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https://www.readbyqxmd.com/read/28541008/protein-losing-enteropathy-as-a-complication-of-the-ketogenic-diet
#1
Won Kee Ahn, Soyoung Park, Heung Dong Kim
The ketogenic diet is an effective treatment for the patients with intractable epilepsy, however, the diet therapy can sometimes be discontinued by complications. Protein-losing enteropathy is a rarely reported serious complication of the ketogenic diet. We present a 16-month-old Down syndrome baby with protein-losing enteropathy during the ketogenic diet as a treatment for West syndrome. He suffered from diarrhea, general edema and hypoalbuminemia which were not controlled by conservative care for over 1 month...
July 2017: Yonsei Medical Journal
https://www.readbyqxmd.com/read/28530481/hormone-replacement-therapy-in-pre-menopausal-women-undergoing-bilateral-salpingo-oophorectomy-for-benign-disease-a-review-of-practice
#2
Alison Richardson, Emily West, Mike Cust
Introduction The National Institute for Health and Care Excellence (NICE) guideline on the diagnosis and management of the menopause states that women who are likely to go through the menopause as a result of surgical treatment should be offered information about the menopause and the importance of starting hormonal replacement therapy before they have their treatment. Objectives To determine compliance with NICE guidelines at the Royal Derby Hospital. Study design We undertook a retrospective review of all pre-menopausal women undergoing bilateral salpingo-oophorectomy for benign pathology between 1 January 2016 and 30 June 2016...
January 1, 2017: Post Reproductive Health
https://www.readbyqxmd.com/read/28524224/-epileptic-encephalopathies-in-infancy-how-do-we-treat-them-does-the-aetiology-influence-the-response-to-treatment
#3
S Roldan
INTRODUCTION: Resistance to treatments is a common feature of Ohtahara, Aicardi, West and Dravet syndromes, as well as malignant migrating epilepsy in infancy. AIMS: To update the therapeutic management and to analyse whether the aetiology somehow determines the treatment. DEVELOPMENT: Convulsive seizures in the first year of life may be due to a potentially treatable aetiology, which makes it essential to carry out a complete evaluation so as to be able to begin, as early as possible, the most suitable and the non-specific symptomatic treatments to control the seizures, which prevents or minimises their deleterious effects...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28524223/-infantile-epileptic-encephalopathies-what-matters-is-genetics
#4
J J Garcia-Penas, M Jimenez-Legido
INTRODUCTION: Epileptic encephalopathies in infancy are defined as conditions where the sustained epileptic activity itself may contribute to the severe neurological and cognitive impairment. These epileptic encephalopathies include Ohtahara syndrome, early myoclonic epileptic encephalopathy, West syndrome, Dravet syndrome, and malignant migrating epilepsy in infancy. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28524219/-epileptic-encephalopathies
#5
J Ramos-Lizana
According to the International League Against Epilepsy's (ILAE) Commission on Classification and Terminology, the term 'epileptic encephalopathy' reflects the notion that epileptic activity in itself can contribute to the genesis of severe cognitive or behavioural disabilities, beyond what could be expected from the pathology underlying the epilepsy. However, in many cases it is difficult to define the boundary between the relative contribution of the epileptic seizures and the underlying cause in the genesis of cognitive deficits...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28523387/seroprevalence-of-economically-important-viral-pathogens-in-swine-populations-of-trinidad-and-tobago-west-indies
#6
Jamie R V Sookhoo, Arianne Brown-Jordan, Lemar Blake, Ridley B Holder, Sharon M Brookes, Stephen Essen, Christine V F Carrington, Ian H Brown, Christopher A L Oura
The objective of this study was to evaluate the seroprevalence and identify the strains of swine influenza virus (SwIV), as well as the seroprevalence of porcine parvovirus (PPV), transmissible gastroenteritis virus (TGEV), porcine reproductive and respiratory syndrome virus (PRRSV), porcine respiratory coronavirus (PRCV), porcine circovirus type 2 (PCV-2), and classical swine fever virus (CSFV) in pigs in Trinidad and Tobago (T&T). Blood samples (309) were randomly collected from pigs at farms throughout T&T...
May 18, 2017: Tropical Animal Health and Production
https://www.readbyqxmd.com/read/28508417/score-of-toxic-epidermal-necrosis-predicts-the-outcomes-of-pediatric-epidermal-necrolysis
#7
Jennifer Sorrell, Lisa Anthony, Alfred Rademaker, Steven M Belknap, Shields Callahan, Dennis P West, Amy S Paller
BACKGROUND/OBJECTIVES: Epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) includes immune-mediated, life-threatening inflammatory blistering disorders that can affect children. The Score of Toxic Epidermal Necrosis (SCORTEN) tool has accurately predicted the outcome of these disorders in adults but has not been tested in children. METHODS: We performed a retrospective chart review to compare the accuracy of the adult SCORTEN tool with that of two modifications tailored to children in predicting disease outcome...
May 16, 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28504627/-high-isolation-preparedness-for-very-contagious-and-potential-severely-ill-patients
#8
Gitte Kronborg, Sanne Jespersen, Helle Ingmer, Merete Storgaard
Ten and seven years ago respectively, the two high-isolation units in Denmark became prepared to receive highly contagious and potential severely ill patients. The units are located in the departments of infectious diseases in Hvidovre Hospital and Aarhus University Hospital. Both departments had little experience until the Ebola outbreak in West Africa, 2013-2015. Altogether, 15 possible Ebola patients were examined in the two departments, but the diagnosis was ruled out for all of them. Besides, a few possible candidates for Middle East respiratory syndrome patients were examined...
May 15, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28499308/outcome-of-neonates-with-meconium-aspiration-syndrome-at-the-university-hospital-of-the-west-indies-jamaica-a-resource-limited-setting
#9
L Panton, H Trotman
No abstract text is available yet for this article.
May 12, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28489313/in-utero-seizures-revealing-dentato-olivary-dysplasia-caused-by-scn2a-mutation
#10
Fanny Sauvestre, Sébastien Moutton, Catherine Badens, Bernard Broussin, Dominique Carles, Nada Houcinat, Caroline Lacoste, Florent Marguet, Christophe Pecheux, Laurent Villard, Fanny Pelluard, Annie Laquerrière, Gwenaëlle André
Most early-onset epileptic encephalopathies (EOEE) are caused by genetic defects. In the past, mutations, especially in genes encoding sodium channels, have been identified using linkage studies, array-CGH and more recently next-generation sequencing (NGS) [1]. Mutations in SCN2A gene have been identified in a wide variety of early-onset epileptic syndromes including benign familial neonatal infantile seizures (BFNIS) [2] and more severe forms leading to encephalopathy such as Ohtahara or West syndromes [3], epilepsy of infancy with migrating focal seizures (EIMFS) [4] and autism spectrum disorders [5]...
May 10, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28487361/biochemical-characterization-of-purified-mammalian-arl13b-indicate-that-it-is-an-atypical-gtpase-and-arl3-guanine-nucleotide-exchange-factor-gef
#11
Anna A Ivanova, Tamara Caspary, Nicholas T Seyfried, Duc M Duong, Andrew B West, Zhiyong Liu, Richard A Kahn
Primary cilia play central roles in signaling during metazoan development. Several key regulators of ciliogenesis and ciliary signaling are mutated in humans, resulting in a number of ciliopathies, including Joubert Syndrome (JS). ARL13B is a ciliary GTPase with at least three missense mutations identified in JS patients. ARL13B is a member of the ARF family of regulatory GTPases, but is atypical in having a non-homologous, C-terminal domain of ~20 kDa and at least one key residue difference in the consensus GTP binding motifs...
May 9, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28483078/clinical-course-and-treatment-outcome-of-koro-a-follow-up-study-from-a-koro-epidemic-reported-from-west-bengal-india
#12
Amitava Dan, Tanushree Mondal, Kaustav Chakraborty, Aditi Chaudhuri, Asish Biswas
BACKGROUND: Koro, as a culture bound syndrome is predominantly reported from Asian countries. There is dearth of well-designed research focussing on course and outcome of Koro. METHOD: In the index study, 64 consecutive consenting patients with symptoms of Koro reported in different disciplines of a tertiary care Government Hospital of West Bengal were recruited over a period of 3 months. They were treated by standard treatment protocol and followed up for next 3 months...
April 2017: Asian Journal of Psychiatry
https://www.readbyqxmd.com/read/28471433/identifying-ownership-through-role-descriptions-to-support-implementing-universal-colorectal-cancer-tumor-screening-for-lynch-syndrome
#13
Kathleen M West, Wylie Burke, Diane M Korngiebel
PurposeLynch syndrome cases are underidentified, and universal colorectal cancer tumor screening for Lynch syndrome (UTS) has been recommended. UTS implementation is challenging and few successful examples exist to date, and colorectal cancer patients and at-risk family members exhibit low uptake of genetic services. This study sought to identify the elements that could guide the choice of specialties to implement UTS through three main stages: initiating the screen, returning positive screen results, and providing follow-up...
May 4, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28462453/preclinical-screening-for-treatments-for-infantile-spasms-in-the-multiple-hit-rat-model-of-infantile-spasms-an-update
#14
Aristea S Galanopoulou, Wenzhu B Mowrey, Wei Liu, Qianyun Li, Oleksii Shandra, Solomon L Moshé
Infantile spasms are the typical seizures of West syndrome, an infantile epileptic encephalopathy with poor outcomes. There is an increasing need to identify more effective and better tolerated treatments for infantile spasms. We have optimized the rat model of infantile spasms due to structural etiology, the multiple-hit rat model, for therapy discovery. Here, we test three compounds administered after spasms induction in the multiple hit model for efficacy and tolerability. Specifically, postnatal day 3 (PN3) male Sprague-Dawley rats were induced by right intracerebral injections of doxorubicin and lipopolysaccharide...
May 2, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28461209/insights-from-clinical-research-completed-during-the-west-africa-ebola-virus-disease-epidemic
#15
REVIEW
Amanda Rojek, Peter Horby, Jake Dunning
The west Africa Ebola virus disease (EVD) epidemic was extraordinary in scale. Now that the epidemic has ended, it is a relevant time to examine published studies with direct relevance to clinical care and, more broadly, to examine the implications of the clinical research response mounted. Clinically relevant research includes literature detailing risk factors for and clinical manifestations of EVD, laboratory and other investigation findings in patients, experimental vaccine and therapeutic clinical trials, and analyses of survivor syndrome...
April 28, 2017: Lancet Infectious Diseases
https://www.readbyqxmd.com/read/28437459/management-and-outcomes-of-severe-dengue-patients-presenting-with-sepsis-in-a-tropical-country
#16
Prapit Teparrukkul, Viriya Hantrakun, Nicholas P J Day, T Eoin West, Direk Limmathurotsakul
BACKGROUND: Dengue is a common cause of infection in adults in tropical countries. Sepsis is a syndrome of systemic manifestations induced by infection of any organisms; including bacterial, fungal and viral agents. Here, we investigated the diagnosis, management and outcomes of dengue patients presenting with sepsis in a prospective study of community-acquired sepsis in Thailand. METHODS: From June to December 2015, 874 adult patients (age≥18 years) with suspected or documented community-acquired infection, with ≥3 diagnostic criteria for sepsis according to the Surviving Sepsis Campaign 2012, and within 24 hours of admission were evaluated...
2017: PloS One
https://www.readbyqxmd.com/read/28427564/inflammation-in-epileptic-encephalopathies
#17
Oleksii Shandra, Solomon L Moshé, Aristea S Galanopoulou
West syndrome (WS) is an infantile epileptic encephalopathy that manifests with infantile spasms (IS), hypsarrhythmia (in ~60% of infants), and poor neurodevelopmental outcomes. The etiologies of WS can be structural-metabolic pathologies (~60%), genetic (12%-15%), or of unknown origin. The current treatment options include hormonal treatment (adrenocorticotropic hormone and high-dose steroids) and the GABA aminotransferase inhibitor vigabatrin, while ketogenic diet can be given as add-on treatment in refractory IS...
2017: Advances in Protein Chemistry and Structural Biology
https://www.readbyqxmd.com/read/28424778/estimation-of-time-dependent-reproduction-numbers-for-porcine-reproductive-and-respiratory-syndrome-across-different-regions-and-production-systems-of-the-us
#18
Andréia G Arruda, Moh A Alkhamis, Kimberly VanderWaal, Robert B Morrison, Andres M Perez
Porcine reproductive and respiratory syndrome (PRRS) is, arguably, the most impactful disease for the North American swine industry, due to its known considerable economic losses. The Swine Health Monitoring Project (SHMP) monitors and reports weekly new PRRS cases in 766 sow herds across the US. The time-dependent reproduction number (TD-R) is a measure of a pathogen's transmissibility. It may serve to capture and report PRRS virus (PRRSV) spread at the regional and system levels. The primary objective of the study here was to estimate the TD-R values for PRRSV using regional and system-level PRRS data, and to contrast it with commonly used metrics of disease, such as incidence estimates and space-time clusters...
2017: Frontiers in Veterinary Science
https://www.readbyqxmd.com/read/28417301/overview-of-coronary-heart-disease-risk-initiatives-in-south-asia
#19
REVIEW
Ankur Kalra, Deepak L Bhatt, Sanjay Rajagopalan, Kunal Suri, Sundeep Mishra, Romaina Iqbal, Salim S Virani
PURPOSE OF REVIEW: Cardiovascular disease (CVD) is now the leading cause of morbidity and mortality worldwide. Industrialization and economic growth have led to an unprecedented increment in the burden of CVD and their risk factors in less industrialized regions of the world. While there are abundant data on CVD and their risk factors from longitudinal cohort studies done in the West, good-quality data from South Asia are lacking. RECENT FINDINGS: Several multi-institutional, observational, prospective registries, and epidemiologic cohorts in South Asia have been established to systematically evaluate the burden of CVD and their risk factors...
June 2017: Current Atherosclerosis Reports
https://www.readbyqxmd.com/read/28401986/auditory-processing-following-infantile-spasms-an-event-related-potential-study
#20
Tangunu Fosi, Klaus Werner, Stewart G Boyd, Michelle De Haan, Rod C Scott, Brian G Neville
OBJECTIVES: To investigate acoustic auditory processing in patients with recent infantile spasms (IS). METHODS: Patients (n = 22; 12 female; median age 8 months; range 5-11 months) had normal preceding development, brain magnetic resonance imaging (MRI), and neurometabolic testing (West syndrome of unknown cause, uWS). Controls were healthy babies (n = 22; 11 female; median age 6 months; range 3-12 months). Event-related potentials (ERPs) and psychometry (Bayley Scales of Infant Development, Second Edition, BSID-II) took place at a month following IS remission...
April 12, 2017: Epilepsia
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