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https://www.readbyqxmd.com/read/28719479/a-simulation-based-quality-improvement-initiative-improves-pediatric-readiness-in-community-hospitals
#1
Travis Whitfill, Marcie Gawel, Marc Auerbach
BACKGROUND: The National Pediatric Readiness Project Pediatric Readiness Survey (PRS) measured pediatric readiness in 4149 US emergency departments (EDs) and noted an average score of 69 on a 100-point scale. This readiness score consists of 6 domains: coordination of pediatric patient care (19/100), physician/nurse staffing and training (10/100), quality improvement activities (7/100), patient safety initiatives (14/100), policies and procedures (17/100), and availability of pediatric equipment (33/100)...
July 17, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28714494/computerized-axial-tomography-findings-in-children-with-afebrile-seizures-a-hospital-based-study-at-eastern-nepal
#2
P Poudel, M K Gupta, S P Kafle
BACKGROUND: Computerized Tomography can be performed in resource limited areas where Magnetic Resonance Imaging is less practical. This study was conducted to find out the proportion of cases with abnormal CT scan and findings of CT scan in children with afebrile seizures in a resource limited area. METHODS: This prospective study was conducted from 1st July 2009 to 31st March 2014 in a university hospital of Nepal. Patients (1 month to 20 years of age) presenting with history of afebrile seizure were included...
January 2017: Journal of Nepal Health Research Council
https://www.readbyqxmd.com/read/28709121/spatiotemporal-propagation-patterns-of-generalized-ictal-spikes-in-childhood-absence-epilepsy
#3
Vasileios Kokkinos, Andreas M Koupparis, Michalis Koutroumanidis, George K Kostopoulos
OBJECTIVE: This work investigates the spatial distribution in time of generalized ictal spikes in the typical absences of childhood absence epilepsy (CAE). METHODS: We studied twelve children with CAE, who had more than two typical absences during their routine video-EEG. Seizures were identified, and ictal spikes were marked over the maximum electronegative peak, clustered, waveform-averaged and spatiotemporaly analyzed in 2D electrode space. RESULTS: Consistency of spatiotemporal patterns of ictal spikes was high between the absences of the same child, but low between children...
June 17, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28707961/encephalocraniocutaneous-lipomatosis-a-case-report-with-review-of-literature
#4
Shaista Siddiqui, Shazia Naaz, Mehtab Ahmad, Zafar Ahmad Khan, Shagufta Wahab, Basmah Abdur Rashid
Encephalocraniocutaneous lipomatosis (ECCL) or Haberland syndrome is an uncommon sporadic neurocutaneous syndrome of unknown origin. The rarity and common ignorance of the condition often makes diagnosis difficult. The hallmark of this syndrome is the triad of skin, ocular and central nervous system (CNS) involvement and includes a long list of combination of conditions. Herein we report a case of a 5-month-old male child who presented to our centre with complaint of seizure. The patient had various cutaneous and ocular stigmatas of the disease in the form of patchy alopecia of the scalp, right-sided limbal dermoid and a nodular skin tag near the lateral canthus of the right eye...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28697163/utility-of-lumbar-puncture-in-children-presenting-with-status-epilepticus
#5
Kenneth A Michelson, Todd W Lyons, Kara B Johnson, Lise E Nigrovic, Marvin B Harper, Amir A Kimia
OBJECTIVES: Because meningitis may trigger seizures, we sought to determine its frequency in children with first-time status epilepticus (SE). METHODS: We performed a retrospective cross-sectional study of children aged 1 month to 21 years who presented to a single pediatric emergency department between 1995 and 2012 with SE and who had a lumbar puncture (LP) performed as part of the diagnostic evaluation. We defined bacterial meningitis as a cerebrospinal fluid (CSF) culture positive for a bacterial pathogen or CSF pleocytosis (CSF white blood cells ≥10 cells/mm) with a blood culture positive for a bacterial pathogen...
July 11, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28691502/associations-of-quality-of-life-with-health-related-characteristics-among-children-with-autism
#6
Karen A Kuhlthau, Erin McDonnell, Dan L Coury, Nalin Payakachat, Eric Macklin
We examine whether behavioral, mental health, and physical health characteristics of children with autism are associated with baseline and change in health-related quality of life. We measured health-related quality of life with the Pediatric Quality of Life Inventory 4.0 total scores from children enrolled in the Autism Treatment Network. We used linear mixed model regressions with random slopes. Predictors of lower health-related quality of life at baseline included demographic and insurance characteristics, diagnosis, higher Child Behavior Checklist internalizing and externalizing scores, sleep problems by Children's Sleep Habits Questionnaire, seizures, gastrointestinal problems, and mental health problems...
July 1, 2017: Autism: the International Journal of Research and Practice
https://www.readbyqxmd.com/read/28691134/seizure-disorders-and-developmental-disorders-impact-on-life-of-affected-families-a-structured-interview
#7
Ulrike Petra Spindler, Lena Charlott Hotopp, Vivien Angela Bach, Frauke Hornemann, Steffen Syrbe, Anna Andreas, Andreas Merkenschlager, Wieland Kiess, Matthias Karl Bernhard, Thilo Bertsche, Martina Patrizia Neininger, Astrid Bertsche
Seizure disorder and developmental disorder are two of the most common chronic disorders in childhood. Data on perceived parental burden and specific effects on daily life is scarce. We performed a structured interview, consecutively talking to all parents of pediatric outpatients of our university hospital diagnosed with seizure or developmental disorder. Three hundred seven parents (of 317 affected children: 53 with seizure disorder, 44 with specific developmental disorder, 35 with learning disorder, 71 with intellectual disability, 15 with seizure + specific developmental disorder, 23 with seizure + learning disorder, 76 with seizure disorder + intellectual disability) were interviewed...
August 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28687558/seizure-management-in-children-requiring-palliative-care-a-review-of-current-practice
#8
Nicola Harris, Megumi Baba, Charlotte Mellor, Rebekah Rogers, Kirsty Taylor, Antonia Beringer, Peta Sharples
OBJECTIVES: Controlling seizures in children approaching death can be difficult, and there is a limited evidence base to guide best practice. We compared current practice against the guidance for seizure management produced by the Association of Paediatric Palliative Medicine (APPM). METHODS: Retrospective case note review of episodes of challenging seizure management in children receiving end-of-life care over a 10-year period (2006-2015) in the south-west region of England...
July 7, 2017: BMJ Supportive & Palliative Care
https://www.readbyqxmd.com/read/28681755/dravet-syndrome-with-scn1b-gene-mutation-a-rare-entity
#9
Devdeep Mukherjee, Swapan Mukherjee, Prabal Niyogi, Manas Mahapatra
Early infantile epileptic encephalopathy has a grave outcome. Dravet syndrome (DS), characterized by early onset, refractory seizures, and intellectual deficit is one of the variants of the condition. SCN1B gene mutation is one of the lesser known variants of DS. Increased awareness of genetic analysis has increased the early diagnosis of DS for an early prognostication as well as genetic counselling of parents. We present the case of a 7-month old male child who started having recurrent febrile, and thereafter, afebrile seizures, following administration of a vaccination at 3 months...
July 2017: Neurology India
https://www.readbyqxmd.com/read/28680303/an-autopsy-proven-child-onset-chronic-traumatic-encephalopathy
#10
Kyuho Lee, Seong-Ik Kim, Yujin Lee, Jae Kyung Won, Sung-Hye Park
Here we present an autopsy case of chronic traumatic encephalopathy (CTE) in a 36-year-old man. He had a history of febrile seizures at the age of four and was severely demented at age 10 when he was admitted to a mental hospital. He had suffered repetitive self-harm, such as frequent banging of the head on the wall in his hospital record, but he had no clear history between the ages of four and ten. Autopsy revealed global cerebral atrophy, including the basal ganglia, thalamus, hippocampus, amygdala, mammilary bodies and lateral geniculate bodies...
June 2017: Experimental Neurobiology
https://www.readbyqxmd.com/read/28672318/effect-of-depth-and-duration-of-cooling-on-death-or-disability-at-age-18-months-among-neonates-with-hypoxic-ischemic-encephalopathy-a-randomized-clinical-trial
#11
RANDOMIZED CONTROLLED TRIAL
Seetha Shankaran, Abbot R Laptook, Athina Pappas, Scott A McDonald, Abhik Das, Jon E Tyson, Brenda B Poindexter, Kurt Schibler, Edward F Bell, Roy J Heyne, Claudia Pedroza, Rebecca Bara, Krisa P Van Meurs, Carolyn M Petrie Huitema, Cathy Grisby, Uday Devaskar, Richard A Ehrenkranz, Heidi M Harmon, Lina F Chalak, Sara B DeMauro, Meena Garg, Michelle E Hartley-McAndrew, Amir M Khan, Michele C Walsh, Namasivayam Ambalavanan, Jane E Brumbaugh, Kristi L Watterberg, Edward G Shepherd, Shannon E G Hamrick, John Barks, C Michael Cotten, Howard W Kilbride, Rosemary D Higgins
Importance: Hypothermia for 72 hours at 33.5°C for neonatal hypoxic-ischemic encephalopathy reduces death or disability, but rates continue to be high. Objective: To determine if cooling for 120 hours or to a temperature of 32.0°C reduces death or disability at age 18 months in infants with hypoxic-ischemic encephalopathy. Design, Setting, and Participants: Randomized 2 × 2 factorial clinical trial in neonates (≥36 weeks' gestation) with hypoxic-ischemic encephalopathy at 18 US centers in the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network between October 2010 and January 2016...
July 4, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28668218/-severe-nutritional-rickets-in-young-children-resurgence-of-an-old-disease
#12
S Estrade, C Majorel, N Tahhan, Y Dulac, C Baunin, I Gennero, Y Chaix, J-P Salles, T Edouard
Nutritional rickets remains a significant public health issue for children worldwide. Although it has almost disappeared in industrialized countries following routine vitamin D supplementation, recent evidence suggests an increasing incidence, especially in young children. In addition to the classical clinical consequences on bone and the growth plate, rickets may also be associated with life-threatening neurological and cardiac complications in the most severe forms. Consequently, early screening and treatment are required...
June 28, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28658653/perceived-need-for-restrictions-on-activity-for-children-with-epilepsy
#13
Paula M Brna, Kevin E Gordon, Elaine Woolridge, Joseph M Dooley, Ellen Wood
BACKGROUND: Children and youth with epilepsy have long been subjected to excessive restrictions on extracurricular activities due to concerns over risk of injury. Over time physicians and medical regulatory associations have liberalized the advice given for people with epilepsy to promote independence, self-esteem and general health benefits of physical activity. Current evidence suggests that few restrictions are needed for children with epilepsy beyond water-related precautions and avoidance of very high-risk activities...
June 25, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28643274/an-unexplained-congenital-disorder-of-glycosylation-ii-in-a-child-with-neurohepatic-involvement-hypercholesterolemia-and-hypoceruloplasminemia
#14
Pier Luigi Calvo, Marco Spada, Ivana Rabbone, Michele Pinon, Francesco Porta, Fabio Cisarò, Stefania Reggiani, Angelo B Cefalù, Luisella Sturiale, Domenico Garozzo, Dirk J Lefeber, Jaak Jaeken
We report on a 12-year-old adopted boy with psychomotor disability, absence seizures, and normal brain MRI. He showed increased (but initially, at 5 months, normal) serum cholesterol, increased alkaline phosphatases, transiently increased transaminases and hypoceruloplasminemia with normal serum and urinary copper. Blood levels of immunoglobulins, haptoglobin, antithrombin, and factor XI were normal. A type 2 serum transferrin isoelectrofocusing and hypoglycosylation of apoCIII pointed to a combined N- and O-glycosylation defect...
June 23, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28641166/care-coordination-at-a-pediatric-accountable-care-organization-aco-a-qualitative-analysis
#15
Baria Hafeez, Sophia Miller, Anup D Patel, Zachary M Grinspan
OBJECTIVE: Care coordinators may help manage care for children with chronic illness. Their role in pediatric epilepsy care is understudied. We aimed to qualitatively describe the content of a care coordination intervention for children with epilepsy. METHODS: We conducted nine semi-structured interviews and one focus group with care coordinators at a pediatric accountable care organization (ACO) in Ohio. The care coordinators used a modified version of a published care coordination checklist for children with epilepsy (Patel AD, 2014)...
June 19, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28635423/inner-retinal-dystrophy-in-a-patient-with-biallelic-sequence-variants-in-brat1
#16
Julius T Oatts, Jacque L Duncan, Creig S Hoyt, Anne M Slavotinek, Anthony T Moore
BACKGROUND: Mutations in the BRCA1-associated protein required for the ataxia telangiectasia mutated (ATM) activation-1 (BRAT1) gene cause lethal neonatal rigidity and multifocal seizure syndrome characterized by rigidity and intractable seizures and a milder phenotype with intellectual disability, seizures, nonprogressive cerebellar ataxia or dyspraxia, and cerebellar atrophy. To date, nystagmus, cortical visual impairment, impairment of central vision, optic nerve hypoplasia, and optic atrophy have been described in this condition...
March 2, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28632327/photosensitivity-is-an-early-marker-of-neuronal-ceroid-lipofuscinosis-type-2-disease
#17
Nicola Specchio, Marcello Bellusci, Nicola Pietrafusa, Marina Trivisano, Luca de Palma, Federico Vigevano
OBJECTIVE: This study aimed to identify early clinical, magnetic resonance imaging (MRI), and electroencephalographic (EEG) characteristics of neuronal ceroid lipofuscinosis type 2 (CLN2) disease to enable early diagnosis, thus providing the key to early treatment, and optimized care and outcomes. METHODS: Retrospective clinical chart review of a series of patients diagnosed with CLN2 disease from 2005 to 2015 at a single center in Italy. Clinical, MRI, and EEG findings were reviewed...
June 20, 2017: Epilepsia
https://www.readbyqxmd.com/read/28620564/moyamoya-syndrome-in-a-child-with-neurofibromatosis-type-1-magnetic-resonance-imaging-as-a-tool-for-clinical-decision-making
#18
Jonathan Mayl, Hanisha Patel, Tushar Chandra
Moyamoya syndrome is a rare cerebrovasculopathy of unknown etiology which is associated with multiple risk factors. Moyamoya was first discovered in Japan and is reported to have an increased prevalence in the Japanese population. The term "Moyamoya" translates into "puff of smoke" and is named after the finding of the collateral cerebral vasculature that develops secondary to occlusion of an internal carotid artery at the entrance into the circle of Willis. This collateral vasculature characterizes the disease...
May 9, 2017: Curēus
https://www.readbyqxmd.com/read/28615945/ictal-blinking-an-under-recognized-phenomenon-our-experience-and-literature-review
#19
Marco Andrea Nicola Saporito, Giovanna Vitaliti, Piero Pavone, Giuseppa Di Stefano, Pasquale Striano, Roberto Horacio Caraballo, Raffaele Falsaperla
Ictal blinking (IB) is a very rare disease manifesting as an epileptic motor event in children and adults. Until now it has not been included in any classification of focal seizures of the International League Against Epilepsy Commission. It could be unilateral or bilateral, isolated or in association with other motor manifestations such as limbs' clonus and spasms. Its pathogenesis has not been clearly established: paroxysmal discharges from different areas of the brain could cause IB by activation of trigeminal fibers...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28612338/nutritional-rickets-and-osteomalacia-in-the-twenty-first-century-revised-concepts-public-health-and-prevention-strategies
#20
REVIEW
Suma Uday, Wolfgang Högler
PURPOSE OF REVIEW: Nutritional rickets and osteomalacia are common in dark-skinned and migrant populations. Their global incidence is rising due to changing population demographics, failing prevention policies and missing implementation strategies. The calcium deprivation spectrum has hypocalcaemic (seizures, tetany and dilated cardiomyopathy) and late hypophosphataemic (rickets, osteomalacia and muscle weakness) complications. This article reviews sustainable prevention strategies and identifies areas for future research...
June 13, 2017: Current Osteoporosis Reports
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