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https://www.readbyqxmd.com/read/28099398/utilizing-a-novel-mobile-health-selfie-application-to-improve-compliance-to-iron-chelation-in-pediatric-patients-receiving-chronic-transfusions
#1
Sarah Leonard, Lindsay M Anderson, Jude Jonassaint, Charles Jonassaint, Nirmish Shah
Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes. Pediatric patients were recruited to participate in the ITP over a 90-day period and were followed for 6 months...
January 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28090489/effect-of-short-term-high-dose-methylprednisolone-on-oxidative-stress-in-children-with-acute-immune-thrombocytopenia
#2
Musa Cura, Ahmet Koç, Nurten Aksoy, Zeynep Canan Özdemir
BACKGROUND: Immune thrombocytopenia (ITP) is the most common cause of acquired childhood thrombocytopenia and is characterized by increased immune-mediated destruction of circulating thrombocytes. Oxidative damage may be involved in ITP pathogenesis; paraoxonase (PON) and arylesterase (ARE) enzymes are closely associated with the cellular antioxidant system. We investigated the effect of short-term high-dose methylprednisolone (HDMP) treatment on the total oxidant status (TOS), total antioxidant capacity (TAC), oxidative stress index (OSI), and PON and ARE enzymatic activity in children with acute ITP...
December 2016: Blood Research
https://www.readbyqxmd.com/read/28090042/autoimmune-hepatitis-associated-with-immune-thrombocytopenic-purpura
#3
Akihiro Ito, Kaname Yoshizawa, Kazuya Fujimori, Susumu Morita, Takashi Shigeno, Toshitaka Maejima
Although autoimmune hepatitis (AIH) is frequently complicated with chronic thyroiditis or other autoimmune disorders, reports on its association with immune thrombocytopenic purpura (ITP) are scarce. We herein describe a case of AIH associated with ITP. A 75-year-old Japanese woman was admitted to our hospital due to increased aminotransferase levels and severe thrombocytopenia. Elevated serum immunoglobulin G (IgG) was detected, and tests for platelet-associated IgG and anti-nuclear antibody were positive...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28078756/infections-in-non-splenectomized-persistent-or-chronic-primary-immune-thrombocytopenia-adults-risk-factors-and-vaccination-effect
#4
Guillaume Moulis, Maryse Lapeyre-Mestre, Aurore Palmaro, Laurent Sailler
INTRODUCTION: Risk factors for infection and protective effect of vaccines in immune thrombocytopenia (ITP) patients in the era of rituximab therapy are unknown. OBJECTIVES: To assess the risk factors for serious and non-serious infections (respectively, SIs and NSIs) in non-splenectomized adults treated for persistent or chronic primary ITP, including the effect of pneumococcal and influenza vaccines. PATIENTS/METHODS: The population was the 2009-2012 FAITH cohort (n=1805), which is the cohort of all incident (newly diagnosed) primary ITP adults treated >3months in France built in the national health insurance database (SNIIRAM)...
January 12, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28073042/rapid-infusions-of-human-normal-immunoglobulin-50g-l-are-safe-and-well-tolerated-in-immunodeficiencies-and-immune-thrombocytopenia
#5
Giuseppe Spadaro, Alessandra Vultaggio, A Alberto Bosi, Dietmar Reichert, Jan Janssen, Donatella Lamacchia, Liliana Nappi, Antonio Pecoraro, Cinzia Milito, Andrea Ferraro, Andrea Matucci, Francesca Bacchiarri, Valentina Carrai, Azra Hibbeler, Elisabet Speckman, Chiara Guarnieri, Serena Bongiovanni, Isabella Quinti
Intravenous immunoglobulin (IVIg) is accepted as an effective and well-tolerated treatment for primary and secondary immunodeficiencies (ID) and immune thrombocytopenia (ITP). Adverse reactions of IVIg are usually mild, comprising transient flu-like symptoms, change in blood pressure and tachycardia. However IVIg therapy can be burdensome for both patients and healthcare facilities, since the infusion may take up to 4h to administer. The objective of our multicentre, prospective, open-label phase III trial was to evaluate the tolerability and safety of human normal immunoglobulin 50g/l (Ig VENA) at high intravenous infusion rates in adult patients with ID and ITP who had previously tolerated IVIg treatment, by progressively increasing infusion rate up to 8ml/kg/hr...
January 7, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28064294/primary-versus-secondary-immune-thrombocytopenia-in-adults-a-comparative-analysis-of-clinical-and-laboratory-attributes-in-newly-diagnosed-patients-in-southern-pakistan
#6
S Sultan, S J Ahmed, S Murad, S M Irfan
BACKGROUND: Immune thrombocytopenic purpura (ITP) is a hemorrhagic diathesis, characterized by platelets destruction alongside impaired production. Patients from Asian regions often exhibit distinctive characteristics in comparison to the western patients. We accomplished this study to evaluate the prevalence of primary versus secondary ITP along with the comparative analysis between them. The secondary objective was to determine the etiological spectrum of secondary ITP. METHODS: We illustrate the results of a large cohort of newly diagnosed adults ITP from southern Pakistan...
October 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28063954/autoimmune-cytopenias-associated-with-inflammatory-bowel-diseases-insights-from-a-multicenter-retrospective-cohort
#7
Mathieu Uzzan, Lionel Galicier, Jean-Marc Gornet, Eric Oksenhendler, Claire Fieschi, Matthieu Allez, Yoram Bouhnik, Julien Kirchgesner, David Boutboul, Xavier Treton, Laurence Gérard, Matthieu Mahévas, Jacques Cosnes, Aurélien Amiot
INTRODUCTION: Autoimmune cytopenias (AIC) including autoimmune hemolytic anemia (AIHA) and immunologic thrombocytopenia (ITP) are rare immunologic disorders, scarcely reported in inflammatory bowel diseases (IBD). We conducted a multicentric retrospective study, including a case-control analysis, that aimed to describe the characteristics and outcomes of patients affected by AIC and IBD. METHOD: Forty cases were recruited from 4 IBD centers and 2 AIC tertiary centers...
December 23, 2016: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28054583/common-variable-immunodeficiency-patients-with-a-phenotypic-profile-of-immunosenescence-present-with-thrombocytopenia
#8
Jan Stuchlý, Veronika Kanderová, Marcela Vlková, Ivana Heřmanová, Lucie Slámová, Ondřej Pelák, Eli Taraldsrud, Dalibor Jílek, Pavlína Králíc Ková, Børre Fevang, Marie Trková, Ondřej Hrušák, Eva Froňková, Anna Šedivá, Jiří Litzman, Tomáš Kalina
Common variable immunodeficiency (CVID) is a heterogeneous group of diseases. Our aim was to define sub-groups of CVID patients with similar phenotypes and clinical characteristics. Using eight-color flow cytometry, we analyzed both B- and T-cell phenotypes in a cohort of 88 CVID patients and 48 healthy donors. A hierarchical clustering of probability binning "bins" yielded a separate cluster of 22 CVID patients with an abnormal phenotype. We showed coordinated proportional changes in naïve CD4+ T-cells (decreased), intermediate CD27- CD28+ CD4+ T-cells (increased) and CD21low B-cells (increased) that were stable for over three years...
January 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28052942/refractory-primary-immune-thrombocytopenia-with-subsequent-del-5q-mds-complete-remission-of-both-after-lenalidomide
#9
Thomas Bech Mortensen, Henrik Frederiksen, Claus Werenberg Marcher, Birgitte Preiss
A patient with refractory primary immune thrombocytopenia (ITP) characterised by severe skin and mucosal bleedings was treated with several ITP-directed therapies including cyclophosphamide. He later developed therapy-related del(5q) myelodysplastic syndrome with no dysplastic morphological features in bone marrow. He remained severely thrombocytopenic, which suggests ongoing immune mediated platelet destruction. After two 3 week cycles of low-dose lenalidomide, complete cytogenetic remission and complete normalisation of platelet count were observed...
January 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28047796/we-de-201-01-best-in-physics-therapy-a-fast-multi-target-inverse-treatment-planning-strategy-optimizing-dosimetric-measures-for-high-dose-rate-hdr-brachytherapy
#10
C Guthier, A Damato, A Viswanathan, J Hesser, R Cormack
PURPOSE: Inverse treatment planning (ITP) for interstitial HDR brachytherapy of gynecologic cancers seeks to maximize coverage of the clinical target volumes (tumor and vagina) while respecting dose-volume-histogram related dosimetric measures (DMs) for organs at risk (OARs). Commercially available ITP tools do not support DM-based planning because it is computationally too expensive to solve. In this study we present a novel approach that allows fast ITP for gynecologic cancers based on DMs for the first time...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28034281/is-there-any-role-for-splenectomy-in-adulthood-onset-chronic-immune-thrombocytopenia-in-the-era-of-tpo-receptors-agonists-a-critical-overview
#11
Vibor Milunovic, Inga Mandac Rogulj, Slobodanka Kolonic Ostojic
Immune thrombocytopenia (ITP) in adulthood is characterized by chronic relapsing course. Despite the efficacious first line treatment (corticosteroid, intravenous immunoglobulin), majority of patients will enter the chronic phase warranting another treatment approach. Until recently, splenectomy performed in ITP chronic phase represented the standard of care with long-term remissions in more than 70% of patients, but however has never been tested in clinical trials. However, with the advances of our understanding of ITP pathophysiology and the shifting focus on megakaryocyte impairment, novel drugs were introduced in treatment paradigm, mainly trombopoietin receptor agonists (TPO-RAs); romiplostim and eltrombopag...
December 29, 2016: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/28028748/mouse-immune-thrombocytopenia-is-associated-with-th1-bias-and-expression-of-activating-fc%C3%AE-receptors
#12
Tetsuya Nishimoto, Yuka Okazaki, Miku Numajiri, Masataka Kuwana
Immune thrombocytopenia (ITP) is an autoimmune disease mediated by anti-platelet autoantibodies. We recently established a mouse ITP model exhibiting regulatory T-cell (Treg) deficiency, although only one-third of the Treg-deficient mice developed ITP. To clarify mechanisms involved in the emergence of platelet-specific autoimmunity in this model, we examined the T helper (Th)-cell balance and macrophage Fcγ receptor (FcγR) expression profiles in Treg-deficient mice with and without ITP. Splenocytes from both populations of Treg-deficient mice and control BALB/c mice were subjected to flow cytometry-based analyses to evaluate Th cell subset proportions and the expression of activating and inhibitory FcγRs on macrophages...
December 27, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/28024506/-clinical-significance-of-the-balanceshift-of-th1-and-th2-type-cytokines-in-patients-with-primary-immune-thrombocytopenic-purpura-detected-by-cytometric-bead-array
#13
Hong-Qiang Luo, Wei-Ying Feng, Yong-Geng Zhong, Guo-Zhong Zhou, Jia-Ping Fu
OBJECTIVE: To investigate the levels of Th1/Th2 cytokines in peripheral blood of patients with primary immune thrombocytopenic purpura(ITP) before and after treatment and their clinical significance. METHODS: Ninety-eight cases of ITP were treated with glucocorticoid(GC), then were divided into 2 groups: effectively treated group and uneffectively treated group according to efficacy of treatment, 40 healthy persons confirmed by health examination were selected and enrolled in control group...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28024504/-significance-of-plasma-hmgb1-ifn-%C3%AE-il-4-and-tlr4-expression-on-cd4-t-cell-surface-in-patients-with-immune-thrombocytopenia
#14
Xin Dai, Wen-Qian Li, Jian-Ping Li, Jian-Ming Feng
OBJECTIVE: To investigate the effects of plasma HMGB1, IFN-γ, IL-4 and CD4(+)T cell surface TLR4 expression on the immune thrombocytopenia(ITP). METHODS: Twenty-five patients diagnosed as ITP in our hospital from October 2014 to October 2015, and 20 healthy persons as controls were selected. The ELISA was used to detect the plasma levels of HMGB1, IFN-γ and IL-4 in 2 groups; the flow cytometry was used to detect the expression level of TLR4 on the surface of CD4(+) cells...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28024503/-role-and-significance-of-t-help-cells-17-in-pathogenesis-of-idiopathic-thrombocytopenic-purpura
#15
Xiao Wang, Yu-Hong Zhou, Xiao-Hong Chen, Li-Ming Yin, Yan-Na Zhao, Li-Ke Wo
OBJECTIVE: To investigate the role and significance of T help cells 17(Th17) in pathogenesis of idiopathic thrombocytopenic purpura (ITP). METHODS: Peripheral blood samples from ITP patients and normal controls were examined for Th17 cell proportion by flow cytometry (FCM). Expression of IL-17, IL-23, IL-6 and TGF-β1 in hematoplasma was detected by ELISA. The mRNA expression level of IL-17 and RORγt in peripheral blood mononuclear cells (PBMNC) from patients with ITP and normal controls were measured by RT-PCR technique, and expression levels of pSTAT3 and RORγt proteins were analyzed by Western-blot...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28006767/a-2-year-longitudinal-prospective-study-of-the-effects-of-eltrombopag-on-bone-marrow-in-patients-with-chronic-immune-thrombocytopenia
#16
Russell K Brynes, Raymond S M Wong, Maung M Thein, Kalpana K Bakshi, Paul Burgess, Dickens Theodore, Attilio Orazi
BACKGROUND: The long-term effects of eltrombopag on bone marrow (BM) reticulin and/or collagen deposition in previously treated adults with chronic immune thrombocytopenia (ITP) were assessed. METHODS: Three BM biopsies were collected at baseline and after 1 and 2 years of eltrombopag treatment. Specimens were centrally processed, stained for reticulin and collagen, independently reviewed by 2 hematopathologists, and rated according to the European Consensus 0-3 scale of marrow fibrosis (MF)...
December 23, 2016: Acta Haematologica
https://www.readbyqxmd.com/read/28001314/new-aspects-on-the-efficacy-of-high-dose-intravenous-immunoglobulins-in-patients-with-autoimmune-thrombocytopenia
#17
B Mayer, F Depré, F Ringel, A Salama
BACKGROUND AND OBJECTIVES: The clinical significance of autoimmune thrombocytopenia (ITP) is reflected by bleeding and/or an increased bleeding risk due to low platelet counts. In most cases, treatment with high-dose (1-2 g/kg body weight) intravenous immunoglobulin has been demonstrated to result in an increase in platelet counts after one day of treatment. Until now, there is little information on the true beginning of therapy effect in patients treated with high-dose intravenous immunoglobulin...
January 2017: Vox Sanguinis
https://www.readbyqxmd.com/read/27991534/efficacy-and-safety-of-thrombopoietin-receptor-agonists-in-patients-with-primary-immune-thrombocytopenia-a-systematic-review-and-meta-analysis
#18
Li Wang, Zhe Gao, Xiao-Ping Chen, Hai-Yan Zhang, Nan Yang, Fei-Yan Wang, Li-Xun Guan, Zhen-Yang Gu, Sha-Sha Zhao, Lan Luo, Hua-Ping Wei, Chun-Ji Gao
Immune thrombocytopenia (ITP) is an autoimmune disease characterized by increased platelet destruction and impaired platelet production. In this study, we conducted a systematic review and meta-analysis to determine the efficacy and safety of thrombopoietin receptor agonists (TPO-RAs) in primary ITP patients. Thirteen randomized controlled trials were included in this study, the pooled results of which demonstrated that TPO-RAs significantly increased platelet response (R) and durable response (DR) rates [risk ratio (RR): 2...
December 19, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27981682/rituximab-in-immune-thrombocytopenia-gender-age-and-response-as-predictors-of-long-term-response
#19
Miriam Marangon, Nicola Vianelli, Francesca Palandri, Maria Gabriella Mazzucconi, Cristina Santoro, Wilma Barcellini, Bruno Fattizzo, Stefano Volpetti, Elisa Lucchini, Nicola Polverelli, Monica Carpenedo, Miriam Isola, Renato Fanin, Francesco Zaja
OBJECTIVES: To evaluate the efficacy of a salvage treatment with Rituximab in adults with primary immune thrombocytopenia (ITP), in terms of short-term response and long-term response (LTR, i.e. probability to achieve and maintain response) and to identify biological and clinical predictors of response. METHODS: We retrospectively evaluated the outcome of patients with primary ITP treated with standard dosage RTX (375 mg/m(2) x 4) as salvage therapy in five Italian centres...
December 16, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27977588/clinical-characteristics-of-immune-thrombocytopenia-associated-with-autoimmune-disease-a-retrospective-study
#20
Yuan Liu, Shiju Chen, Yuechi Sun, Qingyan Lin, Xining Liao, Junhui Zhang, Jiao Luo, Hongyan Qian, Lihua Duan, Guixiu Shi
To clarify clinical characteristics of immune thrombocytopenia (ITP) subsets associated with autoimmune diseases (AIDs).Five thousand five hundred twenty patients were reviewed retrospectively. One hundred four ITP patients were included for analysis. Clinical manifestations at first thrombocytopenic episode were recorded.Systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) accounted for a large part in AIDs associated with secondary ITP. SLE-ITP, pSS-ITP, and primary ITP (pITP) patients were different in several aspects in clinical and immunological characteristics...
December 2016: Medicine (Baltimore)
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