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https://www.readbyqxmd.com/read/28081533/eeg-monitoring-technique-influences-the-management-of-hypoxic-ischemic-seizures-in-neonates-undergoing-therapeutic-hypothermia
#1
Saber Jan, Frances J Northington, Charlamaine M Parkinson, Carl E Stafstrom
Electroencephalogram (EEG) monitoring techniques for neonatal hypoxia-ischemia (HI) are evolving over time, and the specific type of EEG utilized could influence seizure diagnosis and management. We examined whether the type of EEG performed affected seizure treatment decisions (e.g., the choice and number of antiseizure drugs [ASDs]) in therapeutic hypothermia-treated neonates with HI from 2007 to 2015 in the Johns Hopkins Hospital Neonatal Intensive Care Unit. During this period, 3 different EEG monitoring protocols were utilized: Period 1 (2007-2009), single, brief conventional EEG (1 h duration) at a variable time during therapeutic hypothermia treatment, i...
January 17, 2017: Developmental Neuroscience
https://www.readbyqxmd.com/read/28079875/early-postnatal-illness-severity-scores-predict-neurodevelopmental-impairments-at-10-years-of-age-in-children-born-extremely-preterm
#2
J W Logan, O Dammann, E N Allred, C Dammann, K Beam, R M Joseph, T M O'Shea, A Leviton, K C K Kuban
OBJECTIVE: A neonatal illness severity score, The Score for Neonatal Acute Physiology-II (SNAP-II), predicts neurodevelopmental impairments at two years of age among children born extremely preterm. We sought to evaluate to what extent SNAP-II is predictive of cognitive and other neurodevelopmental impairments at 10 years of age. STUDY DESIGN: In a cohort of 874 children born before 28 weeks of gestation, we prospectively collected clinical, physiologic and laboratory data to calculate SNAP-II for each infant...
January 12, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28076783/cdyl-deficiency-disrupts-neuronal-migration-and-increases-susceptibility-to-epilepsy
#3
Rui Qin, Shuai Cao, Tianjie Lyu, Cai Qi, Weiguang Zhang, Yun Wang
During brain development, the correct migration of newborn neurons is one of the determinants of circuit formation, and neuronal migration defects may lead to neurological and psychiatric disorders. The molecular mechanisms underlying neuronal migration and related disorders are poorly understood. Here, we report that Chromodomain Y-like (CDYL) is critical for neuronal migration in mice. Knocking down CDYL caused neuronal migration defects and disrupted both mobility and multipolar-to-bipolar transition of migrating neurons...
January 10, 2017: Cell Reports
https://www.readbyqxmd.com/read/28065256/a-newborn-infant-with-oxygen-desaturation-during-sleep
#4
Ashley Deschamp, Ameet Daftary
The patient is a 1-week-old boy born at 37+2 weeks' gestational age through spontaneous vaginal delivery. The pregnancy was complicated by maternal gestational diabetes mellitus and pre-eclampsia. The Apgar score was 9 at both 1 minute and 5 minutes after birth. Because of hypoglycemia at delivery that required IV dextrose, he was admitted to the local neonatal intensive care unit. His blood glucose levels quickly stabilized, dextrose administration was discontinued, and the patient began breast-feeding. On day 2 of life, the patient began having intermittent oxygen desaturation, with oxygen saturation as measured by pulse oximetry down to 70% while sleeping; he was transferred to a tertiary-care NICU for further management...
January 2017: Chest
https://www.readbyqxmd.com/read/28042096/different-response-to-antiepileptic-drugs-according-to-the-type-of-epileptic-events-in-a-neonatal-ischemia-reperfusion-model
#5
Luc Morin, Julie Enderlin, Pierre-Louis Leger, Gaëtan Perrotte, Philippe Bonnin, Nina Dupuis, Olivier Baud, Christiane Charriaut-Marlangue, Stéphane Auvin
Perinatal arterial stroke is the most frequent form of cerebral infarction in children. Neonatal seizures are the most frequent symptom during the neonatal period. The current management of perinatal stroke is based on supportive care. It is currently unknown if treatment of the seizures modifies the outcome, and no clinical studies have focused on seizures during neonatal stroke. We studied the effect of phenobarbital and levetiracetam on an ischemic-reperfusion stroke model in P7 rats using prolonged electroencephalographic recordings and a histologic analysis of the brain (24h after injury)...
December 30, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/28018462/a-novel-mutation-of-abcc8-gene-in-a-patient-with-diazoxide-unresponsive-congenital-hyperinsulinism
#6
Ji Sook Park, Hong-Jun Lee, Chan-Hoo Park
Congenital hyperinsulinism (CHI) is a rare condition that can cause irreversible brain damage during the neonatal period owing to the associated hypoglycemia. Hypoglycemia in CHI occurs secondary to the dysregulation of insulin secretion. CHI has been established as a genetic disorder of islet-cell hyperplasia, associated with a mutation of the ABCC8 or KCNJ11 genes, which encode the sulfonylurea receptor 1 and the inward rectifying potassium channel (Kir6.2) subunit of the ATP-sensitive potassium channel, respectively...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28018444/compound-heterozygous-mutations-of-acads-gene-in-newborn-with-short-chain-acyl-coa-dehydrogenase-deficiency-case-report-and-literatures-review
#7
Se Jin An, Sook Za Kim, Gu Hwan Kim, Han Wook Yoo, Han Hyuk Lim
Short-chain acyl-CoA dehydrogenase deficiency (SCADD) is a rare autosomal recessive mitochondrial disorder of fatty acid β-oxidation, and is associated with mutations in the acyl-CoA dehydrogenase (ACADS) gene. Recent advances in spectrometric screening for inborn errors of metabolism have helped detect several metabolic disorders, including SCADD, without symptoms in the neonate period. This allows immediate initiation of treatment and monitoring, so they remain largely symptomless metabolic disease. Here, we report a 15-month-old asymptomatic male, who was diagnosed with SCADD by newborn screening...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28018437/1p36-deletion-syndrome-confirmed-by-fluorescence-in-situ-hybridization-and-array-comparative-genomic-hybridization-analysis
#8
Dong Soo Kang, Eunsim Shin, Jeesuk Yu
Pediatric epilepsy can be caused by various conditions, including specific syndromes. 1p36 deletion syndrome is reported in 1 in 5,000-10,000 newborns, and its characteristic clinical features include developmental delay, mental retardation, hypotonia, congenital heart defects, seizure, and facial dysmorphism. However, detection of the terminal deletion in chromosome 1p by conventional G-banded karyotyping is difficult. Here we present a case of epilepsy with profound developmental delay and characteristic phenotypes...
November 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28011082/-neonatal-arterial-ischemic-stroke-review-of-the-current-guidelines
#9
E Saliba, T Debillon, S Auvin, O Baud, V Biran, J-L Chabernaud, S Chabrier, F Cneude, A-G Cordier, V Darmency-Stamboul, J-F Diependaele, T Debillon, M Dinomais, C Durand, A Ego, G Favrais, Y Gruel, L Hertz-Pannier, B Husson, S Marret, S N'Guyen The Tich, T Perez, E Saliba, J-B Valentin, C Vuillerot
Neonatal arterial ischemic stroke (NAIS) is a rare event that occurs in approximately one in 5000 term or close-to-term infants. Most affected infants will present with seizures. Although a well-recognized clinical entity, many questions remain regarding diagnosis, risk factors, treatment, and follow-up modalities. In the absence of a known pathophysiological mechanism and lack of evidence-based guidelines, only supportive care is currently provided. To address these issues, a French national committee set up by the French Neonatal Society (Société française de néonatologie) and the national referral center (Centre national de référence) for arterial ischemic stroke in children drew up guidelines based on an HAS (Haute Autorité de santé [HAS]; French national authority for health) methodology...
December 20, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28007989/cad-mutations-and-uridine-responsive-epileptic-encephalopathy
#10
Johannes Koch, Johannes A Mayr, Bader Alhaddad, Christian Rauscher, Jörgen Bierau, Reka Kovacs-Nagy, Karlien L M Coene, Ingrid Bader, Monika Holzhacker, Holger Prokisch, Hanka Venselaar, Ron A Wevers, Felix Distelmaier, Tilman Polster, Steffen Leiz, Cornelia Betzler, Tim M Strom, Wolfgang Sperl, Thomas Meitinger, Saskia B Wortmann, Tobias B Haack
Unexplained global developmental delay and epilepsy in childhood pose a major socioeconomic burden. Progress in defining the molecular bases does not often translate into effective treatment. Notable exceptions include certain inborn errors of metabolism amenable to dietary intervention. CAD encodes a multifunctional enzyme involved in de novo pyrimidine biosynthesis. Alternatively, pyrimidines can be recycled from uridine. Exome sequencing in three families identified biallelic CAD mutations in four children with global developmental delay, epileptic encephalopathy, and anaemia with anisopoikilocytosis...
December 21, 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27940321/monitoring-infants-by-automatic-video-processing-a-unified-approach-to-motion-analysis
#11
Luca Cattani, Davide Alinovi, Gianluigi Ferrari, Riccardo Raheli, Elena Pavlidis, Carlotta Spagnoli, Francesco Pisani
A unified approach to contact-less and low-cost video processing for automatic detection of neonatal diseases characterized by specific movement patterns is presented. This disease category includes neonatal clonic seizures and apneas. Both disorders are characterized by the presence or absence, respectively, of periodic movements of parts of the body-e.g., the limbs in case of clonic seizures and the chest/abdomen in case of apneas. Therefore, one can analyze the data obtained from multiple video sensors placed around a patient, extracting relevant motion signals and estimating, using the Maximum Likelihood (ML) criterion, their possible periodicity...
December 1, 2016: Computers in Biology and Medicine
https://www.readbyqxmd.com/read/27931774/neonatal-venlafaxine-discontinuation-syndrome-a-mini-review
#12
REVIEW
Jonathon Holland, Richard Brown
During pregnancy, the developing fetal brain may be exposed to a range of psychotropic medications. The serotonin-noradrenergic reuptake inhibitor venlafaxine is one such drug, when used as a maternal antidepressant. Here we review the discontinuation phenomenon that may follow in exposed neonates following birth. Adults who abruptly stop taking venlafaxine can experience withdrawal symptoms. Venlafaxine and its metabolites cross the placenta and so the newborn can be exposed to this risk, as well as potential toxicity...
November 25, 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/27928548/microglia-and-neurogenesis-in-the-epileptic-dentate-gyrus
#13
Cong Luo, Yuji Ikegaya, Ryuta Koyama
Microglia are recognized as major immune cells in the brain. They have been traditionally studied in various contexts of disease, where their activation has been assumed to induce mostly detrimental effects. Recent studies, however, have challenged the current view of microglia, clarifying their essential contribution to the development of neural circuits and brain function. In this review, we particularly discuss the role of microglia as the major orchestrators that regulate adult neurogenesis in the hippocampus...
2016: Neurogenesis (Austin, Tex.)
https://www.readbyqxmd.com/read/27830119/the-first-mongolian-cases-of-phenylketonuria-in-selective-screening-of-inborn-errors-of-metabolism
#14
Jamiyan Purevsuren, Baasandai Bolormaa, Chogdon Narantsetseg, Renchindorj Batsolongo, Ochirbat Enkhchimeg, Munkhuu Bayalag, Yuki Hasegawa, Haruo Shintaku, SeijiYamaguchi
BACKGROUND: Inborn errors of metabolism (IEM) are rare genetic disorders in which a single gene defect causes a clinically significant block in a metabolic pathway. Clinical problems arise due to either accumulation of substrates that are toxic or interfere with normal function, or deficiency of the products that are used to synthesize essential compounds. There is no report of screening results or confirmed cases of IEM in Mongolia. Only pilot study of newborn screening for congenital hypothyroidism was implemented in Mongolia, where the incidence of congenital hypothyroidism is calculated to be 1:3057 in Mongolia...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27800071/neonatal-transport-practices-in-ibadan-nigeria
#15
Muhydeen Abiodun Abdulraheem, Olukemi Oluwatoyin Tongo, Adebola Emmanuel Orimadegun, Olukayode Felix Akinbami
INTRODUCTION: Neonatal transport involves moving sick neonates in optimal conditions to ensure good outcomes. It is well organized in most developed countries but receives little attention in developing countries where the highest burden of neonatal mortality exists and a large number of newborns require referrals daily for better medical care. This study sought to evaluate the modes of transport, pre- and intra-transport care of neonates referred to the University College Hospital (UCH), Ibadan, Nigeria...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27796549/-endovascular-embolic-hemispherectomy-a-strategy-for-the-initial-management-of-catastrophic-holohemispheric-epilepsy-in-the-neonate
#16
Chima Oluigbo, Monica S Pearl, Tammy N Tsuchida, Taeun Chang, Cheng-Ying Ho, William D Gaillard
PURPOSE: Conflicting challenges abound in the management of the newborn with intractable epilepsy related to hemimegalencephaly. Early hemispherectomy to stop seizures and prevent deleterious consequences to future neurocognitive development must be weighed against the technical and anesthetic challenges of performing major hemispheric surgery in the neonate. METHODS: We hereby present our experience with two neonates with hemimegalencephaly and intractable seizures who were managed using a strategy of initial minimally invasive embolization of the cerebral blood supply to the involved hemisphere...
October 29, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27751684/women-s-experiences-of-having-had-and-recovered-from-eclampsia-at-a-tertiary-hospital-in-tanzania
#17
Annamagreth M Mukwenda, Columba K Mbekenga, Andrea B Pembe, Pia Olsson
BACKGROUND: Eclampsia is a major cause of maternal and perinatal mortality that requires advanced care and long hospital stays with uncertain outcomes for mother and baby. Care of eclamptic women is particularly challenging in low-income settings. Standards for medical care for eclampsia are established but the psychosocial needs of women are under-researched. AIM: To explore and describe women's experiences of having had, and recovered from, eclampsia at a tertiary hospital in Tanzania...
October 14, 2016: Women and Birth: Journal of the Australian College of Midwives
https://www.readbyqxmd.com/read/27749459/detecting-neonatal-seizures-with-computer-algorithms
#18
Andriy Temko, Gordon Lightbody
It is now generally accepted that EEG is the only reliable way to accurately detect newborn seizures and, as such, prolonged EEG monitoring is increasingly being adopted in neonatal intensive care units. Long EEG recordings may last from several hours to a few days. With neurophysiologists not always available to review the EEG during unsociable hours, there is a pressing need to develop a reliable and robust automatic seizure detection method-a computer algorithm that can take the EEG signal, process it, and output information that supports clinical decision making...
October 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/27709092/antegrade-cerebral-perfusion-at-25-%C3%A2-c-for-arch-reconstruction-in-newborns-and-children-preserves-perioperative-cerebral-oxygenation-and-serum-creatinine
#19
Bhawna Gupta, Ali Dodge-Khatami, Juan Tucker, Mary B Taylor, Douglas Maposa, Miguel Urencio, Jorge D Salazar
BACKGROUND: Antegrade cerebral perfusion (ACP) typically is used with deep hypothermia for cerebral protection during aortic arch reconstructions. The impact of ACP on cerebral oxygenation and serum creatinine at a more tepid 25 °C was studied in newborns and children. METHODS: Between 2010 and 2014, 61 newborns and children (<5 years old) underwent aortic arch reconstruction using moderate hypothermia (25.0±0.9 °C) with ACP and a pH-stat blood gas management strategy...
July 2016: Translational pediatrics
https://www.readbyqxmd.com/read/27698899/is-mtor-inhibitor-good-enough-for-treatment-all-tumors-in-tsc-patients
#20
REVIEW
Samy L Habib, Noor Y Al-Obaidi, Maciej Nowacki, Katarzyna Pietkun, Barbara Zegarska, Tomasz Kloskowski, Wojciech Zegarski, Tomasz Drewa, Edward A Medina, Zhenze Zhao, Sitai Liang
Tuberous sclerosis complex (TSC) is an autosomal dominant and multi-system genetic disorder in humans. TSC affects around 25,000 to 40,000 individuals in the United States and about 1 to 2 million individuals worldwide, with an estimated prevalence of one in 6,000 newborns. TSC occurs in all races and ethnic groups, and in both genders. TSC is caused by defects or mutations in two genes, TSC1 and TSC2. Loss of TSC1/TSC2 leads to dysregulation of mTOR, resulting in aberrant cell differentiation and development, and abnormal enlargement of cells...
2016: Journal of Cancer
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