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pediatrics, thrombocytopenia

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https://www.readbyqxmd.com/read/29334536/nationwide-trend-analysis-of-pediatric-inpatients-with-immune-thrombocytopenia-in-the-united-states
#1
Yusuke Okubo, Atsuhiko Handa
BACKGROUND: Several studies have reported the epidemiology of immune thrombocytopenia (ITP) among children in the United States and other countries. However, recent trends in ITP among hospitalized children and hospital course remain unknown at a national level in the United States. METHOD: Hospital discharge records of patients with ITP aged 19 years and younger were obtained for the years 2003, 2006, 2009, and 2012 using the Kids' Inpatient Database. Data were weighted to estimate the annual hospitalization rates in the United States with trend analyses...
January 12, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29333010/sinusoidal-obstruction-syndrome-during-treatment-for-wilms-tumor-a-life-threatening-complication
#2
Sidharth Totadri, Amita Trehan, Deepak Bansal, Richa Jain
Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29330464/eltrombopag-versus-romiplostim-in-treatment-of-children-with-persistent-or-chronic-immune-thrombocytopenia-a-systematic-review-incorporating-an-indirect-comparison-meta-analysis
#3
Jiaxing Zhang, Yi Liang, Yuan Ai, Xiaosi Li, Juan Xie, Youping Li, Wenyi Zheng, Rui He
In absence of direct comparison, we conducted an indirect-comparison meta-analysis to evaluate the efficacy and safety of thrombopoietin-receptor agonists(TPO-RAs) in treatment of pediatric persistent or chronic immune thrombocytopenia(ITP). PubMed, Embase, Cochrane Library, Clinical Trials.gov, China National Knowledge Infrastructure, and Chinese Biomedical Literature Database were searched from their earliest records to May 2017. Randomized controlled trials comparing the TPO-RAs with placebo in pediatric ITP were included...
January 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29330115/pediatric-onset-evans-syndrome-heterogeneous-presentation-and-high-frequency-of-monogenic-disorders-including-lrba-and-ctla4-mutations
#4
Caroline Besnard, Eva Levy, Nathalie Aladjidi, Marie-Claude Stolzenberg, Aude Magerus-Chatinet, Olivier Alibeu, Patrick Nitschke, Stéphane Blanche, Olivier Hermine, Eric Jeziorski, Judith Landman-Parker, Guy Leverger, Nizar Mahlaoui, Gérard Michel, Isabelle Pellier, Felipe Suarez, Isabelle Thuret, Geneviève de Saint-Basile, Capucine Picard, Alain Fischer, Bénédicte Neven, Frédéric Rieux-Laucat, Pierre Quartier
Evans syndrome (ES) is defined by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. Clinical presentation includes manifestations of immune dysregulation, found in primary immune deficiencies, autoimmune lymphoproliferative syndrome with FAS (ALPS-FAS), Cytotoxic T Lymphocyte Antigen-4 (CTLA-4) and Lipopolysaccharide-Responsive vesicle trafficking Beige-like and Anchor protein (LRBA) defects. We report the clinical history and genetic results of 18 children with ES after excluding ALPS-FAS...
January 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29313460/thrombopoietin-and-its-receptor-expression-in-pediatric-patients-with-chronic-immune-thrombocytopenia
#5
Shanshan Li, Jingbo Shao, Min Xia, Na Zhang, Jingwei Yang, Hong Li, Hui Jiang
OBJECTIVES: Chronic immune thrombocytopenia (cITP) is common in children. However, the pathogenesis has not been fully elucidated. This study aimed to determine whether thrombopoietin (TPO) and its receptor c-mannosylation of the TPO receptor (c-Mpl) have an impact on childhood cITP. METHODS: Sixty-four patients with newly diagnosed ITP (nITP), 64 patients with persistent ITP, 80 patients with cITP, and 64 healthy children (control) were enrolled in this study. Plasma TPO was measured with an ELISA, and c-Mpl was determined by flow cytometry...
January 9, 2018: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29310365/ebv-lymphoproliferative-associated-disease-and-primary-cardiac-t-cell-lymphoma-in-a-stk4-deficient-patient-a-case-report
#6
Roya Sherkat, Mohammad Reza Sabri, Bahar Dehghan, Hamid Bigdelian, Nahid Reisi, Nooshin Afsharmoghadam, Hamid Rahimi, Narges Rahmanian, Cristoph Klein
RATIONALE: Primary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world. PATIENT CONCERNS: An eleven-year-old girl, a known case of PID, was referred to the pediatric cardiology department because of chest pain and dyspnea...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29301470/risk-of-complications-of-ultrasound-guided-renal-biopsy-for-adult-and-pediatric-patients-with-systemic-lupus-erythematosus
#7
Y S Sun, I T Sun, H K Wang, A H Yang, C Y Tsai, C J Huang, D F Huang, C C Lai
Objective The objective of this paper is to identify the risk of complications of real-time ultrasound-guided renal biopsy in adult and pediatric patients with systemic lupus erythematosus (SLE). Materials and methods This retrospective study examined outcomes of 296 renal biopsy procedures in 275 SLE patients. Imaging-confirmed symptomatic hematoma was regarded as a major complication when intervention (blood transfusion, angiographic embolization, or surgery) was required or as a minor complication otherwise...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29296878/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#8
REVIEW
Cindy E Neunert
Immune thrombocytopenia resulting from antibody-mediated platelet destruction combined with impaired platelet production is a common cause of thrombocytopenia. The decision to treat newly diagnosed patients is based on several factors including ceasing hemorrhagic manifestations, increasing the platelet count, prevention of bleeding, and inducing remission. Current standard first-line therapy is a course of corticosteroids. Although this treatment paradigm increases the platelet count in the majority of patients, a high percentage relapse after discontinuation of corticosteroid therapy...
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29281614/potential-contribution-of-erythrocyte-microrna-to-secondary-erythrocytosis-and-thrombocytopenia-in-congenital-heart-disease
#9
Nobuhiro Mukai, Yoshinobu Nakayama, Satoshi Murakami, Toshihito Tanahashi, Daniel I Sessler, Sachiyo Ishii, Satoru Ogawa, Natsuko Tokuhira, Toshiki Mizobe, Teiji Sawa, Yasufumi Nakajima
BACKGROUND: Children with cyanotic heart disease develop secondary erythrocytosis and thrombocytopenia via unknown mechanisms. Mature erythrocyte microRNAs may reflect clinical pathologies and cell differentiation processes pre-enucleation. This study evaluated erythrocyte microRNAs in children with cyanotic heart disease. METHODS: Erythrocyte microRNAs from children with cyanotic and acyanotic heart disease and without cardiac disease were quantified with Ion PGM System (n=10 per group)...
December 27, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29242302/a-distinct-subtype-of-epstein-barr-virus-positive-t-nk-cell-lymphoproliferative-disorder-adult-patients-with-chronic-active-epstein-barr-virus-infection-like-features
#10
Keisuke Kawamoto, Hiroaki Miyoshi, Takaharu Suzuki, Yasuji Kozai, Koji Kato, Masaharu Miyahara, Toshiaki Yujiri, Naoki Oishi, Ilseung Choi, Katsumichi Fujimaki, Tsuyoshi Muta, Masaaki Kume, Sayaka Moriguchi, Shinobu Tamura, Takeharu Kato, Hiroyuki Tagawa, Junya Makiyama, Yuji Kanisawa, Yuya Sasaki, Daisuke Kurita, Kyohei Yamada, Joji Shimono, Hirohito Sone, Jun Takizawa, Masao Seto, Hiroshi Kimura, Koichi Ohshima
The characteristics of adult patients with chronic active Epstein-Barr virus infection (adult-onset CAEBV) are poorly recognized, hindering early diagnosis and an improved prognosis. Adult-onset CAEBV (n = 54) diagnosed between 2005 and 2015 were conducted. Adult-onset was defined as an estimated age of onset ≥15 years. To characterize the clinical features of adult-onset CAEBV, we compared them to those of pediatric-onset (estimated age of onset <15 years) patients (n = 75). We compared the prognosis of adult-onset CAEBV with that for patients with nasal-type (n = 37) and non-nasal-type (n = 45) extranodal NK/T-cell lymphoma (ENKTL)...
December 14, 2017: Haematologica
https://www.readbyqxmd.com/read/29227167/diagnosis-of-platelet-function-disorders-a-standardized-rational-and-modular-flow-cytometric-approach
#11
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/29222284/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#12
REVIEW
Cindy E Neunert
Immune thrombocytopenia resulting from antibody-mediated platelet destruction combined with impaired platelet production is a common cause of thrombocytopenia. The decision to treat newly diagnosed patients is based on several factors including ceasing hemorrhagic manifestations, increasing the platelet count, prevention of bleeding, and inducing remission. Current standard first-line therapy is a course of corticosteroids. Although this treatment paradigm increases the platelet count in the majority of patients, a high percentage relapse after discontinuation of corticosteroid therapy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29182495/platelet-transfusion-for-patients-with-cancer-american-society-of-clinical-oncology-clinical-practice-guideline-update
#13
Charles A Schiffer, Kari Bohlke, Meghan Delaney, Heather Hume, Anthony J Magdalinski, Jeffrey J McCullough, James L Omel, John M Rainey, Paolo Rebulla, Scott D Rowley, Michael B Troner, Kenneth C Anderson
Purpose To provide evidence-based guidance on the use of platelet transfusion in people with cancer. This guideline updates and replaces the previous ASCO platelet transfusion guideline published initially in 2001. Methods ASCO convened an Expert Panel and conducted a systematic review of the medical literature published from September 1, 2014, through October 26, 2016. This review builds on two 2015 systematic reviews that were conducted by the AABB and the International Collaboration for Transfusion Medicine Guidelines...
November 28, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29172661/-novelties-in-the-treatment-of-pediatric-immune-thrombocytopenia-2017
#14
Gábor Kovács, Csongor Kiss
Immune thrombocitopenia in children is a very variable disease. International recommendations give therapeutic possibilities without strong protocols. In 2011, a therapeutic algorithm was published based on Hungarian practice. Recently, new innovative drugs have been available even in Hungary, so there is a need for modification of the therapeutic protocols. In this summary we give an overview about the current up-to-date management. In infancy and in childhood, high-dose immunglobulin treatment is recommended henceforward...
December 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/29149805/observational-cohort-study-safety-outcomes-in-children-using-proton-pump-inhibitors-or-histamine-2-receptor-antagonists
#15
Eline Houben, Saga Johansson, Péter Nagy, Fernie J A Penning-van Beest, Ernst J Kuipers, Ron M C Herings
OBJECTIVE: Acid suppression with histamine-2 receptor antagonists (H2RAs) or proton pump inhibitors (PPIs) is recommended for children with persistent gastroesophageal reflux disease symptoms. In this retrospective, observational postauthorization study, we aimed to assess and compare safety outcomes in pediatric first-time users of esomeprazole, other PPIs or H2RAs. METHODS: Data on children (aged 0-18 years) first dispensed esomeprazole, other PPIs or H2RAs between September 2008 and August 2011 was obtained from the Dutch PHARMO Database Network...
November 24, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/29050793/anaplasmosis-in-pediatric-patients-case-report-and-review
#16
Vaka K Sigurjonsdottir, Henry M Feder, Gary P Wormser
Human granulocytic anaplasmosis (HGA) is a tick-borne infection, characterized as an acute and sometimes severe febrile illness which may be associated with leukopenia and thrombocytopenia. Most case reports of HGA have been in adults, with only 8 case reports of HGA in children. We add a ninth case of HGA, which occurred in a 5-year-old. The paucity of pediatric HGA case reports maybe because publication bias, or HGA in children is a mild illness and children with HGA are less likely than adults to seek medical care, or the diagnosis of HGA requires a blood draw and adults (versus children) are more likely to get diagnostic testing...
November 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/29050748/pediatric-kikuchi-fujimoto-disease-a-clinicopathologic-study-and-the-therapeutic-effects-of-hydroxychloroquine
#17
Yung-Chih Lin, Hsiu-Hui Huang, Bao-Ren Nong, Po-Yen Liu, Ying-Yao Chen, Yung-Feng Huang, Yee-Hsuan Chiou, Herng-Sheng Lee
BACKGROUND: To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) in children, and place an emphasis on the therapeutic effects of hydroxychloroquine as monotherapy. METHODS: We retrospectively reviewed the medical records of all children diagnosed with KFD during the period January 1992 to September 2016 at a tertiary medical center in Taiwan. RESULTS: 40 patients were histopathologically confirmed as KFD, and the mean age of the patients was 13...
September 29, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29050117/-pediatric-idiopathic-hypereosinophilic-syndrome-with-pulmonary-embolism-a-case-report-and-review-of-literature
#18
N Dong, X Y Dong
Objective: To explore clinical features of idiopathic hypereosinophilic syndrome combined with pulmonary embolism. Method: A retrospective analysis of a patient with idiopathic hypereosinophilic syndrome and pulmonary embolism diagnosed and treated in the Respiratory Department of Shanghai Children's Hospital in September 2016 was performed. A literature search was performed with"Eosinophils increased, thrombosis"as the Chinese keywords in Wanfang database and"idiopathic hypereosinophilic syndrome, deep vein thrombosis"as the English key words in PubMed database...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29040243/toxic-shock-syndrome-still-a-timely-diagnosis
#19
Kira L Gossack-Keenan, April J Kam
BACKGROUND: Toxic shock syndrome (TSS) is an acute, severe, toxin-mediated disease, characterized by fever, hypotension, and multiorgan system involvement. Toxic shock syndrome has made headlines because of its high associated morbidity and mortality rate in previously healthy young females. Incidence peaked in the early 1980s owing to increased usage of ultra-absorbent tampons. After improved patient education and tampon labeling, the incidence of menstrual TSS has declined. CASE: A previously healthy 14-year-old girl presented to an urgent care center with a 2-day history of fever, erythematous maculopapular rash, vomiting, diarrhea, and malaise...
October 16, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29034009/a-multicenter-randomized-study-of-decitabine-as-epigenetic-priming-with-induction-chemotherapy-in-children-with-aml
#20
Lia Gore, Timothy J Triche, Jason E Farrar, Daniel Wai, Christophe Legendre, Gerald C Gooden, Winnie S Liang, John Carpten, David Lee, Frank Alvaro, Margaret E Macy, Carola Arndt, Philip Barnette, Todd Cooper, Laura Martin, Aru Narendran, Jessica Pollard, Soheil Meshinchi, Jessica Boklan, Robert J Arceci, Bodour Salhia
BACKGROUND: Decitabine is a deoxycytidine nucleoside derivative inhibitor of DNA-methyltransferases, which has been studied extensively and is approved for myelodysplastic syndrome in adults but with less focus in children. Accordingly, we conducted a phase 1 multicenter, randomized, open-label study to evaluate decitabine pre-treatment before standard induction therapy in children with newly diagnosed AML to assess safety and tolerability and explore a number of biologic endpoints. RESULTS: Twenty-four patients were fully assessable for all study objectives per protocol (10 in Arm A = epigenetic priming induction, 14 in Arm B = standard induction)...
2017: Clinical Epigenetics
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