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https://www.readbyqxmd.com/read/29149705/toll-like-receptor-4-deficiency-increases-resistance-in-sepsis-induced-immune-dysfunction
#1
Chao Cao, Yanfen Chai, Songtao Shou, Jun Wang, Ying Huang, Tao Ma
Sepsis constitutes a serious life-threatening syndrome associated with complications of deregulated inflammatory response against endotoxin/lipopolysaccharide (LPS)-mediated severe infection. Toll-like receptor 4 (TLR4) plays a critical role in the activation of innate immunity through recognition of LPS. However, the impact of TLR4 signaling on the development of sepsis-induced immune dysfunction remains unclear. The aim of this study was to investigate the effect of TLR4 on regulatory T cells (Tregs) and its potential mechanism...
November 14, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/29149650/iron-chelation-therapy-in-lower-ipss-risk-myelodysplastic-syndromes-which-subtypes-benefit
#2
Shannon A Wong, Heather A Leitch
BACKGROUND: Analyses suggest MDS patients with higher serum ferritin levels (SF) have inferior overall survival (OS), in one study across MDS subtypes. Multiple analyses suggest those with high SF receiving iron chelation therapy (ICT) have superior OS, but which MDS subtypes benefit from ICT remains undefined. METHODS: We performed survival analyses of MDS subtypes by receipt of ICT. RESULTS: 182 MDS were lower IPSS risk and received red blood cell (RBC) transfusions; 63 received ICT...
November 11, 2017: Leukemia Research
https://www.readbyqxmd.com/read/29149393/challenges-in-minor-tbi-and-indications-for-head-ct-in-pediatric-tbi-an-update
#3
Navneet Singh, Ash Singhal
INTRODUCTION: Pediatric head trauma is one of the commonest presentations to emergency departments. Over 90% of such head injuries are considered mild, but still present risk acute clinical deterioration and longer term morbidity. Identifying which children are at risk of clinically important brain injuries remains challenging and much of the data on minor head injuries is based on the adult population. CHALLENGES IN PEDIATRICS: Children, however, are different, both anatomically and in terms of mechanism of injury, to adults and, even within the pediatric group, there are differences with age and stage of development...
October 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29148534/genetic-testing-of-complement-and-coagulation-pathways-in-patients-with-severe-hypertension-and-renal-microangiopathy
#4
Christopher P Larsen, Jon D Wilson, Alejandro Best-Rocha, Marjorie L Beggs, Randolph A Hennigar
A diagnosis of thrombotic microangiopathy on kidney biopsy in a patient presenting with hypertensive emergency has historically elicited the diagnosis of malignant hypertension-associated thrombotic microangiopathy. Recent studies, however, have raised awareness that a number of these patients may actually represent atypical hemolytic uremic syndrome. To further investigate this premise, we performed next-generation sequencing to interrogate the coding regions of 29 complement and coagulation cascade genes associated with atypical hemolytic uremic syndrome in 100 non-elderly patients presenting with severe hypertension, renal failure and a kidney biopsy showing microangiopathic changes limited to the classic accelerated hypertension-associated lesion of arterial intimal edema ('mucoid intimal hyperplasia') in isolation and without accompanying glomerular microthrombi...
November 17, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29148418/infliximab-for-uveitis-of-beh%C3%A3-et-s-syndrome-a-trend-for-earlier-initiation
#5
Gul Guzelant, Didar Ucar, Sinem Nihal Esatoglu, Gulen Hatemi, Yilmaz Ozyazgan, Sebahattin Yurdakul, Emire Seyahi, Hasan Yazici, Vedat Hamuryudan
OBJECTIVES: The prognosis of uveitis in Behçet's syndrome (BS) has improved over decades. Whether this is related to the use of more aggressive management strategies is not known. METHODS: This is a retrospective study of BS patients who received infliximab (IFX) for refractory eye disease between 2003-2015. The patients were divided into two groups according to the date of onset of in IFX treatment as before and after 2013. We compared the two groups in terms of disease characteristics at the onset of IFX treatment and response to treatment...
October 24, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29148415/exacerbation-of-beh%C3%A3-et-s-syndrome-and-familial-mediterranean-fever-with-menstruation
#6
Gul Guzelant, Yesim Ozguler, Sinem Nihal Esatoglu, Guzin Karatemiz, Huri Ozdogan, Sebahattin Yurdakul, Hasan Yazici, Emire Seyahi
OBECTIVES: Menstruation triggers several conditions such as migraine, recurrent aphthous stomatitis and acne vulgaris in healthy individuals. There is evidence that Behçet's syndrome (BS) and familial Mediterranean fever (FMF) may exacerbate during menstruation. The aim is to assess whether BS and FMF patients experience menstrual flares. METHODS: Females of reproductive age with BS and FMF seen consecutively at the outpatient clinic of Cerrahpasa Medical Faculty at Istanbul, as well as apparently healthy hospital workers were studied using a standardised questionnaire...
October 24, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29148264/negative-symptoms-in-first-episode-psychosis-clinical-correlates-and-1-year-follow-up-outcomes-in-london-early-intervention-services
#7
Aikaterini Rammou, Helen L Fisher, Sonia Johnson, Barnaby Major, Nikola Rahaman, Nick Chamberlain-Kent, James M Stone
AIM: Negative symptoms (NS) have been associated with poor outcome and remain difficult to treat in patients with psychosis. This study examined the association of NS with clinical features at first presentation to mental health services for psychosis and with outcomes at 1-year follow-up. METHODS: Clinical data were utilized from five London Early Intervention Services (EIS) included in the MiData audit database. The sample comprised 484 first-episode psychosis patients with complete Positive and Negative Syndrome Scale data at baseline and 1-year follow-up...
November 16, 2017: Early Intervention in Psychiatry
https://www.readbyqxmd.com/read/29148241/peri-procedural-myocardial-infarction-plaques-and-patients-at-risk
#8
EDITORIAL
James A Goldstein
Patients with acute coronary syndrome are at risk for peri-procedural myocardial infarction (PMI) Lipid-laden plaques with thinned disrupted fibrous caps are most prone to PMI PMI is associated with worse outcome over time, though whether such damaging events are causal or instead reflect patients prone to future instability from non-culprit vulnerable plaques requires further delineation.
November 15, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/29148179/clinical-implications-of-de-barsy-syndrome
#9
Lindsay L Warner, David A Olsen, Hugh M Smith
BACKGROUND: De Barsy syndrome is a rare, autosomal recessive syndrome characterized by cutis laxa, progeroid appearance, ophthalmic opacification, skeletal malformations, growth delays, and intellectual disability. AIMS: The aim of this case series is to identify the anesthetic considerations in the clinical management of patients with de Barsy syndrome. METHODS: A retrospective case review from 1968 to 2016 was performed at a single tertiary medical center to identify patients with de Barsy syndrome who underwent anesthesia for diagnostic and surgical procedures...
November 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29148123/donohue-syndrome-a-review-of-literature-case-series-and-anesthetic-considerations
#10
REVIEW
Alana Kirkwood, Grant Stuart, Louise Harding
BACKGROUND: Donohue syndrome is a rare autosomal recessive disorder of insulin resistance, causing a functional defect in insulin receptor function, and affecting the ability of the insulin to bind the receptor. Features include severe hyperinsulinism and fasting hypoglycemia, along with severe failure to thrive despite feeding. An accelerated fasting state results in muscle wasting, decreased subcutaneous fat, and an excess of thick skin. A reduced thoracic diameter is accentuated by increased abdominal distension, which impacts on respiratory reserve...
November 17, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29148000/the-feasibility-and-safety-of-piccs-accessed-via-the-superficial-femoral-vein-in-patients-with-superior-vena-cava-syndrome
#11
Yonghui Wan, Yuxin Chu, Yanru Qiu, Qian Chen, Wei Zhou, Qibin Song
OBJECTIVE: To investigate the feasibility and safety of the peripherally inserted central catheters (PICCs) accessed via the superficial femoral vein in patients with superior vena cava syndrome (SVCS). METHODS: From October 2010 to December 2014, 221 cancer patients with SVCS in our center received real-time ultrasound-guidance of the superficial femoral vein inserted central catheters (FICCs) at the mid-thigh. PICC insertion via upper extremity veins had also been investigated in 2604 cancer patients without SVCS as control...
November 10, 2017: Journal of Vascular Access
https://www.readbyqxmd.com/read/29147930/snp-association-study-in-pms2-associated-lynch-syndrome
#12
Sanne W Ten Broeke, Fadwa A Elsayed, Lisa Pagan, Maran J W Olderode-Berends, Encarna Gomez Garcia, Hans J P Gille, Liselot P van Hest, Tom G W Letteboer, Lizet E van der Kolk, Arjen R Mensenkamp, Theo A van Os, Liesbeth Spruijt, Bert J W Redeker, Manon Suerink, Yvonne J Vos, Anja Wagner, Juul T Wijnen, E W Steyerberg, Carli M J Tops, Tom van Wezel, Maartje Nielsen
Lynch syndrome (LS) patients are at high risk of developing colorectal cancer (CRC). Phenotypic variability might in part be explained by common susceptibility loci identified in Genome Wide Association Studies (GWAS). Previous studies focused mostly on MLH1, MSH2 and MSH6 carriers, with conflicting results. We aimed to determine the role of GWAS SNPs in PMS2 mutation carriers. A cohort study was performed in 507 PMS2 carriers (124 CRC cases), genotyped for 24 GWAS SNPs, including SNPs at 11q23.1 and 8q23.3...
November 17, 2017: Familial Cancer
https://www.readbyqxmd.com/read/29147923/anti-aminoacyl-trna-synthetase-related-myositis-and-dermatomyositis-clues-for-differential-diagnosis-on-muscle-biopsy
#13
Bruna Cerbelli, Annalinda Pisano, Serena Colafrancesco, Maria Gemma Pignataro, Marco Biffoni, Silvia Berni, Antonia De Luca, Valeria Riccieri, Roberta Priori, Guido Valesini, Giulia d'Amati, Carla Giordano
Anti-synthetase syndrome is an autoimmune disease characterized by autoantibodies toward amino acyl-tRNA synthetases (ARS), anti-Jo 1 being the most commonly detected. Muscle damage develops in up to 90% of ARS-positive patients, characterized by a necrotizing myositis restricted to the perifascicular region. This topographic distribution of muscle damage may lead to a misdiagnosis of dermatomyositis (DM) at muscle biopsy. We compared morphological, immunohistochemical, and histoenzymatic features of muscle from ARS-positive patients (n = 11) with those of DM (n = 7) providing clues for their differential diagnosis...
November 16, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29147905/transcriptional-regulation-of-dj-1
#14
Kazuko Takahashi-Niki, Takeshi Niki, Sanae M M Iguchi-Ariga, Hiroyoshi Ariga
DJ-1 is an oncogene and also a causative gene for familial Parkinson's disease. DJ-1 has various functions, and the oxidative status of a cysteine residue at position 106 (C106) is crucial for determination of the activation level of DJ-1.DJ-1 binds to many proteins, including various transcription factors, and acts as a coactivator or corepressor for regulating their target genes without direct binding to DNA, thereby affecting various cell functions. DJ-1-regulating transcription factors and their modified proteins are the androgen receptor and its regulatory proteins, p53; polypyrimidine tract-binding protein-associated splicing factor (PSF); Keap1, an inhibitor for nuclear factor erythroid2-related factor 2 (Nrf2); sterol regulatory element-binding protein (SREBP); Ras-responsive element-binding protein (RREB1); signal transducer and activator of transcription 1 (STAT1); and Nurr1...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29147744/consistency-in-patient-reported-outcome-measures-after-total-knee-arthroplasty-using-patient-specific-instrumentation-a-5-year-follow-up-of-200-consecutive-cases
#15
Daphne A L Schoenmakers, Martijn G M Schotanus, Bert Boonen, Nanne P Kort
PURPOSE: The purpose of this study was to evaluate the 5-year follow-up results of the first 200 total knee arthroplasties (TKA) performed by one high-volume surgeon, using patient-specific information (PSI). To date, there has been no other research into the mid-term follow-up of TKA performed using PSI. MATERIALS AND METHODS: A total of 184 consecutive patients (200 TKA) were evaluated. Outcome measures included implant survival rate, adverse events, and the following patient-reported outcome measures (PROMs); Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), Oxford Knee Score (OKS), Pain Visual Analogue Score (VAS) and EuroQol-5D Score (EQ-5D)...
November 17, 2017: Knee Surgery, Sports Traumatology, Arthroscopy: Official Journal of the ESSKA
https://www.readbyqxmd.com/read/29147669/cervical-intervertebral-disc-degeneration-and-low-cervical-extension-independently-associated-with-a-history-of-stinger-syndrome
#16
Takayoshi Hakkaku, Koichi Nakazato, Koji Koyama, Karina Kouzaki, Kenji Hiranuma
Background: Stinger syndrome frequently occurs in athletes who compete in collision sports. Sharp pain and impairment of neck motion are major symptoms. Cervical intervertebral disc degeneration (CIDD) is also frequently observed in those who compete in collision sports. Purpose/Hypothesis: To investigate whether CIDD and neck functionality are related to a history of stinger syndrome. The hypothesis was that a significant relationship exists between CIDD and neck motion and a history of stinger syndrome in Japanese collegiate football players...
November 2017: Orthopaedic Journal of Sports Medicine
https://www.readbyqxmd.com/read/29147641/severity-of-acute-zika-virus-infection-a-prospective-emergency-room-surveillance-study-during-the-2015-2016-outbreak-in-suriname
#17
Pieter Vroon, Jimmy Roosblad, Fauzia Poese, Jan Wilschut, John Codrington, Stephen Vreden, Rens Zonneveld
Acute Zika virus (ZIKV) infection is usually mild and self-limiting. Earlier, we reported three cases of fatal acute ZIKV infection in patients without typical signs of ZIKV, but rather with criteria of systemic inflammation response syndrome (SIRS). To follow up these observations, we prospectively included patients at the emergency room with temperature instability and suspected to have acute ZIKV infection, SIRS, or both. A total of 102 patients were included of whom N = 21 (21%) were suspected of acute ZIKV infection, N = 56 (55%) of acute ZIKV infection with SIRS criteria, and N = 25 (24%) of SIRS alone...
2017: IDCases
https://www.readbyqxmd.com/read/29147080/effect-of-the-ginger-derivative-6-shogaol-on-ferritin-levels-in-patients-with-low-to-intermediate-1-risk-myelodysplastic-syndrome-a-small-investigative-study
#18
Terry Golombick, Terrence H Diamond, Arumugam Manoharan, Rajeev Ramakrishna, Vladimir Badmaev
Background: Myelodysplastic syndrome (MDS) is a heterogeneous group of clonal stem cell disorders characterized by dysplastic and ineffective hematopoiesis and peripheral cytopenias. Elevated serum ferritin (SF) is often observed in nontransfused, lower risk MDS. It has been reported that ineffective erythropoiesis enhances iron absorption in MDS through downregulation of hepcidin and its prohormones such that SF rises. Aim: To determine the effect of 6-shogaol, a dehydration derivative of ginger, known to have hepatoprotective and chemotherapeutic activity, on 6 early-stage, transfusion-independent patients with MDS, 3 of whom had raised levels of SF...
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/29146941/developmental-excitatory-to-inhibitory-gaba-polarity-switch-is-disrupted-in-22q11-2-deletion-syndrome-a-potential-target-for-clinical-therapeutics
#19
Hayder Amin, Federica Marinaro, Davide De Pietri Tonelli, Luca Berdondini
Individuals with 22q11.2 microdeletion syndrome (22q11.2 DS) show cognitive and behavioral dysfunctions, developmental delays in childhood and risk of developing schizophrenia and autism. Despite extensive previous studies in adult animal models, a possible embryonic root of this syndrome has not been determined. Here, in neurons from a 22q11.2 DS mouse model (Lgdel (+/-)), we found embryonic-premature alterations in the neuronal chloride cotransporters indicated by dysregulated NKCC1 and KCC2 protein expression levels...
November 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29146936/hypomethylation-of-hoxa4-promoter-is-common-in-silver-russell-syndrome-and-growth-restriction-and-associates-with-stature-in-healthy-children
#20
Mari Muurinen, Katariina Hannula-Jouppi, Lovisa E Reinius, Cilla Söderhäll, Simon Kebede Merid, Anna Bergström, Erik Melén, Göran Pershagen, Marita Lipsanen-Nyman, Dario Greco, Juha Kere
Silver-Russell syndrome (SRS) is a growth retardation syndrome in which loss of methylation on chromosome 11p15 (11p15 LOM) and maternal uniparental disomy for chromosome 7 [UPD(7)mat] explain 20-60% and 10% of the syndrome, respectively. To search for a molecular cause for the remaining SRS cases, and to find a possible common epigenetic change, we studied DNA methylation pattern of more than 450 000 CpG sites in 44 SRS patients. Common to all three SRS subgroups, we found a hypomethylated region at the promoter region of HOXA4 in 55% of the patients...
November 16, 2017: Scientific Reports
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