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https://www.readbyqxmd.com/read/29332223/long-term-efficacy-of-partial-splenic-embolization-for-the-treatment-of-steroid-resistant-chronic-immune-thrombocytopenia
#1
Emi Togasaki, Naomi Shimizu, Yuhei Nagao, Chika Kawajiri-Manako, Ryoh Shimizu, Nagisa Oshima-Hasegawa, Tomoya Muto, Shokichi Tsukamoto, Shio Mitsukawa, Yusuke Takeda, Naoya Mimura, Chikako Ohwada, Masahiro Takeuchi, Emiko Sakaida, Tohru Iseki, Hideyuki Yoshitomi, Masayuki Ohtsuka, Masaru Miyazaki, Chiaki Nakaseko
Thrombopoietin-receptor agonists have been recently introduced for a second-line treatment of immune thrombocytopenia (ITP). Splenectomy has tended to be avoided because of its complications, but the response rate of splenectomy is 60-80% and it has still been considered for steroid-refractory ITP. We performed partial splenic embolization (PSE) as an alternative to splenectomy. Between 1988 and 2013, 91 patients with steroid-resistant ITP underwent PSE at our hospital, and we retrospectively analyzed the efficacy and long-term outcomes of PSE...
January 13, 2018: Annals of Hematology
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
#2
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/29327472/mesenchymal-stem-cell-deficiency-influences-megakaryocytopoiesis-through-the-tnfaip3-nf-%C3%AE%C2%BAb-smad-pathway-in-patients-with-immune-thrombocytopenia
#3
Yun He, Lin-Lin Xu, Fei-Er Feng, Qian-Ming Wang, Xiao-Lu Zhu, Chen-Cong Wang, Jia-Min Zhang, Hai-Xia Fu, Lan-Ping Xu, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang
Immune thrombocytopenia (ITP) is an autoimmune disease. Mesenchymal stem cells (MSCs) play important roles in the physiology and homeostasis of the haematopoietic system, including supporting megakaryocytic differentiation from CD34+ haematopoietic progenitor cells. Tumour necrosis factor alpha-induced protein 3 (TNFAIP3, also termed A20) plays a key role in terminating NF-κB signalling. Human genetic studies showed that the polymorphisms of the TNFAIP3 gene may contribute to ITP susceptibility. In this study, we showed a significant decrease in TNFAIP3 and increase in NF-κB/SMAD7 in ITP-MSCs...
January 12, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29327325/circulating-level-of-th17-cells-is-associated-with-sensitivity-to-glucocorticoids-in-patients-with-immune-thrombocytopenia
#4
YiChan Zhang, TingTing Ma, Xuan Zhou, JunHao Chen, Juan Li
Glucocorticoids are a widely recognized first-line therapy for immune thrombocytopenia (ITP). However, some patients are unresponsive to glucocorticoid therapy for reasons that remain unclear. Accumulating evidence suggests that CD4+ T-cell abnormalities play a crucial role in the development of ITP. In the present study, we investigated peripheral blood CD4+ T cells, Th17-associated cytokines, and the mRNA expression level of Th17 transcription factor-RORγt-in patients with newly-diagnosed ITP before glucocorticoid therapy...
January 11, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29326868/essential-thrombocytemia-following-immune-thrombocytopenia-with-jak2v617f-mutation
#5
Giovanni Caocci, Sandra Atzeni, Monica Usai, Giorgio La Nasa
JAK2V617F mutation is found in about 60% of cases of essential thrombocytemia (ET) and represents a driving mutation. Immune thrombocytopenia (ITP) is an autoimmune disease characterized by a low platelet (PLT) count. So far, only 2 reports described ET following ITP. For the first time we analyzed in a patient the JAK2V617F allele burden at ITP onset occurred 13 years before the ET diagnosis and found the presence of a small clone JAK2V617F positive clone (3%) raised to 27% in the following years. The association of ET and ITP could suggest similar pathogenetic mechanisms that should be further investigated...
2018: Leukemia Research Reports
https://www.readbyqxmd.com/read/29317351/a-new-physiological-model-for-studying-the-effect-of-chest-compression-and-ventilation-during-cardiopulmonary-resuscitation-the-thiel-cadaver
#6
Emmanuel Charbonney, Stéphane Delisle, Dominique Savary, Gilles Bronchti, Marceau Rigollot, Adrien Drouet, Bilal Badat, Paul Ouellet, Patrice Gosselin, Alain Mercat, Laurent Brochard, Jean-Christophe M Richard
BACKGROUND: Studying ventilation and intrathoracic pressure (ITP) induced by chest compressions (CC) during Cardio Pulmonary Resuscitation is challenging and important aspects such as airway closure have been mostly ignored. We hypothesized that Thiel Embalmed Cadavers could constitute an appropriate model. METHODS: We assessed respiratory mechanics and ITP during CC in 11 cadavers, and we compared it to measurements obtained in 9 out-of-hospital cardiac arrest patients and to predicted values from a bench model...
January 6, 2018: Resuscitation
https://www.readbyqxmd.com/read/29313460/thrombopoietin-and-its-receptor-expression-in-pediatric-patients-with-chronic-immune-thrombocytopenia
#7
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/29308775/a-case-of-immune-thrombocytopenic-purpura-with-prolonged-aptt-time-a-clotter-hidden-in-a-bleeder
#8
M H Lee, P J Khoo, L T Gew, C F Ng
We report the case of a 23-year-old woman who presented with prolonged menstruation and multiple bruises on the limbs and trunk. Investigations revealed severe thrombocytopenia and deranged coagulation profile with markedly prolonged activated partial thromboplastin time (aPTT). Lupus anticoagulant, anti-cardiolipin antibody and anti-beta-2-glycoprotein 1 antibody were positive. She was diagnosed with Immune Thrombocytopenic Purpura (ITP) with positive antiphospholipid antibody serology and given a course of intravenous methylprednisolone and tapering doses of oral prednisolone...
December 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29307971/cerebral-venous-thrombosis-after-intravenous-immunoglobulin-therapy-in-immune-thrombocytopenic-purpura
#9
Joe James, P V Shiji, Chandni Radhakrishnan
A common misconception is that immune thrombocytopenic purpura (ITP) causes only bleeding diathesis. From this case vignette of a young male with ITP who had cerebral venous thrombosis, we highlight the importance of considering venous thrombosis in such patients when they present with focal cerebral signs.
December 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/29304534/platelet-function-in-itp-independent-of-platelet-count-is-consistent-over-time-and-is-associated-with-both-current-and-subsequent-bleeding-severity
#10
A L Frelinger, R F Grace, A J Gerrits, S L Carmichael, E E Forde, A D Michelson
BACKGROUND:  Treatment decisions for patients with immune thrombocytopenia (ITP) are difficult because patients with similarly low platelet counts differ in their bleeding tendency. We recently reported that platelet function tests, independent of platelet count, are associated with concurrent bleeding severity, suggesting that these tests may be useful indicators of future bleeding in ITP. OBJECTIVES:  To test this hypothesis, we evaluated the consistency of these platelet function tests over time and their association with subsequent bleeding severity...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29297977/the-association-between-idiopathic-thrombocytopenic-purpura-and-cardiovascular-disease-a-retrospective-cohort-study
#11
Joht Singh Chandan, Tom Thomas, Sophie Lee, Tom Marshall, Brian Willis, Krishnarajah Nirantharakumar
BACKGROUND: Idiopathic Thrombocytopenic Purpura (ITP) is classically characterized by a transient or persistent decrease of platelet count. Mortality is higher in the ITP population than the general population, with a possible association to increased cardiovascular disease (CVD). OBJECTIVES: The objective was to assess the strength of the association between ITP and CVD, with a secondary aim to assess the impact of splenectomy on CVD. METHODS: A population-based retrospective, open cohort study using clinical codes was performed using data from 6,591 patients with ITP and 24,275 randomly matched controls (up to 1:4 ratio matched by age, sex, body mass index and smoking status)...
January 3, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29297955/predictive-factors-for-response-to-ivig-in-children-with-itp
#12
Yukiko Higashide, Tsukasa Hori, Yuko Yoto, Hiroyuki Kabutoya, Saho Honjo, Yoshiyuki Sakai, Masanori Nojima, Minami Yoda, Masaki Yamamoto, Hiroyuki Tsutsumi
BACKGROUND: Immune thrombocytopenic purpura (ITP) is commonly treated with intravenous immunoglobulin (IVIG). METHODS: We retrospectively evaluated whether pretreatment clinical and laboratory findings could predict the short- and long-term response to IVIG. RESULTS: Short-term response was estimated by platelet counts 2 weeks after IVIG, and long-term response was assessed by thrombocytopenia-free survival (TFS). TFS was defined as the probability of survival without treatment failure after initial IVIG, such as relapse, requiring additional therapeutic interventions, or progressing to chronic ITP...
January 3, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29296891/misdiagnosis-of-primary-immune-thrombocytopenia-and-frequency-of-bleeding-lessons-from-the-mcmaster-itp-registry
#13
Donald M Arnold, Ishac Nazy, Rumi Clare, Anushka M Jaffer, Brandon Aubie, Na Li, John G Kelton
Nonspecific diagnostic criteria and uncertain estimates of severe bleeding events are fundamental gaps in knowledge of primary immune thrombocytopenia (ITP). To address these issues, we created the McMaster ITP Registry. In this report, we describe the methodology of the registry, the process for arriving at the diagnosis, and the frequency of bleeding. Consecutive patients with platelets <150 × 109/L from a tertiary hematology clinic in Canada were eligible. Patients completed a panel of investigations and were managed per clinical need...
November 28, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296878/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#14
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/29296876/itp-and-thrombosis-an-intriguing-association
#15
Francesco Rodeghiero
No abstract text is available yet for this article.
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295846/splenectomy-for-immune-thrombocytopenia-down-but-not-out
#16
Shruti Chaturvedi, Donald M/ Arnold, Keith R McCrae
Splenectomy is an effective therapy for steroid-refractory or dependent immune thrombocytopenia (ITP). With the advent of medical alternatives such as rituximab and thrombopoietin receptor antagonists (TPO-RA), the use of splenectomy has declined and is generally reserved for patients that fail multiple medical therapies. Splenectomy removes the primary site of platelet clearance and autoantibody production, and offers the highest rate of durable response (50-70%) compared to other ITP therapies. However, there are no reliable predictors of splenectomy response, and long-term risks of infection and cardiovascular complications must be considered...
January 2, 2018: Blood
https://www.readbyqxmd.com/read/29289988/rerouting-the-internal-thoracic-pedicle-a-novel-solution-for-maxillofacial-reconstruction-in-vessel-depleted-situations-a-preliminary-anatomic-study
#17
François Morel, Frédéric Crampon, Jérôme Adnot, Pierre-Yves Litzler, Fabrice Duparc, Olivier Trost
PURPOSE: Microsurgical reconstruction in a vessel-depleted neck is a challenge due to the lack of reliable vessels in or nearby the host site. The use of the internal thoracic pedicle (ITP) by rib section or sparring is a limited option due to the small length of the pedicle of some flaps. However, in cardiac surgery, the internal thoracic artery (ITA) is widely used for myocardial revascularization, providing a long and versatile pedicle. We aimed at determining precise anatomical bases for the use of the ITP, approached by sternotomy and rerouted in the neck, as recipient vessels for free-flap facial reconstructions...
December 30, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/29288170/atorvastatin-enhances-bone-marrow-endothelial-cell-function-in-corticosteroid-resistant-immune-thrombocytopenia-patients
#18
Yuan Kong, Xie-Na Cao, Xiao-Hui Zhang, Min-Min Shi, Yue-Yun Lai, Yu Wang, Lan-Ping Xu, Ying-Jun Chang, Xiao-Jun Huang
The pathogenesis of corticosteroid-resistant immune thrombocytopenia (ITP), a clinically challenging condition in which patients exhibit either no response to corticosteroids or are corticosteroid-dependent, remains poorly understood. Murine studies suggest that bone marrow (BM) endothelial progenitor cells (EPCs) play a crucial role in regulating megakaryocytopoiesis. However, little is known regarding the number and function of BM EPCs or how to improve impaired BM EPCs in corticosteroid-resistant ITP patients...
December 29, 2017: Blood
https://www.readbyqxmd.com/read/29287794/effect-of-glucocorticoid-treatment-on-baff-and-april-expression-in-patients-with-immune-thrombocytopenia-itp
#19
Julian Kamhieh-Milz, Nuha Ghosoun, Viktor Sterzer, Abdulgabar Salama
Immune thrombocytopenic purpura (ITP) is an idiopathic bleeding disorder. B cell activating factor (BAFF) and 'A proliferation-inducing ligand' (APRIL) have regulatory effects on B and T cells and may represent relevant factors in the pathogenesis of ITP. Serum levels and gene expression were investigated in ITP patients. Both BAFF and APRIL serum levels were significantly elevated in active ITP. However, gene expression analysis revealed both factors to have a tendency toward downregulation. Glucocorticoid treatment significantly reduced BAFF but not APRIL serum levels, which may be mediated by differences in transcription factor binding sites...
December 26, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29281994/successful-treatment-of-cerebral-aspergillosis-case-report-of-a-patient-with-t-cell-large-granular-lymphocytic-leukemia-t-lgl
#20
Amin T Turki, Jassin Rashidi-Alavijeh, Jan Dürig, Guido Gerken, Peter-Michael Rath, Oliver Witzke
BACKGROUND: Invasive aspergillosis involving patients with neutropenia or severe immunosuppression, such as patients with hematologic malignancies is associated with high mortality. Patients with T-cell large granular lymphocytic leukemia (T-LGL) on the other hand are considered to be less vulnerable for severe opportunistic fungal infection as their course of disease is chronic and marked by less violent cytopenia then in e.g. Aplastic Anemia. Only neutropenia is regarded as independent risk factor for severe opportunistic infection in T-LGL patients...
December 28, 2017: BMC Infectious Diseases
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