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https://www.readbyqxmd.com/read/29330742/henoch-sch%C3%A3-nlein-purpura-nephritis-initial-risk-factors-and-outcomes-in-a-latin-american-tertiary-center
#1
Izabel M Buscatti, Beatriz B Casella, Nadia E Aikawa, Andrea Watanabe, Sylvia C L Fahrat, Lucia M A Campos, Clovis Artur Silva
The objective of this study was to evaluate prevalence, initial risk factors, and outcomes in Henoch-Schönlein purpura nephritis (HSPN) patients in Latin America. Two hundred ninety-six patients (validated EULAR/PRINTO/PRES HSP criteria) were assessed by demographic data, clinical/laboratorial involvements, and treatments in the first 3 months after diagnosis. They were followed-up in a Latin American tertiary center and were divided in two groups: with and without nephritis. Persistent non-nephrotic proteinuria, nephrotic proteinuria, and acute/chronic kidney injury were also systematically evaluated at 1, 5, 10, and 15 years after diagnosis...
January 13, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29328034/characteristics-of-articular-syndrome-in-systemic-vasculitis
#2
O Syniachenko, O Khaniukov, Ye Yehudina, H Taktashov, K Gashynova
The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29324461/efficacy-of-treatment-immune-thrombocytopenic-purpura-in-pregnancy-with-corticosteroids-and-intravenous-immunoglobulin-a-prospective-follow-up-of-suggested-practice
#3
Gisela Wegnelius, Katarina Bremme, Pelle G Lindqvist
: The current study is performed to assess a routine for treatment of immune thrombocytopenic purpura in pregnancy. A prospective programme for monitoring and treatment with intravenous immunoglobulin or cortisone in pregnancies with immune thrombocytopenic purpura was suggested to all delivery units in Sweden. Treatment should be avoided if platelet counts were more than 20 × 10/l during pregnancy with no bleeding complications and with a target of 100 × 10/l at delivery. Descriptive statistics and logistic regression analysis were used...
January 10, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29314277/erasmus-syndrome-associated-with-an-immune-thrombocytopenic-purpura
#4
Maxime Fouchard, Brigitte Pan Petesch, Claire Abasq-Thomas, Alain Saraux, Laurent Misery, Emilie Brenaut
We report the observation of a 57-year-old patient followed for an Erasmus syndrome (a systemic sclerosis developing secondary to silica exposure) (1) diagnosed in 2002 after being exposed as a dental technician, for whom a severe thrombocytopenia was discovered. He suffered from a pulmonary fibrosis, polyarthralgia, esophageal dysphagia and cutaneous involvement such as finger ulcerations and sclerodactylia. The treatment was made of nifedipine 30mg/day, esomeprazole 20mg/day and bosentan 25 mg twice a day...
January 5, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29313056/generalized-purpura-as-an-atypical-skin-manifestation-of-adult-onset-still-s-disease-in-a-patient-with-beh%C3%A3-et-s-disease
#5
Chika Omigawa, Takashi Hashimoto, Takaaki Hanafusa, Takeshi Namiki, Ken Igawa, Hiroo Yokozeki
No abstract text is available yet for this article.
January 9, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29308775/a-case-of-immune-thrombocytopenic-purpura-with-prolonged-aptt-time-a-clotter-hidden-in-a-bleeder
#6
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/29308771/clinical-features-and-prognostic-factors-of-cutaneous-vasculitis-among-dermatology-patients-in-johor-bahru-malaysia
#7
S Latha, S E Choon, K E Tey, Y N Chee
BACKGROUND: Cutaneous vasculitis is common, yet the risk factors for its chronicity have not been established. OBJECTIVE: To describe the clinical spectrum and identify risk factors for chronicity of cutaneous vasculitis. METHODS: Retrospective data analysis of 275 patients diagnosed with cutaneous vasculitis from January 2008 to December 2013. RESULTS: The mean age was 33.7 (±17.89) years, with female predominance...
December 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29308213/case-series-of-3-patients-diagnosed-with-atypical-hemolytic-uremic-syndrome-successfully-treated-with-steroids-plasmapheresis-and-rituximab
#8
Jeffery M Patterson, Lauren Bolster, Loree Larratt
Rationale: Atypical hemolytic uremic syndrome, which has a high probability of chronic kidney disease, morbidity, and mortality, needs to be promptly recognized when patients present with microangiopathic hemolysis. Presenting Concerns of the Patient: Three patients present with laboratory parameters consistent with a thrombotic microangiopathy. With a suspected diagnosis of thrombotic thrombocytopenic purpura, steroids with plasmapheresis were initiated. Diagnoses: With ADAMTS13 levels reported normal, the suspected diagnoses were reevaluated...
2018: Canadian Journal of Kidney Health and Disease
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/29304917/ultraviolet-purpura-in-iga-vasculitis
#10
Daisuke Tsukui, Hajime Kono
No abstract text is available yet for this article.
January 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29304523/insights-into-3d-structure-of-adamts13-a-stepping-stone-towards-novel-therapeutic-treatment-of-thrombotic-thrombocytopenic-purpura
#11
Bogac Ercig, Kanin Wichapong, Chris P M Reutelingsperger, Karen Vanhoorelbeke, Jan Voorberg, Gerry A F Nicolaes
ADAMTS13 (A D: isintegrin A: nd M: etalloprotease with a T: hromboS: pondin type-1 motif, member 13: ) and von Willebrand factor (VWF) can be considered as scale weights which control platelet adhesion during primary haemostasis. In a very uncommon condition designated thrombotic thrombocytopenic purpura (TTP), functional absence of ADAMTS13 tips the balance toward VWF-mediated platelet adhesion in the microcirculation. TTP is associated with a high mortality and arises from either a congenital or acquired autoimmune deficiency of the plasma enzyme ADAMTS13...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29302735/a-new-human-derived-acellular-dermal-matrix-for-breast-reconstruction-available-for-the-european-market-preliminary-results
#12
Secondo Folli, Annalisa Curcio, Davide Melandri, Elena Bondioli, Nicola Rocco, Giuseppe Catanuto, Fabio Falcini, Valeria Purpura, Matteo Mingozzi, Federico Buggi, Francesco Marongiu
INTRODUCTION: The introduction of acellular dermal matrices (ADMs) contributed to the growing diffusion of direct-to-implant breast reconstruction (DTI-BR) following mastectomy for breast cancer. According to specific legislations, European specialists could not benefit from the use of human-derived ADMs, even though most evidence in the literature are available for this kind of device, showed optimal outcomes in breast reconstruction. The Skin Bank of the Bufalini Hospital (Cesena, Italy) obtained in 2009 the approval for the production and distribution of a new human cadaver-donor-derived ADM (named with the Italian acronym, MODA, for matrice omologa dermica acellulata) from the Italian National Transplant Center and National Health Institute...
January 4, 2018: Aesthetic Plastic Surgery
https://www.readbyqxmd.com/read/29297977/the-association-between-idiopathic-thrombocytopenic-purpura-and-cardiovascular-disease-a-retrospective-cohort-study
#13
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
#14
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/29296878/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#15
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/29296863/management-of-thrombotic-thrombocytopenic-purpura-without-plasma-exchange-the-jehovah-s-witness-experience
#16
James N George, Steven A Sandler, Joanna Stankiewicz
TTP in Jehovah's Witness patients has been managed successfully without PEX.This experience, plus new TTP treatments, may make it possible for patients who are not Jehovah's Witnesses to avoid PEX in the future.
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296854/cyclosporine-or-steroids-as-an-adjunct-to-plasma-exchange-in-the-treatment-of-immune-mediated-thrombotic-thrombocytopenic-purpura
#17
Spero R Cataland, Peter J Kourlas, Shangbin Yang, Susan Geyer, Leslie Witkoff, Haiwa Wu, Camila Masias, James N George, Haifeng M Wu
Although steroids are routinely used as an adjunct to plasma exchange (PEX) therapy in the treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP), limited data regarding their efficacy or effect on ADAMTS13 biomarkers are available. We report the results of a prospective, randomized study that compared the effectiveness of prednisone or cyclosporine (CSA) as adjuncts to PEX in the treatment of iTTP. A total of 26 of the planned 72 subjects were enrolled and treated from November 2007 until February 2014 before the study was halted after a planned interim analysis...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296757/rituximab-prophylaxis-to-prevent-thrombotic-thrombocytopenic-purpura-relapse-outcome-and-evaluation-of-dosing-regimens
#18
John-Paul Westwood, Mari Thomas, Ferras Alwan, Vickie McDonald, Sylvia Benjamin, William A Lester, Gillian C Lowe, Tina Dutt, Quentin A Hill, Marie Scully
Acute antibody-mediated thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with high morbidity and mortality. Rituximab is highly effective as prophylaxis in patients at risk of acute TTP relapse, but the ideal dosing regimen is unknown. A multicenter retrospective cohort study evaluated outcomes of patients given rituximab prophylaxis to prevent TTP relapse. Rituximab was given in 76 episodes to 45 patients (34 women and 11 men). Four once-per-week infusions of standard- (375 mg/m2 [24 episodes]), reduced- (200 mg [19 episodes]), and intermediate- (500 mg [17 episodes]) dose rituximab were given; in the remaining 16 episodes, patients received 100 to 1000 mg rituximab in 1 to 5 doses...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296701/thrombotic-thrombocytopenic-purpura-diagnostic-criteria-clinical-features-and-long-term-outcomes-from-1995-through-2015
#19
Evaren E Page, Johanna A Kremer Hovinga, Deirdra R Terrell, Sara K Vesely, James N George
Our objective was to describe new observations from the Oklahoma Thrombotic Thrombocytopenic Purpura (TTP) Registry experience (November 1995 through December 2015) on the diagnosis of TTP along with patients' clinical features and their outcomes. Among 363 patients with an initial episode of clinically suspected TTP, the diagnosis of TTP was supported by both ADAMTS13 activity <10% and clinical features in 78 patients (21%). ADAMTS13 activity was measured in all 363 patients by 2 methods: fluorescence resonance energy transfer (FRET) and immunoblotting (IB)...
April 11, 2017: Blood Advances
https://www.readbyqxmd.com/read/29287794/effect-of-glucocorticoid-treatment-on-baff-and-april-expression-in-patients-with-immune-thrombocytopenia-itp
#20
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
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