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https://read.qxmd.com/read/30769278/lestaurtinib-cep-701-modulates-the-effects-of-early-life-hypoxic-seizures-on-cognitive-and-emotional-behaviors-in-immature-rats
#1
Yasser Medlej, Houssein Salah, Lara Wadi, Sarah Saad, Bashir Bashir, Jad Allam, Zahraa Atoui, Nora Darwish, Nabil Karnib, Hala Darwish, Firas Kobeissy, Kevin K W Wang, Eva Hamade, Makram Obeid
Hypoxic encephalopathy of the newborn is a major cause of long-term neurological sequelae. We have previously shown that CEP-701 (lestaurtinib), a drug with an established safety profile in children, attenuates short-term hyperexcitability and tropomyosin-related kinase B (TrkB) receptor activation in a well-established rat model of early life hypoxic seizures (HS). Here, we investigated the potential long-term neuroprotective effects of a post-HS transient CEP-701 treatment. Following exposure to global hypoxia, 10 day old male Sprague-Dawley pups received CEP-701 or its vehicle and were sequentially subjected to the light-dark box test (LDT), forced swim test (FST), open field test (OFT), Morris water maze (MWM), and the modified active avoidance (MAAV) test between postnatal days 24 and 44 (P24-44)...
February 12, 2019: Epilepsy & Behavior: E&B
https://read.qxmd.com/read/30744982/punctate-white-matter-lesions-in-the-full-term-newborn-underlying-aetiology-and-outcome
#2
Michael Hayman, Gerda van Wezel-Meijler, Henrica van Straaten, Eva Brilstra, Floris Groenendaal, Linda S de Vries
BACKGROUND: Punctate white matter lesions (PWMLs) are small focal patches of increased signal intensity (SI) on T1- and decreased SI on T2-weighted magnetic resonance imaging (MRI). To date, there have been few reports of PWMLs in term born infants. OBJECTIVE: To identify associated diagnoses and factors predictive of clinical outcome in (near) term infants with PWMLs. METHODS: MRI studies and clinical records of (near) term infants, with PWMLs on MRI scans performed in two institutions in the first 28 postnatal days were reviewed...
January 26, 2019: European Journal of Paediatric Neurology: EJPN
https://read.qxmd.com/read/30737142/predictive-factors-and-prognostic-value-for-status-epilepticus-in-newborns
#3
Zeynep Gokce-Samar, Karine Ostrowsky-Coste, Dominique Gauthier-Morel, Pascale Keo-Kosal, Julitta De Regnauld De Bellescize, Alexandra Montavont, Eleni Panagiotakaki, Olivier Claris, Alexis Arzimanoglou
OBJECTIVES: To evaluate the predictive factors for status epilepticus (SE) in neonates and prognostic factors for patient outcomes in newborns suffering either isolated seizures or SE. METHODS: A retrospective single-center study from January 2010 to December 2014, included 91 newborns who had neonatal seizures. Among them, 50 newborns experienced SE and 41 newborns presented isolated seizures only. SE was defined as a single seizure lasting more than 15 min or repeated seizures without return to preictal neurological baseline for more than 15 min...
January 29, 2019: European Journal of Paediatric Neurology: EJPN
https://read.qxmd.com/read/30714130/independent-role-of-neonatal-seizures-in-subsequent-neurological-outcomes-a-population-based-study
#4
Ahyuda Oh, David J Thurman, Hyunmi Kim
AIM: This population-based study aimed to estimate the impact of neonatal seizures on subsequent neurological outcomes, regardless of underlying etiology. METHOD: We performed a retrospective cohort study (1st January 2009-31st December 2014), using a USA nationwide claims database. Newborn infants enrolled in 2009 were followed for up to 6 years. Neonatal seizures were identified by combining the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis code of 779...
February 4, 2019: Developmental Medicine and Child Neurology
https://read.qxmd.com/read/30713278/cost-effective-hplc-uv-method-for-quantification-of-vitamin-d-2-and-d-3-in-dried-blood-spot-a-potential-adjunct-to-newborn-screening-for-prophylaxis-of-intractable-paediatric-seizures
#5
Elizabeth Mary Mathew, Sudheer Moorkoth, Pankaj D Rane, Leslie Lewis, Pragna Rao
25-Hydroxyvitamin D (25-(OH)D) deficiency is recently been described as one of the multiple factors responsible for pediatric seizures. 25-Hydroxyvitamin D3 and 25-Hydroxyvitamin D2 are the well-known markers to determine Vitamin D status. In this work we report the development of a sensitive and cost effective HPLC technique for the quantification of the vitamin D metabolites from dried blood spot samples (DBS). The metabolites were extracted using acetonitrile-methanol-0.1% formic acid (60 : 20 : 20 (v/v)) and analyzed on an Acclaim C18 column (150 × 4...
2019: Chemical & Pharmaceutical Bulletin
https://read.qxmd.com/read/30702169/guidance-for-assessment-of-erythroderma-in-neonates-and-infants-for-the-pediatric-immunologist
#6
REVIEW
Hagen Ott
Neonatal and infantile erythroderma (NIE) represent the common clinical phenotype of heterogenous diseases ranging from benign and transient skin conditions to fatal multiorgan disorders. NIE regularly demands a comprehensive diagnostic workup in a multiprofessional setting, especially if newborns and young infants with the disease develop a failure to thrive and concomitant infectious, neurological or metabolic complications. By obtaining a detailed medical history and performing a thorough clinical examination, targeted diagnostic steps can be scheduled for most affected children...
January 31, 2019: Pediatric Allergy and Immunology
https://read.qxmd.com/read/30691927/recessive-gm3-synthase-deficiency-natural-history-biochemistry-and-therapeutic-frontier
#7
Lauren E Bowser, Millie Young, Olivia K Wenger, Zineb Ammous, Karlla W Brigatti, Vincent J Carson, Teresa Moser, James Deline, Kazuhiro Aoki, Thierry Morlet, Ethan M Scott, Erik G Puffenberger, Donna L Robinson, Christine Hendrickson, Jonathan Salvin, Steven Gottlieb, Adam D Heaps, Michael Tiemeyer, Kevin A Strauss
GM3 synthase, encoded by ST3GAL5, initiates synthesis of all downstream cerebral gangliosides. Here, we present biochemical, functional, and natural history data from 50 individuals homozygous for a pathogenic ST3GAL5 c.862C>T founder allele (median age 8.1, range 0.7-30.5 years). GM3 and its derivatives were undetectable in plasma. Weight and head circumference were normal at birth and mean Apgar scores were 7.7 ± 2.0 (1 min) and 8.9 ± 0.5 (5 min). Somatic growth failure, progressive microcephaly, global developmental delay, visual inattentiveness, and dyskinetic movements developed within a few months of life...
January 21, 2019: Molecular Genetics and Metabolism
https://read.qxmd.com/read/30690204/biallelic-loss-of-function-variants-in-atp1a2-cause-hydrops-fetalis-microcephaly-arthrogryposis-and-extensive-cortical-malformations
#8
Fabiola P Monteiro, Cynthia J Curry, Robert Hevner, Stephen Elliott, Jamie H Fisher, John Turocy, William B Dobyns, Larissa A Costa, Erika Freitas, João Paulo Kitajima, Fernando Kok
The Na+ /K+ - ATPase acts as an ion pump maintaining the essential plasma membrane potential in all mammalian cell types, and is essential for many cellular functions. There are four α isoforms (α1, α2, α3 and α4) with distinct expression patterns, kinetic properties and substrate affinity. The α2-isoform is encoded by ATP1A2 and evidence supports its utmost importance in Cl- homeostasis in neurons, and in the function of respiratory neurons at birth. Monallelic pathogenic variants in ATP1A2 are associated with familial hemiplegic migraine type 2 (FHM2) and on rare occasions with alternating hemiplegia of childhood 1 (AHC1)...
January 25, 2019: European Journal of Medical Genetics
https://read.qxmd.com/read/30688037/sleep-disordered-breathing-is-common-among-term-and-near-term-infants-in-the-nicu
#9
Meera S Meerkov, Fauziya Hassan, Ronald D Chervin, John D Barks, Martha D Carlson, Renée A Shellhaas
OBJECTIVE: Among older infants and children, sleep-disordered breathing (SDB) has negative neurocognitive consequences. We evaluated the frequency and potential impact of SDB among newborns who require intensive care. STUDY DESIGN: Term and near-term newborns at risk for seizures underwent 12-h attended polysomnography in the neonatal intensive care unit (NICU). Bayley Scales of Infant Development, third edition (Bayley-III) were administered at 18-22 months. RESULT: The 48 newborns (EGA 39...
January 27, 2019: Pediatric Pulmonology
https://read.qxmd.com/read/30679288/levetiracetam-optimal-dose-finding-as-first-line-treatment-for-neonatal-seizures-occurring-in-the-context-of-hypoxic-ischaemic-encephalopathy-levneonat-1-study-protocol-of-a-phase-ii-trial
#10
Geraldine Favrais, Moreno Ursino, Catherine Mouchel, Estelle Boivin, Vincent Jullien, Sarah Zohar, Elie Saliba
INTRODUCTION: Therapeutic schedules for treating neonatal seizures remain elusive. First-line treatment with phenobarbital is widely supported but without strong scientific evidence. Levetiracetam (LEV) is an emerging and promising antiepileptic drug (AED). The aim of this phase II trial is to determine the benefits of LEV by applying a strict methodology and to estimate the optimal dose of LEV as a first-line AED to treat seizures in newborns suffering from hypoxic-ischaemic encephalopathy...
January 24, 2019: BMJ Open
https://read.qxmd.com/read/30676288/lack-of-variability-in-cerebral-oximetry-tendency-in-infants-with-severe-hypoxic-ischemic-encephalopathy-under-hypothermia
#11
María Arriaga-Redondo, Juan Arnaez, Isabel Benavente-Fernández, Simón Lubián-López, Montesclaros Hortigüela, Cristina Vega-Del-Val, Alfredo Garcia-Alix
Cerebral oximetry using near-infrared spectroscopy (NIRS) provides continuous, noninvasive assessment of the degree of hemoglobin saturation of the brain tissue. Previous studies suggest that high values of regional cerebral tissue oxygen saturation (rScO2 ) during the first days in neonates with significant hypoxic-ischemic encephalopathy (HIE) are correlated with an adverse neurological outcome. However, the results are not consistent among the studies. To examine the correlation of rScO2 values and their variability over time with HIE severity, amplitude integrated electroencephalography (aEEG) background and seizure activity, neuron-specific enolase levels in cerebrospinal fluid, magnetic resonance imaging (MRI) findings, and neurological outcome...
January 24, 2019: Therapeutic Hypothermia and Temperature Management
https://read.qxmd.com/read/30663059/identification-of-a-novel-biomarker-for-pyridoxine-dependent-epilepsy-implications-for-newborn-screening
#12
Michael F Wempe, Amit Kumar, Vijay Kumar, Yu J Choi, Michael A Swanson, Marisa W Friederich, Keith Hyland, Wyatt W Yue, Johan L K Van Hove, Curtis R Coughlin
Pyridoxine-dependent epilepsy (PDE) is often characterized as an early onset epileptic encephalopathy with dramatic clinical improvement following pyridoxine supplementation. Unfortunately, not all patients present with classic neonatal seizures or respond to an initial pyridoxine trial, which can result in the under diagnosis of this treatable disorder. Restriction of lysine intake and transport is associated with improved neurologic outcomes, although treatment should be started in the first year of life to be effective...
January 21, 2019: Journal of Inherited Metabolic Disease
https://read.qxmd.com/read/30657134/hypothermia-for-newborns-with-hypoxic-ischemic-encephalopathy
#13
REVIEW
Brigitte Lemyre, Vann Chau
Therapeutic hypothermia is a standard of care for infants ≥36 weeks gestational age (GA) with moderate-to-severe hypoxic-ischemic encephalopathy. Because some studies included infants born at 35 weeks GA, hypothermia should be considered if they meet other criteria. Cooling for infants <35 weeks GA is not recommended. Passive cooling should be started promptly in community centres, in consultation with a tertiary care centre neonatologist, while closely monitoring the infant's temperature. Best evidence suggests that maintaining core body temperature between 33°C and 34°C for 72 hours, followed by a period of rewarming of 6 to 12 hours, is optimal...
July 2018: Paediatrics & Child Health
https://read.qxmd.com/read/30656683/association-between-indel-polymorphism-rs145204276-in-the-promoter-region-of-lncrna-gas5-and-the-risk-of-febrile-convulsion
#14
REVIEW
Wenna Xiang, Zhishu Li, Zongze Lin, Keyou You, Minli Pan, Ge Zheng
BACKGROUND: This study aimed to explore the regulatory relationship between growth arrest special 5 (GAS5) and interleukin-1β (IL-1β) implicated in the development of febrile seizure (FS). METHOD: The presence of FS and the genotype of GAS5 were used as two different indicators to divide the 50 newborn babies, recruited in this study, into different groups. The potential regulatory relationship among GAS5, miR-21, and IL-1β was identified by measuring their expression using quantitative reverse-transcription polymerase chain reaction and immunohistochemistry assays among different sample groups...
January 17, 2019: Journal of Cellular Physiology
https://read.qxmd.com/read/30642234/pattern-of-brain-injury-predicts-long-term-epilepsy-following-neonatal-encephalopathy
#15
Qi Xu, Vann Chau, Chinnuwat Sanguansermsri, Katherine E Muir, Emily W Y Tam, Steven P Miller, Darren S T Wong, Hao Chen, Peter K H Wong, Jill G Zwicker, Kenneth J Poskitt, Alan Hill, Elke H Roland
OBJECTIVE: To determine if patterns of hypoxic-ischemic brain injury on magnetic resonance imaging (MRI) in term newborns predict subsequent childhood epilepsy. Methods: This retrospective cohort study includes term newborns with encephalopathy (n = 181) born between 2004-2012 and admitted to British Columbia Children's Hospital. MRI was performed between 3 and 5 days of age. The predominant patterns of hypoxic-ischemic injury were classified as Normal, Watershed, Basal Nuclei, Total, and Focal-Multifocal...
January 15, 2019: Journal of Child Neurology
https://read.qxmd.com/read/30619713/severe-cystic-degeneration-and-intractable-seizures-in-a-newborn-with-molybdenum-cofactor-deficiency-type-b
#16
Fady Hannah-Shmouni, Lauren MacNeil, Murray Potter, Rebekah Jobling, Grace Yoon, Suzanne Laughlin, Susan Blaser, Michal Inbar-Feigenberg
Newborns with cystic degeneration with or without intractable seizures should be investigated for inborn errors of metabolism, including molybdenum cofactor deficiency (MoCoD). MoCoD may present with non-specific hypoxic ischemic injury in the neonatal period with MRI showing extensive prenatally acquired cystic encephalomalacia involving grey and white matter. Most newborns with MoCoD will present with normal head size and brain appearance at birth and postnatally rapidly develop cystic encephalomalacia. A significant minority will present with signs of prenatal brain injury or malformation...
March 2019: Molecular Genetics and Metabolism Reports
https://read.qxmd.com/read/30615984/effects-of-oral-domoic-acid-exposure-on-maternal-reproduction-and-infant-birth-characteristics-in-a-preclinical-nonhuman-primate-model
#17
Thomas M Burbacher, Kimberly S Grant, Rebekah Petroff, Sara Shum, Brenda Crouthamel, Courtney Stanley, Noelle McKain, Jing Jing, Nina Isoherranen
Domoic Acid (DA) is a naturally-occurring excitotoxin, produced by marine algae, which can bioaccumulate in shellfish and finfish. The consumption of seafood contaminated with DA is associated with gastrointestinal illness that, in the case of high DA exposure, can evolve into a spectrum of responses ranging from agitation to hallucinations, memory loss, seizures and coma. Because algal blooms that produce DA are becoming more widespread and very little is known about the dangers of chronic, low-dose exposure, we initiated a preclinical study focused on the reproductive and developmental effects of DA in a nonhuman primate model...
January 4, 2019: Neurotoxicology and Teratology
https://read.qxmd.com/read/30585100/parental-perceptions-of-neonatal-therapeutic-hypothermia-emotional-and-healing-experiences
#18
Alexa K Craig, Christine James, Janelle Bainter, Scott Evans, Roslyn Gerwin
INTRODUCTION: Parents of infants who undergo therapeutic hypothermia experience emotional challenges that have not been fully characterized. Comprehensive understanding of the parental experience of hypothermia is needed to provide better care to the family of the infant. This study aimed to improve the understanding of the parental emotional experience of therapeutic hypothermia in the Neonatal Intensive Care Unit (NICU). METHODS: Semistructured interviews were conducted in a group setting with parents matched into groups according to the severity of the infant's presenting encephalopathy...
December 26, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://read.qxmd.com/read/30567812/chromosome-16p13-11-microdeletion-syndrome-in-a-newborn-a-case-study
#19
Amanda Elizabeth Smith, Amy Jnah, Desi Newberry
Chromosome 16p13.11 microdeletion syndrome is a rare copy number variant that carries increased risks for complications in the neonatal period and throughout the life span. Clinical manifestations and associated defects known to present in the neonatal period include motor delay, facial dysmorphisms, microcephaly, gastroesophageal reflux disease (GERD), and congenital heart defects. Management in the neonatal period focuses on associated comorbidities, including motor delay with or without GERD, which commonly manifests as feeding difficulties...
September 2018: Neonatal Network: NN
https://read.qxmd.com/read/30507953/rna-sequencing-analysis-of-umbilical-cord-plasma-micrornas-from-healthy-newborns
#20
Gary P Brennan, Dimitrios M Vitsios, Sophie Casey, Ann-Marie Looney, Boubou Hallberg, David C Henshall, Geraldine B Boylan, Deirdre M Murray, Catherine Mooney
MicroRNAs are a class of small non-coding RNA that regulate gene expression at a post-transcriptional level. MicroRNAs have been identified in various body fluids under normal conditions and their stability as well as their dysregulation in disease has led to ongoing interest in their diagnostic and prognostic potential. Circulating microRNAs may be valuable predictors of early-life complications such as birth asphyxia or neonatal seizures but there are relatively few data on microRNA content in plasma from healthy babies...
2018: PloS One
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