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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/29273073/pulmonary-tumor-thrombotic-microangiopathy-successfully-treated-with-corticosteroids-a-case-report
#3
Shinichi Miyazaki, Takuya Ikeda, Genshi Ito, Masahide Inoue, Keiji Nara, Yuko Nishinaga, Yoshinori Hasegawa
BACKGROUND: Pulmonary tumor thrombotic microangiopathy is a special type of tumor thromboembolism. We report the case of a patient who developed pulmonary tumor thrombotic microangiopathy with alveolar hemorrhage. Almost all patients with pulmonary tumor thrombotic microangiopathy die within 1 week of the onset of dyspnea; however, the prognosis in this case was better, with 10 weeks of survival from presentation. CASE PRESENTATION: A 62-year-old Japanese man was referred to our hospital with a 4-week history of dyspnea on exertion and severe pulmonary hypertension...
December 23, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29227167/diagnosis-of-platelet-function-disorders-a-standardized-rational-and-modular-flow-cytometric-approach
#4
Oliver Andres, Katja Henning, Gabriele Strauß, Annerose Pflug, Georgi Manukjan, Harald Schulze
A high proportion of patients with mucocutaneous bleeding diathesis and suspected inherited or acquired platelet disorder remain without diagnosis even after comprehensive laboratory testing. Since flow cytometry allows investigation of resting and activated platelets on the single cell level by requiring only minimal amounts of blood, this method has become an important assay within the diagnostic algorithm, especially in pediatrics. We therefore developed a standardized and modular flow cytometric approach that contributes to clarify impaired platelet function in a rational step-by-step manner...
December 11, 2017: Platelets
https://www.readbyqxmd.com/read/29224364/new-anticoagulants-for-venous-thromboembolism-and-atrial-fibrillation-what-the-future-holds
#5
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/29200669/adrenal-mass-unusual-presentation-and-outcome
#6
Raghu Sampally Ramareddy, Anand Alladi
Aim: Adrenal mass may be functioning or nonfunctioning with varied clinical presentations. This study aimed to report the nature and management of uncommon adrenal mass and to review literature. Materials and Methods: This was an retrospective observational analysis of children with uncommon adrenal mass admitted during 2009-2015. Clinical features, investigations, and management of patients were analyzed. Results: Among six, two each were adolescent and neonate, and one each was young infant and prenatal...
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29194678/bleeding-complications-in-acute-liver-failure
#7
R Todd Stravitz, Caitlyn Ellerbe, Valerie Durkalski, Michael Schilsky, Robert J Fontana, Carolyn Peterseim, William M Lee
In patients with acute liver failure (ALF), elevatedprothrombin time and thrombocytopeniacan fuel a perception of a bleeding tendency. However, the incidence, site, risk factors, and clinical significance of bleeding complications have not been previously quantified in a large cohort of patients with ALF. We studied 1770adult patients enrolled in the ALF Study Group Registry between 1998 and 2016.Bleeding complications and blood component transfusions were collected for 7days after admission. The relationship of bleeding complications to 21-day mortality was assessed...
December 1, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29185819/reversal-of-glanzmann-thrombasthenia-platelet-phenotype-after-imatinib-treatment-in-a-pediatric-chronic-myeloid-leukemia-patient
#8
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/29159724/turkish-case-of-ethylmalonic-encephalopathy-misdiagnosed-as-short-chain-acyl-coa-dehydrogenase-deficiency
#9
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
#10
REVIEW
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
#11
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
#12
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
#13
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
#14
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...
November 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29027774/rational-approach-to-transfusion-in-liver-transplantation
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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