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https://www.readbyqxmd.com/read/28815344/long-term-survival-after-hematopoietic-stem-cell-transplantation-for-complete-stat1-deficiency
#1
Samuele Naviglio, Elena Soncini, Donatella Vairo, Arnalda Lanfranchi, Raffaele Badolato, Fulvio Porta
PURPOSE: Complete signal transducer and activator of transcription 1 (STAT1) deficiency is a rare autosomal recessive condition characterized by impairment of intracellular signaling from both type I and type II interferons (IFN). Affected patients are prone to early severe mycobacterial and viral infections, which usually result in death before 18 months of age. We previously reported a patient affected by complete STAT1 deficiency who underwent hematopoietic stem cell transplantation (HSCT)...
August 16, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28803714/building-capacity-for-active-surveillance-of-vaccine-adverse-events-in-the-americas-a-hospital-based-multi-country-network
#2
Pamela Bravo-Alcántara, Silvia Pérez-Vilar, Helvert Felipe Molina-León, Miriam Sturkenboom, Steven Black, Patrick L F Zuber, Christine Maure, Jose Luis Castro
New vaccines designed to prevent diseases endemic in low and middle-income countries are being introduced without prior utilization in countries with robust vaccine pharmacovigilance systems. Our aim was to build capacity for active surveillance of vaccine adverse events in the Americas. We describe the implementation of a proof-of-concept study for the feasibility of an international collaborative hospital-based active surveillance system for vaccine safety. The study was developed and implemented in 15 sentinel sites located in seven countries of the region of the Americas, under the umbrella of the World Health Organization (WHO) Global Vaccine Safety Initiative...
August 10, 2017: Vaccine
https://www.readbyqxmd.com/read/28798661/multisensory-based-rehabilitation-approach-translational-insights-from-animal-models-to-early-intervention
#3
REVIEW
Giulia Purpura, Giovanni Cioni, Francesca Tinelli
Multisensory processes permit combinations of several inputs, coming from different sensory systems, allowing for a coherent representation of biological events and facilitating adaptation to environment. For these reasons, their application in neurological and neuropsychological rehabilitation has been enhanced in the last decades. Recent studies on animals and human models have indicated that, on one hand multisensory integration matures gradually during post-natal life and development is closely linked to environment and experience and, on the other hand, that modality-specific information seems to do not benefit by redundancy across multiple sense modalities and is more readily perceived in unimodal than in multimodal stimulation...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28796692/reticulated-purpura-in-the-abdomen-of-a-severely-ill-patient
#4
Caio Formiga, Karla Pacheco, Thiago Jeunon
No abstract text is available yet for this article.
August 3, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28796639/development-and-validation-of-a-multivariable-prediction-rule-for-detecting-a-severe-acquired-adamts13-activity-deficiency-in-patients-with-thrombotic-microangiopathies
#5
Jorge M Nieto, Félix De La Fuente-Gonzalo, Fernando A González, Ana Villegas, Rafael Martínez, Manuel E Fuentes, Paloma Ropero
BACKGROUND: Thrombotic microangiopathies (TMAs) are a group of diseases that have different aetiologies and treatments, but a clinical differential diagnosis remains difficult. Among TMAs, thrombotic thrombocytopenic purpura (TTP) is characterised by a severe ADAMTS13 functional deficiency. However, assays exploring ADAMTS13 activity are limited to some specialised laboratories. Our objective was to develop and validate a diagnostic method for TTP in adult patients with TMA. METHODS: We generated a multivariable model (four predictors) on a cohort of 174 TMA patients in order to predict an ADAMTS13 activity deficiency (AUC of 0...
August 10, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28795238/efficacy-and-safety-of-sofosbuvir-based-interferon-free-therapy-the-management-of-rheumatologic-extrahepatic-manifestations-associated-with-chronic-hepatitis-c%C3%A2-virus-infection
#6
A A Shahin, H S Zayed, M Said, S A Amer
BACKGROUND: The use of pegylated interferon alpha (IFN) has been of concern in chronic hepatitis C virus (HCV) patients with rheumatologic extrahepatic manifestations (EHM) due to the immunostimulatory effects of IFN. AIM: To study the efficacy and safety of sofosbuvir-based, IFN-free antiviral therapy in chronic HCV patients with rheumatologic EHM. MATERIAL AND METHODS: Group A included 24 patients with arthropathy (arthralgia or arthritis, n = 15) or vasculitis (n = 9) who received sofosbuvir and ribavirin (n = 17) or sofosbuvir and simeprevir (n = 7)...
August 9, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28794554/mucocutaneous-manifestations-in-patients-with-rheumatoid-arthritis-a-cross-sectional-study-from-eastern-india
#7
Sudip Kumar Ghosh, Debabrata Bandyopadhyay, Surajit Kumar Biswas, Ivoreen Darung
BACKGROUND: Cutaneous manifestations are fairly common in rheumatoid arthritis (RA) and they can help in early diagnosis, prompt treatment, and hence reduced morbidity from the disease. AIMS: The objective of the present study was to find out the different patterns of dermatoses in a group of patients with RA from Eastern India. METHODOLOGY: Consecutive patients fulfilling the American Rheumatism Association 1987 revised criteria for the classification of RA and who had different dermatoses were included in this cross-sectional study done over a period of 8 years in a tertiary care hospital in Eastern India...
July 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28793953/the-spectrum-of-biopsy-proven-secondary-glomerular-diseases-a-cross-sectional-study-in-china%C3%A2
#8
Ping Wang, Lijun Tang, Jing Yao, Hong Su, Yipeng Liu, Xianglei Kong, Wenbin Li, Meiyu Cui, Qing Sun, Junhui Zhen, Dongmei Xu
AIMS: The distribution of the spectrum of chronic kidney disease (CKD) has been found to vary with time. Limited information is available regarding the changing spectrum of secondary glomerular diseases (SGDs). To further investigate changes in the spectrum of SGDs, we performed a cross-sectional study. METHODS: From June 2010 to May 2015, 5,935 patients from 37 hospitals in Shandong Province were involved in this study. The study was divided into five periods according to 1-year intervals...
August 10, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28793154/purpura-annularis-telangiectodes-of-majocchi-associated-with-the-initiation-and-rechallenge-of-apremilast-for-psoriasis-vulgaris
#9
Jaclyn A Kalik, Harvey Friedman, Mark A Bechtel, Alejandro A Gru, Benjamin H Kaffenberger
No abstract text is available yet for this article.
August 9, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28792176/chikungunya-fever-presenting-as-life-threatening-thrombotic-thrombocytopenic-purpura
#10
Vimal Kumar, Rujul Jain, Arvind Kumar, Neeraj Nischal, Pankaj Jorwal, Manish Soneja, Sudheer Arava, Naveet Wig
It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic thrombocytopenic purpura leading to multiple areas of skin necrosis, peripheral digital gangrene, haemolytic anemia, renal failure and severe thrombocytopenia with bleeding. This complication was most likely due to inhibitor autoantibody formation against ADAMTS13 triggered by chikungunya virus leading to thrombotic thrombocytopenic purpura...
July 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#11
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28781836/urticaria-vasculitis-in-a-child-a-case-report-and-literature-review
#12
Adrián Imbernón-Moya, Elena Vargas-Laguna, Fernando Burgos, Eva Fernández-Cogolludo, Antonio Aguilar-Martínez, Miguel Ángel Gallego-Valdés
Annular urticarial lesions in a child must establish a main differential diagnosis with urticaria multiforme, common urticaria, acute hemorrhagic edema of infancy, erythema marginatum, erythema annulare centrifugum, annular erythema in childhood, erythema multiforme, Sweet's syndrome, Schönlein-Henoch purpura, erythematosus lupus, several systemic vasculitis, and serum sickness.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28781225/a-case-report-of-a-child-with-purpura-severe-abdominal-pain-and-hematochezia
#13
Werner Keenswijk, Katrien Van Renterghem, JohanVande Walle
No abstract text is available yet for this article.
August 3, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28780041/quinine-induced-thrombotic-microangiopathy-a-report-of%C3%A2-19%C3%A2-patients
#14
Evaren E Page, Dustin J Little, Sara K Vesely, James N George
BACKGROUND: Quinine can cause diverse and severe immune-mediated adverse reactions, including thrombotic microangiopathy (TMA). Our objective was to describe the presenting features and long-term outcomes of patients with quinine-induced TMA. STUDY DESIGN: A case series of 19 patients with quinine-induced TMA treated with plasma exchange. SETTING & PARTICIPANTS: Patients with quinine-induced TMA initially suspected of having thrombotic thrombocytopenic purpura (TTP) were identified among patients enrolled in the Oklahoma TTP-Hemolytic Uremic Syndrome Registry...
August 3, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28774273/thrombotic-microangiopathy-associated-with-valproic-acid-toxicity
#15
Sean A Hebert, Timothy P Bohan, Christian L Erikson, Rita D Swinford
BACKGROUND: Thrombotic microangiopathy (TMA) is a serious, sometimes life-threatening disorder marked by the presence of endothelial injury and microvascular thrombi. Drug-induced thrombotic microangiopathy (DI-TMA) is one specific TMA syndrome that occurs following drug exposure via drug-dependent antibodies or direct tissue toxicity. Common examples include calcineurin inhibitors Tacrolimus and Cyclosporine and antineoplastics Gemcitabine and Mitomycin. Valproic acid has not been implicated in DI-TMA...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28774183/annals-express-altered-mean-platelet-volume-in-children-with-henoch-schonlein-purpura-and-its-association-with-disease-activity
#16
Xiang Shi, Wen Chao Li, Li Jun Mo, Xiao Hong Li, Yu Zhen Luo, Liu Qun Qin, Zheng Yang, Wu Ning Mo
BACKGROUND: Henoch-Schonlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. A review of the literature has suggested a correlation between mean platelet volume (MPV) and several inflammatory disorders. However, to the best of our knowledge, any potential correlation between MPV and HSP has not been reported in the literature. Therefore, our study aimed to evaluate the role of MPV levels in patients with HSP. METHODS: This study included 97 children with HSP and 120 healthy individuals as controls...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28771805/cutaneous-small-vessel-vasculitis-in-two-children-with-inflammatory-bowel-disease-case-series-and-review-of-the-literature
#17
Tina Ho, Lauren A V Orenstein, Markus D Boos, Kevin P White, Nicole Fett
Cutaneous small-vessel vasculitis (CSVV) is an infrequent manifestation of pediatric inflammatory bowel disease (IBD). We report two cases of CSVV associated with ulcerative colitis, review the literature, and discuss the diagnostic evaluation of children who present with CSVV and abdominal pain. After excluding more common causes of CSVV and abdominal pain in children, including immunoglobulin A vasculitis (previously Henoch-Schönlein purpura), infectious colitis, and drug-induced vasculitis, alternative diagnoses such as CSVV secondary to IBD or systemic vasculitis with gastrointestinal involvement must be considered...
August 2, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28770879/incidence-and-pattern-of-thrombocytopenia-in-cardiac-surgery-patients
#18
Mohammad Hamid, Mohammad Irfan Akhtar, Hamid Iqil Naqvi, Khalid Ahsan
OBJECTIVE: To observe the incidence and pattern of thrombocytopenia in cardiac surgery patients. METHODS: This prospective, cohort study was conducted at the Aga Khan University Hospital, Karachi, from November 2014 to April 2015, and comprised adult cardiac patients. Patients with platelet count less than 150,000 x 109/L, history of malignancy, immune thrombocytopenic purpura and on chemo or radiotherapy were excluded. All information including demographics, platelet count, heparin doses, total cardiopulmonary bypass time, cross-clamp time, blood products transfused, any thromboembolic complication and the presence of infection were recorded on a pre-designed proforma...
July 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28768626/pathophysiology-of-thrombotic-thrombocytopenic-purpura
#19
J Evan Sadler
The discovery of ADAMTS13 revolutionized our approach to thrombotic thrombocytopenic purpura (TTP). Inherited or acquired ADAMTS13 deficiency allows the unrestrained growth of microthrombi that are composed of von Willebrand factor (VWF) and platelets, which account for the thrombocytopenia, hemolytic anemia, schistocytes and tissue injury that characterize TTP. Most patients with acquired TTP respond to a combination of plasma exchange and rituximab, but some die or acquire irreversible neurological deficits before they can respond, and relapses can occur unpredictably...
August 2, 2017: Blood
https://www.readbyqxmd.com/read/28767914/comparison-of-enterovirus-detection-in-cerebrospinal-fluid-with-bacterial-meningitis-score-in-children
#20
Frederico Ribeiro Pires, Andréia Christine Bonotto Farias Franco, Alfredo Elias Gilio, Eduardo Juan Troster
Objective: To measure the role of enterovirus detection in cerebrospinal fluid compared with the Bacterial Meningitis Score in children with meningitis. Methods: A retrospective cohort based on analysis of medical records of pediatric patients diagnosed as meningitis, seen at a private and tertiary hospital in São Paulo, Brazil, between 2011 and 2014. Excluded were patients with critical illness, purpura, ventricular shunt or recent neurosurgery, immunosuppression, concomitant bacterial infection requiring parenteral antibiotic therapy, and those who received antibiotics 72 hours before lumbar puncture...
April 2017: Einstein
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