Hideaki Bujo, Ichiro Tatsuno
Osteogenesis imperfecta (OI) is a rare inherited disorder of the connective tissue with many reports on its association with bleeding diatheses. OI patients with blue sclera, hearing loss, and bone vulnerability are classified as having van der Hoeve syndrome. Here, we report the first case of rapidly progressing, massive esophageal submucosal hematoma in this syndrome. Bleeding in OI is reportedly due to defective capillary integrity and platelet dysfunction; however, our patient did not show such findings...
February 2018: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29224364/new-anticoagulants-for-venous-thromboembolism-and-atrial-fibrillation-what-the-future-holds
#3
Gerasimos Dimitropoulos, S M Zubair Rahim, Alexandra Sophie Moss, Gregory Y H Lip
The field of anticoagulation has seen impressive progress over the last decade. The introduction of the Non Vitamin K Oral Anticoagulants (NOACs) has revolutionized practice surrounding thromboprophylaxis, treatment of thromboembolic disease and stroke prevention in atrial fibrillation (AF). However, the search for the 'holy grail' of anticoagulation, an agent that combines optimal efficacy with minimal bleeding diathesis, continues. Areas covered: In this paper we aim to summarize the current evidence from pre-clinical studies and early phase clinical trials, presenting the pharmacodynamic and pharmacokinetic properties as well as the safety and efficacy profiles of the most important antithrombotic agents in development...
December 14, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/29185819/reversal-of-glanzmann-thrombasthenia-platelet-phenotype-after-imatinib-treatment-in-a-pediatric-chronic-myeloid-leukemia-patient
#4
Richa Chauhan, Sudha Sazawal, Kanwaljeet Singh, R Ragesh R Nair, Sunita Chhikara, Roopam Deka, Rekha Chaubey, Karthika Kundil Veetil, Prasad Dange, Manoranjan Mahapatra, Renu Saxena
Chronic Myelogenous Leukemia (CML) is a myeloproliferative neoplasm characterized by proliferation of Philadelphia positive clonal pluripotent hematopoietic cells. Bleeding is a rare presentation of CML that can occur due to platelet dysfunction. Both pre-treatment and post-treatment platelet function abnormalities in CML have been described in the literature. We describe a rare case of childhood CML who presented with mucocutateous bleeding manifestations. On laboratory workup, a Glanzmann Thrombasthenia (GT) like platelet phenotype was demonstrated along with confirmation of diagnosis of CML in chronic phase...
November 29, 2017: Platelets
https://www.readbyqxmd.com/read/29125443/neurosurgical-management-in-children-with-bleeding-diathesis-auditing-neurological-outcome
#5
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/29048211/ureteroscopy-in-patients-with-bleeding-diathesis-a-systematic-review-and-meta-analysis-of-the-literature
#6
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/28903884/successful-outcome-of-severe-intra-cerebral-bleeding-associated-with-acquired-factor-v-inhibition-utilization-of-multiple-therapeutic-agents
#7
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/28861294/retrograde-intrarenal-surgery-for-renal-stones-part-2
#8
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/28799202/immune-tolerance-induced-by-platelet-targeted-factor-viii-gene-therapy-in-hemophilia-a-mice-is-cd4-t-cell-mediated
#9
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
https://www.readbyqxmd.com/read/28721285/ureteroscopy-for-stone-disease-expanding-roles-in-the-modern-era
#10
REVIEW
Adrian Ho, Piyush Sarmah, Ewa Bres-Niewada, Bhaskar K Somani
INTRODUCTION: The prevalence of urolithiasis is increasing worldwide and is causing significant morbidity and cost to the healthcare systems. While ureteroscopy (URS) has been established as a treatment option, our review highlights the expanding role of URS for the management of more complex stones and patients in the modern era. MATERIAL AND METHODS: Ureteroscopy has shown to have good outcomes with stone free rates (SFR) comparable to other treatment modalities...
June 30, 2017: Central European Journal of Urology
https://www.readbyqxmd.com/read/28720366/the-evolving-role-of-retrograde-intrarenal-surgery-in-the-treatment-of-urolithiasis
#11
REVIEW
Francesco Sanguedolce, Giorgio Bozzini, Ben Chew, Panagiotis Kallidonis, Jean de la Rosette
CONTEXT: Retrograde intrarenal surgery has gained substantial popularity worldwide thanks to continuous improvements in technology and techniques, and is now considered one of the first-line treatment options for active removal of renal stones. OBJECTIVE: To provide a comprehensive synthesis of the main evidences in literature on the current management of kidney stones by means of retrograde intrarenal surgery (RIRS). EVIDENCE ACQUISITION: A review of literature has been conducted using search string "retrograde intrarenal surgery OR flexible ureteroscop* OR ureterorenoscop*", without any language restriction; PubMed, Embase, and Scopus databases were searched in November 2016...
February 2017: European Urology Focus
https://www.readbyqxmd.com/read/28717532/retrograde-intrarenal-surgery-for-renal-stones-part-1
#12
REVIEW
Ben Van Cleynenbreugel, Özcan Kılıç, Murat Akand
The main aim in the treatment of renal stones is to clearance of the stones completely with the least morbidity. Parallel to the improvements in technology during recent years, new flexible ureterorenoscopes and effective lithotripters such as holmium laser have been developed, thus retrograde intrarenal surgery (RIRS) has become an efficient and safe option in the management of urinary system stone disease with a gradually increasing popularity. Therewithal, innovations in auxiliary equipment such as guide-wires, ureteral access sheath and stone baskets have made this procedure more effective...
June 2017: Turkish Journal of Urology
https://www.readbyqxmd.com/read/28643468/eltrombopag-use-in-a-patient-with-wiskott-aldrich-syndrome
#13
Maria Gabelli, Antonio Marzollo, Lucia Dora Notarangelo, Giuseppe Basso, Maria Caterina Putti
Wiskott-Aldrich syndrome (WAS) is an inherited X-linked disorder characterized by microthrombocytopenia, immunodeficiency, and eczema. Hematopoietic stem cell transplantation (HSCT) is the treatment of choice. Eltrombopag, a thrombopoietin receptor agonist, may be useful to prevent bleeding while awaiting HSCT. We present a case of a male with WAS, profound thrombocytopenia, and bleeding diathesis successfully managed with eltrombopag before HSCT. Eltrombopag was given for 32 weeks obtaining a stable platelet count without any platelet transfusion...
December 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28594466/bleeding-is-not-the-main-clinical-issue-in-many-patients-with-inherited-thrombocytopaenias
#14
REVIEW
F Melazzini, C Zaninetti, C L Balduini
Bleeding diathesis has been considered for a long time the main clinical issue impacting the lives of patients affected by inherited thrombocytopaenias. However, the number of known inherited thrombocytopaenias greatly increased in recent years, and careful evaluation of hundreds of patients affected by these 'new' disorders revealed that most of them are at risk of developing additional life-threatening disorders during childhood or adult life. These additional disorders are usually more serious and dangerous than low platelet count...
June 8, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28484587/reducing-error-in-anticoagulant-dosing-via-multidisciplinary-team-rounding-at-point-of-care
#15
Munish Sharma, Mahesh Krishnamurthy, Richard Snyder, James Mauro
The incorporation of a clinical pharmacist in daily rounding can help identify and correct errors related to anticoagulation dosing. Inappropriate anticoagulant dosing increases the risk of developing significant bleeding diathesis. Conversely, inappropriate dosing may also fail to produce a therapeutic response. We retrospectively reviewed electronic medical records of 41 patients to confirm and analyze the errors related to various anticoagulants. A clinical pharmacist in an integrated rounding between the period of February 2016 and April 2016 collected this data...
April 6, 2017: Clinics and Practice
https://www.readbyqxmd.com/read/28480552/hyperfibrinolysis-and-hypofibrinogenemia-diagnosed-with-rotational-thromboelastometry-in-dogs-naturally-infected-with-angiostrongylus-vasorum
#16
N E Sigrist, N Hofer-Inteeworn, R Jud Schefer, C Kuemmerle-Fraune, M Schnyder, A P N Kutter
BACKGROUND: The pathomechanism of Angiostrongylus vasorum infection-associated bleeding diathesis in dogs is not fully understood. OBJECTIVE: To describe rotational thromboelastometry (ROTEM) parameters in dogs naturally infected with A. vasorum and to compare ROTEM parameters between infected dogs with and without clinical signs of bleeding. ANIMALS: A total of 21 dogs presented between 2013 and 2016. METHODS: Dogs with A...
July 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28359132/point-of-care-testing-in-liver-disease-and-liver-surgery
#17
Lasitha Abeysundara, Susan V Mallett, Ben Clevenger
The alterations in coagulation and hemostasis that accompany liver disease are complex, and while patients with this disease have traditionally been perceived as having a bleeding diathesis, it is now understood that in stable patients hemostasis is "re-balanced." Hepatic surgery, and particularly liver transplantation, can be associated with large fluid shifts, massive bleeding, and coagulopathy, as well as postoperative thrombosis. Point-of-care tests (POCTs) of coagulation facilitate goal-directed treatments and hemostatic monitoring in dynamic environments where the coagulation status can alter rapidly and often unpredictably...
June 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28352011/biopsy-of-small-kidneys-a-safe-and-a-useful-guide-to-potentially-treatable-kidney-disease
#18
Kamel El-Reshaid, Wael El-Reshaid, Dalal Al-Bader, Jozsef Varro, John Madda, Hosameldin Tawfik Sallam
Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy. The procedure was performed by an interventional radiologist using a biopsy gun technique and under ultrasound guidance...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27909619/the-role-of-ultra-mini-percutaneous-nephrolithotomy-in-the-treatment-of-kidney-stones
#19
Abdulkadir Tepeler, İsmail Başıbüyük, Muhammed Tosun, Abdullah Armağan
OBJECTIVE: In our study we aimed to evaluate outcomes of ultra-mini-percutaneous nephrolithotomy (UMP) performed for the treatment of kidney stones. MATERIAL AND METHODS: Between October 2014 and September 2015, a single surgeon performed UMP on a total of 49 consecutive patients with symptomatic kidney stones at our center. Pregnants, anticogulant users, patients with bleeding diathesis pyonephrosis, and partial/complete staghorn stones were excluded from the study...
December 2016: Turkish Journal of Urology
https://www.readbyqxmd.com/read/27866582/bleeding-diatheses-approach-to-the-patient-who-bleeds-or-has-abnormal-coagulation
#20
REVIEW
Marcia Paddock, John Chapin
Many complex elements contribute to normal hemostasis, and an imbalance of these elements may lead to abnormal bleeding. In addition to evaluating medication effects, the hematologist must evaluate for congenital or acquired deficiencies in coagulation factors and platelet disorders. This evaluation should include a thorough bleeding history with careful attention to prior hemostatic challenges and common laboratory testing, including coagulation studies and/or functional platelet assays. An accurate diagnosis of a bleeding diathesis and selection of appropriate treatment are greatly aided by a basic understanding of the mechanisms of disease and the tests used to diagnose them...
December 2016: Primary Care
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