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Pediatric encephalopathy

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https://www.readbyqxmd.com/read/28790849/immunotargeting-relapsed-or-refractory-precursor-b-cell-acute-lymphoblastic-leukemia-role-of-blinatumomab
#1
REVIEW
Manon Queudeville, Rupert Handgretinger, Martin Ebinger
Patients with refractory or relapsed (R/R) acute lymphoblastic leukemia (ALL) have a dismal prognosis of around 5% long-term survival when treated with cytotoxic chemotherapy and allogenic stem cell transplantation. T-cell immunobased strategies open up new therapeutic perspectives. Blinatumomab is the first of a new class of antibody constructs that was labeled bispecific T-cell engager (BiTE): it consists of two single chain variable fragment connected with a flexible linker, one side binding CD3, the other CD19...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28770374/mog-antibody-related-disorders-common-features-and-uncommon-presentations
#2
Álvaro Cobo-Calvo, Anne Ruiz, Hyacintha D'Indy, Anne-Lise Poulat, Maryline Carneiro, Nicolas Philippe, Françoise Durand-Dubief, Kumaran Deiva, Sandra Vukusic, Vincent Desportes, Romain Marignier
Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed...
August 2, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28770118/new-onset-insomnia-in-a-pediatric-patient-a-case-of-anti-nmda-receptor-encephalitis
#3
Tamar N Goldberg, Michael F Cellucci
Anti-NMDAR encephalitis is becoming more widely recognized as a cause of encephalopathy in both adults and children. Certain clinical features such as mood lability, movement disorders, speech dysfunction, seizures, and autonomic instability in a pediatric patient should prompt immediate concern and evaluation for autoimmune encephalitis among providers. We present the case of a pediatric patient with anti-NMDAR encephalitis in which the symptom prompting medical evaluation was insomnia. Insomnia has not previously been emphasized in the literature as a presenting feature of this disease in children and has a broad differential...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28762041/neuroinfluenza-evaluation-of-seasonal-influenza-associated-severe-neurological-complications-in-children-a-multicenter-study
#4
Muhammet Sukru Paksu, Kerim Aslan, Tanil Kendirli, Basak Nur Akyildiz, Nazik Yener, Riza Dincer Yildizdas, Mehmet Davutoglu, Ayhan Yaman, Sedat Isikay, Gulnar Sensoy, Haydar Ali Tasdemir
PURPOSE: Although influenza primarily affects the respiratory system, in some cases, it can cause severe neurological complications. Younger children are especially at risk. Pediatric literature is limited on the diagnosis, treatment, and prognosis of influenza-related neurological complications. The aim of the study was to evaluate children who suffered severe neurological manifestation as a result of seasonal influenza infection. METHODS: The medical records of 14 patients from six hospitals in different regions of the country were evaluated...
July 31, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28757754/therapeutic-hypothermia-brings-favorable-neurologic-outcomes-in-children-with-near-drowning
#5
Ming-Chun Chen, Chia-Hsiang Chu, Ching-Feng Cheng, Jun-Song Lin, Jui-Hsia Chen, Yu-Hsun Chang
A 1-year-10-month-old boy was admitted to our pediatric intensive care unit due to near drowning with pulmonary edema. A conventional ventilator with 100% oxygen supplementation was used initially, but was shifted to high frequency oscillatory ventilation as his oxygen saturation was around 84-88%. Therapeutic hypothermia was applied due to hypoxic ischemic encephalopathy with severe acidosis. His respiratory condition improved and he was extubated successfully on the 6(th) hospital day. The patient had no obvious neurological defects and he was discharged in a stable condition after 17 days of hospitalization...
October 2016: Ci Ji Yi Xue za Zhi, Tzu-chi Medical Journal
https://www.readbyqxmd.com/read/28747606/effective-therapy-with-infliximab-for-clinically-mild-encephalitis-encephalopathy-with-a-reversible-splenial-lesion-in-an-infant-with-kawasaki-disease
#6
Yoshie Kurokawa, Hiroshi Masuda, Tohru Kobayashi, Hiroshi Ono, Hitoshi Kato, Ken-Ichi Imadome, Jun Abe, Yuichi Abe, Shuichi Ito, Akira Ishiguro
  Kawasaki disease (KD) is a systemic vasculitis in infants. In KD, encephalopathy is rarely (0.1%) associated, however, clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) has previously been reported in some pediatric patients. Here, we report on a 2-year-old girl who had KD complicated with MERS. The patient experienced generalized clonic convulsion and prolonged consciousness disturbance with fever for 2 days. Her head MRI showed a high signal intensity lesion in the splenium of the corpus callosum in diffusion-weighted images, and low apparent diffusion coefficient (ADC) values on day 3...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28706729/a-retrospective-review-of-the-pediatric-development-clinic-implementation-a-model-to-improve-medical-nutritional-and-developmental-outcomes-of-at-risk-under-five-children-in-rural-rwanda
#7
Eric Ngabireyimana, Christine Mutaganzwa, Catherine M Kirk, Ann C Miller, Kim Wilson, Evodia Dushimimana, Olivier Bigirumwami, Evelyne S Mukakabano, Fulgence Nkikabahizi, Hema Magge
BACKGROUND: As more high-risk newborns survive the neonatal period, they remain at significant medical, nutritional, and developmental risk. However, no follow-up system for early intervention exists in most developing countries. In 2014, a novel Pediatric Development Clinic (PDC) was implemented to provide comprehensive follow-up to at-risk under-five children, led by nurses and social workers in a district hospital and surrounding health centers in rural Rwanda. METHODS: At each PDC visit, children undergo clinical/nutritional assessment and caregivers participate in counseling sessions...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/28698729/neurological-and-vascular-manifestations-of-ethylmalonic-encephalopathy
#8
Ali Reza Tavasoli, Parastoo Rostami, Mahmoud Reza Ashrafi, Parvaneh Karimzadeh
Objective Ethylmalonic encephalopathy (EE) is a severe mitochondrial disease of early infancy clinically characterized by a combination of developmental delay, progressive pyramidal signs, and vascular lesions including petechial purpura, orthostatic acrocyanosis, and chronic hemorrhagic diarrhea. Biochemical hallmarks of the disease are persistently high level of lactate, and C4-C5-acylcarnitines in blood, markedly elevated urinary excretion of methylsuccinic and ethylmalonic (EMA) acids. Here we report two patients with EE as a 16-months-old male infant and a 2-yr-old boy referred to Pediatric Neurology Clinic in Children's Medical Center, Tehran-Iran that in one patient genetic analysis revealed a homozygous mutation of the ETHE1 gene in favor of ethylmalonic acidemia...
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28695691/neonatal-therapeutic-hypothermia-outside-of-standard-guidelines-a-survey-of-u-s-neonatologists
#9
Jennifer Burnsed, Santina A Zanelli
AIM: Therapeutic hypothermia is standard of care in term infants with moderate-to-severe hypoxic-ischemic encephalopathy (HIE). The goal of this survey was to explore the attitudes of U.S. neonatologists caring for infants with HIE who fall outside of current guidelines. METHOD: Case-based survey administered to members of the Section on Neonatal-Perinatal Medicine of American Academy of Pediatrics. RESULTS: 447 responses were analyzed, i...
July 11, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28665923/non-invasive-continuous-cardiac-output-and-cerebral-perfusion-monitoring-in-term-infants-with-neonatal-encephalopathy-assessment-of-feasibility-and-reliability
#10
Eva Forman, Colm R Breatnach, Stephanie Ryan, Jana Semberova, Jan Miletin, Adrienne Foran, Afif El-Khuffash
BACKGROUND: None invasive hemodynamic monitoring of infants with neonatal encephalopathy (NE) undergoing therapeutic hypothermia (TH) would be a potentially useful clinical tool. We aimed assess the feasibility and reliability of non-invasive cardiac output monitoring (NICOM) and near infrared spectroscopy (NIRS) in this cohort. METHODS: NICOM and NIRS were commenced to measure cardiac output (CO), systemic vascular resistance (SVR), blood pressure (BP), and cerebral regional oxygen saturations (SctO2) during TH and rewarming...
June 30, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28619060/incidence-disease-onset-and-short-term-outcome-in-urea-cycle-disorders-cross-border-surveillance-in-germany-austria-and-switzerland
#11
Susanne Nettesheim, Stefan Kölker, Daniela Karall, Johannes Häberle, Roland Posset, Georg F Hoffmann, Beate Heinrich, Florian Gleich, Sven F Garbade
BACKGROUND: Urea cycle disorders (UCDs) are a group of rare inherited metabolic disorders. Affected individuals often present with hyperammonemic encephalopathy (HE) and have an increased risk of severe neurologic disease and early death. The study aims to provide epidemiologic data and to describe the disease manifestation and short-term outcome. METHOD: Cross-border surveillance of newly diagnosed patients with UCDs - below 16 years of age - was performed from July 2012 to June 2015 in Germany and Austria and from January 2012 to December 2015 in Switzerland...
June 15, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28605011/use-of-the-ketogenic-diet-to-manage-refractory-epilepsy-in-cdkl5-disorder-experience-of-100-patients
#12
Zhan Lim, Kingsley Wong, Heather E Olson, Ann M Bergin, Jenny Downs, Helen Leonard
OBJECTIVE: Pathogenic variants involving the CDKL5 gene result in a severe epileptic encephalopathy, often later presenting with features similar to Rett syndrome. Cardinal features of epilepsy in the CDKL5 disorder include early onset at a median age of 6 weeks and poor response to antiepileptic drugs. The ketogenic diet (KD) was first introduced in the 1920s as a treatment option for refractory epilepsy in children. This study investigated use of the KD in the CDKL5 disorder and its influences on seizures...
June 12, 2017: Epilepsia
https://www.readbyqxmd.com/read/28596990/magnetic-resonance-imaging-of-cerebral-malaria-patients-reveals-distinct-pathogenetic-processes-in-different-parts-of-the-brain
#13
Sanjib Mohanty, Laura A Benjamin, Megharay Majhi, Premanand Panda, Sam Kampondeni, Praveen K Sahu, Akshaya Mohanty, Kishore C Mahanta, Rajyabardhan Pattnaik, Rashmi R Mohanty, Sonia Joshi, Anita Mohanty, Ian W Turnbull, Arjen M Dondorp, Terrie E Taylor, Samuel C Wassmer
The mechanisms underlying the rapidly reversible brain swelling described in patients with cerebral malaria (CM) are unknown. Using a 1.5-Tesla (T) magnetic resonance imaging (MRI) scanner, we undertook an observational study in Rourkela, India, of 11 Indian patients hospitalized with CM and increased brain volume. Among the 11 cases, there were 5 adults and 6 children. All patients had reduced consciousness and various degrees of cortical swelling at baseline. The latter was predominately posterior in distribution...
May 2017: MSphere
https://www.readbyqxmd.com/read/28565819/genetic-basis-of-pediatric-epilepsy-syndromes
#14
Dongli Zhang, Xiaoming Liu, Xingqiang Deng
Childhood epilepsy affects ~0.5-1% in the general population worldwide. Early-onset epileptic encephalopathies are considered to be severe neurological disorders, which lead to impaired motor, cognitive, and sensory development due to recurrence of seizures. Many of the observed epilepsy phenotypes are associated with specific chromosomal imbalances and thus display gene dosage effects, and also specific mutations of a variety of genes ranging from ion channels to transcription factors. High throughput sequencing technologies and whole exome sequencing have led to the recognition of several new candidate genes with a possible role in the pathogenesis of epileptic encephalopathies...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28561815/effects-of-therapeutic-hypothermia-on-white-matter-injury-from-murine-neonatal-hypoxia-ischemia
#15
Elliot Koo, R Ann Sheldon, Byong Sop Lee, Zinaida S Vexler, Donna M Ferriero
BackgroundTherapeutic hypothermia (TH) is the standard of care for neonates with hypoxic-ischemic encephalopathy, but it is not fully protective in the clinical setting. Hypoxia-ischemia (HI) may cause white matter injury (WMI), leading to neurological and cognitive dysfunction.MethodsP9 mice were subjected to HI as previously described. Pups underwent 3.5 h of systemic hypothermia or normothermia. Cresyl violet and Perl's iron staining for histopathological scoring of brain sections was completed blindly on all brains...
May 31, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28558955/seizures-in-preterm-neonates-a-multicenter-observational-cohort-study
#16
Hannah C Glass, Renée A Shellhaas, Tammy N Tsuchida, Taeun Chang, Courtney J Wusthoff, Catherine J Chu, M Roberta Cilio, Sonia L Bonifacio, Shavonne L Massey, Nicholas S Abend, Janet S Soul
BACKGROUND: The purpose of this study was to characterize seizures among preterm neonates enrolled in the Neonatal Seizure Registry, a prospective cohort of consecutive neonates with seizures at seven pediatric centers that follow the American Clinical Neurophysiology Society's neonatal electroencephalography monitoring guideline. STUDY DESIGN: Of 611 enrolled neonates with seizures, 92 (15%) were born preterm. Seizure characteristics were evaluated by gestational age at birth for extremely preterm (<28 weeks, N = 18), very preterm (28 to <32 weeks, N = 18), and moderate to late preterm (32 to <37 weeks, N = 56) and compared with term neonates...
July 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28555455/-vagus-nerve-stimulation-treatment-of-158-pediatric-patients-with-a-long-term-follow-up
#17
S Flesler, G Reyes, S Fortini, B Ramos, R Cersosimo, M Bartuluchi, R Caraballo
AIM: To describe a series of patients with drug resistant epilepsy treated with vagus nerve stimulation in a national pediatric hospital, evaluating efficacy, safety and tolerability. PATIENTS AND METHODS: A retrospective analysis of 158 pediatric patients with epilepsy resistant to pharmacological and non pharmacological treatment including surgery that were treated with vagus nerve stimulation between 2001-2015. Patients with progressive encephalopathies, and congenital heart disease were excluded...
June 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28533163/grin2b-gain-of-function-mutations-are-sensitive-to-radiprodil-a-negative-allosteric-modulator-of-glun2b-containing-nmda-receptors
#18
Brice Mullier, Christian Wolff, Zara Amanda Sands, Philippe Ghisdal, Pierandrea Muglia, Rafal Marian Kaminski, Véronique Marie André
De novo gain of function mutations in GRIN2B encoding the GluN2B subunit of the N-methyl-d-aspartate (NMDA) receptor have been linked with epileptic encephalopathies, including infantile spasms. We investigated the effects of radiprodil, a selective GluN2B negative allosteric modulator and other non-selective NMDA receptor inhibitors on glutamate currents mediated by NMDA receptors containing mutated GluN2B subunits. The experiments were performed in Xenopus oocytes co-injected with the following human mRNAs: GRIN1/GRIN2B, GRIN1/GRIN2B-R540H, GRIN1/GRIN2B-N615I and GRIN1/GRIN2B-V618G...
May 19, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28503704/use-of-eeg-in-critically-ill-children-and-neonates-in-the-united-states-of-america
#19
Marina Gaínza-Lein, Iván Sánchez Fernández, Tobias Loddenkemper
The objective of the study was to estimate the proportion of patients who receive an electroencephalogram (EEG) among five common indications for EEG monitoring in the intensive care unit: traumatic brain injury (TBI), extracorporeal membrane oxygenation (ECMO), cardiac arrest, cardiac surgery and hypoxic-ischemic encephalopathy (HIE). We performed a retrospective cross-sectional descriptive study utilizing the Kids' Inpatient Database (KID) for the years 2010-2012. The KID is the largest pediatric inpatient database in the USA and it is based on discharge reports created by hospitals for billing purposes...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28483998/hypoxia-treatment-reverses-neurodegenerative-disease-in-a-mouse-model-of-leigh-syndrome
#20
Michele Ferrari, Isha H Jain, Olga Goldberger, Emanuele Rezoagli, Robrecht Thoonen, Kai-Hung Chen, David E Sosnovik, Marielle Scherrer-Crosbie, Vamsi K Mootha, Warren M Zapol
The most common pediatric mitochondrial disease is Leigh syndrome, an episodic, subacute neurodegeneration that can lead to death within the first few years of life, for which there are no proven general therapies. Mice lacking the complex I subunit, Ndufs4, develop a fatal progressive encephalopathy resembling Leigh syndrome and die at ≈60 d of age. We previously reported that continuously breathing normobaric 11% O2 from an early age prevents neurological disease and dramatically improves survival in these mice...
May 23, 2017: Proceedings of the National Academy of Sciences of the United States of America
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