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Pediatric encephalopathy

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https://www.readbyqxmd.com/read/28434268/ubiquitin-c-terminal-hydrolase-l1-uch-l1-as-a-therapeutic-and-diagnostic-target-in-neurodegeneration-neurotrauma-and-neuro-injuries
#1
Kevin K Wang, Zhihui Yang, George Sarkis, Isabel Torres, Vijaya Raghavan
Since its discovery as a major CNS-abundant protein 25 years ago, Ubiquitin C-terminal hydrolase-L1 (UCH-L1) has emerged as an important enzyme in regulating brain protein metabolism, by coupling to the proteasome pathway of protein degradation. Areas covered: UCH-L1 is implicated in both familial and sporadic Parkinson disease and other chronic neurodegenerative diseases. Also, UCH-L1 has been recently emerging as a biofluid-based biomarker for various forms of acute neurotrauma and CNS injury. Expert opinion: The loss of UCH-L1 activity coupled with the gain of proteinopathy function are linked to neurodegeneration such as Parkinsonism and Alzheimer's disease...
April 24, 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28430674/continuous-eeg-in-pediatric-critical-care-yield-and-efficiency-of-seizure-detection
#2
Arnold J Sansevere, Elizabeth D Duncan, Mark H Libenson, Tobias Loddenkemper, Phillip L Pearl, Robert C Tasker
PURPOSE: Our goal was to define the duration of continuous EEG (cEEG) monitoring needed to adequately capture electrographic seizures and EEG status epilepticus in the pediatric intensive care unit using clinical and background EEG features. METHODS: Retrospective study of patients aged 1 month to 21 years admitted to a tertiary pediatric intensive care unit and undergoing cEEG (>3 hours). Clinical data collected included admission diagnosis, EEG background features, and time variables including time to first seizure after initiation of cEEG...
April 20, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28426698/extensive-alterations-of-blood-metabolites-in-pediatric-cerebral-malaria
#3
Sanchit Gupta, Karl Seydel, Miguel A Miranda-Roman, Catherine M Feintuch, Alex Saidi, Ryung S Kim, Gretchen L Birbeck, Terrie Taylor, Johanna P Daily
Cerebral malaria (CM) presents as an encephalopathy and is due to infection with Plasmodium falciparum. Patients are comatose, often with fever, recurrent seizures and this condition is associated with a high mortality rate. The etiology of the coma and seizures are poorly understood. Circulating small molecules and lipids have bioactive functions and alterations in their concentrations have been implicated in seizure disorders and other forms of encephalopathy. We carried out a comprehensive analysis of blood metabolites during CM to explore a biochemical basis of this encephalopathy...
2017: PloS One
https://www.readbyqxmd.com/read/28420900/intracranial-pressure-monitoring-for-pediatric-acute-encephalopathy
#4
Nobuyuki Nosaka, Kohei Tsukahara, Emily Knaup, Toshihiko Yabuuchi, Tomonobu Kikkawa, Yosuke Fujii, Masato Yashiro, Takao Yasuhara, Ayumi Okada, Toyomu Ugawa, Atsunori Nakao, Hirokazu Tsukahara, Isao Date
Newly published clinical practice guidelines recommend intracranial pressure (ICP) monitoring in critical care for the management of pediatric acute encephalopathy (pAE), but the utility of ICP monitoring for pAE has been poorly studied. We recently performed direct ICP monitoring for two patients. We observed that although the direct ICP monitoring had clinical benefits with less body weight gain and no vasopressor use in both cases, this monitoring technique is still invasive. Future studies should determine the utility of non-invasive ICP monitoring systems in pAE to further improve the quality of intensive-care management...
April 2017: Acta Medica Okayama
https://www.readbyqxmd.com/read/28413125/transient-dysautonomia-in-an-acute-phase-of-encephalopathy-with-biphasic-seizures-and-late-reduced-diffusion
#5
Yuko Ichimiya, Noriyuki Kaku, Yasunari Sakai, Fumiya Yamashita, Wakato Matsuoka, Mamoru Muraoka, Satoshi Akamine, Soichi Mizuguchi, Michiko Torio, Yoshitomo Motomura, Yuichiro Hirata, Yoshito Ishizaki, Masafumi Sanefuji, Hiroyuki Torisu, Hidetoshi Takada, Yoshihiko Maehara, Shouichi Ohga
Paroxysmal sympathetic hyperactivity (PSH) is a dysautonomic condition that is associated with various types of acquired brain injuries. Traumatic brain lesions have been documented as the leading cause of PSH. However, detailed clinical features of pediatric PSH caused by intrinsic brain lesions remain to be elusive. We present a 3-year-old boy, who had been diagnosed as having cerebral palsy, developmental delay and epilepsy after perinatal hypoxia-induced brain injury. He developed status epilepticus with fever on the third day of respiratory infection...
April 13, 2017: Brain & Development
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#6
Filipa Dias Costa, Rita Moinho, Sandra Ferreira, Paula Garcia, Luísa Diogo, Isabel Gonçalves, Carla Pinto
INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...
April 11, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28376079/pattern-of-brain-injury-and-depressed-heart-rate-variability-in-newborns-with-hypoxic-ischemic-encephalopathy
#7
Marina Metzler, Rathinaswamy Govindan, Tareq Al-Shargabi, Gilbert Vezina, Nickie Andescavage, Yunfei Wang, Adre du Plessis, An N Massaro
BACKGROUND: Decreased heart rate variability (HRV) is a measure of autonomic dysfunction and brain injury in newborns with hypoxic ischemic encephalopathy (HIE). This study aimed to characterize the relationship between HRV and brain injury pattern by MRI in newborns with HIE undergoing therapeutic hypothermia. METHODS: HRV metrics were quantified in the time domain (αS, αL, and root mean square at short [RMSS] and long [RMSL] time scales) and frequency domain (relative low-[LF] and high-frequency [HF] power) during the time period 24-27 h of life...
April 4, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28372869/a-pediatric-case-of-reversible-cerebral-vasoconstriction-syndrome-with-similar-radiographic%C3%A2-findings-to-posterior-reversible-encephalopathy-syndrome
#8
Tomoya Kamide, Taishi Tsutsui, Kouichi Misaki, Hiroki Sano, Masanao Mohri, Naoyuki Uchiyama, Mitsutoshi Nakada
BACKGROUND: Reversible cerebral vasoconstriction syndrome occurs predominantly in middle-aged women. Only nine pediatric cases of this syndrome have been reported. PATIENT DESCRIPTION: We present a ten-year-old boy with reversible cerebral vasoconstriction syndrome with radiographic findings similar to those of posterior reversible encephalopathy syndrome (PRES). He presented with a thunderclap headache without a neurological deficit. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral arteries, whereas magnetic resonance imaging (MRI) with diffusion-weighted imaging and fluid-attenuated inversion recovery demonstrated hyperintense lesions in the occipital lobes and the left cerebellum...
February 20, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28338570/a-case-of-all-developing-posterior-reversible-encephalopathy-secondary-to-hyponatremia
#9
Nilgun Eroglu, Aysenur Bahadir, Erol Erduran
Posterior reversible encephalopathy syndrome (PRES), may be due to different causes. It may develop secondary to hypertension, renal decompensation, electrolyte imbalance, and chemotherapeutic drugs. We describe a case of acute lymphoblastic leukemia in which PRES developed secondary to hyponatremia despite being normotensive during receipt of chemotherapy. Magnetic resonance imaging findings were suggestive of PRES. Partial diffusion restriction was observed in lesions in the bilateral occipitoparietal regions and the cerebellum...
March 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28320216/a-neurologic-dysfunction-scoring-protocol-for-jaundiced-neonates-requiring-exchange-transfusion
#10
Bolajoko O Olusanya, Folashade B Osibanjo, Adeniyi A Ajiboye, Oluwafemi E Ayodele, Adebanke A Odunsi, Serah M Olaifa, Abieyuwa A Emokpae
AIM: To evaluate the performance of a neurologic assessment protocol among jaundiced infants requiring exchange transfusion (ET). METHODS: We identified infants in a referral children's hospital who received ET and those who met the American Academy of Pediatrics (AAP) criteria for ET based on total serum bilirubin (TSB) levels. The performance of a bilirubin-induced neurologic dysfunction (BIND-M) scoring protocol for acute bilirubin encephalopathy (ABE) in detecting infants treated with ET in both groups was investigated by logistic regression analysis and c-statistic...
March 20, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#11
Iliyana H Pacheva, Ivan S Ivanov, Krastina Stefanova, Elena Chepisheva, Lyubov Chochkova, Dafina Grozeva, Angelina Stoyanova, Stojan Milenkov, Penka Stefanova, Anna Petrova
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28245682/-hypothermia-treatment-in-asphyxiated-neonates-a-single-center-experience-in-hungary
#12
Kata Kovács, Enikő Szakmár, Ünőke Méder, Márton Kolossváry, Zsolt Bagyura, Lilla Lamboy, Zsuzsanna Élő, Attila Szabó, Miklós Szabó, Ágnes Jermendy
INTRODUCTION AND AIM: We aimed to analyze patient characteristics of term neonates with hypoxic-ischemic encephalopathy treated with hypothermia at the 3rd level Neonatal Intensive Care Unit of the 1st Department of Pediatrics, Semmelweis University. METHOD: We conducted a retrospective cohort analysis between 2013-2015, including 97 asphyxiated neonates with HIE who received hypothermia treatment, using our in-house developed novel registry database. RESULTS: 59...
March 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28245506/should-posterior-reversible-encephalopathy-syndrome-be-mainly-considered-an-epileptic-disorder-results-of-a-sequential-neurophysiological-study-in-a-pediatric-cohort
#13
Daniele Grioni, Fabio Pavan, Giulia Prunotto, Francesco Canonico, Carlo de Grandi, Attilio Rovelli
Despite a wide number of studies trying to define clinical, physiopathological, and neuroradiological features of posterior reversible encephalopathy syndrome (PRES), the true nature of symptoms is still not fully understood. We studied a standard cohort of 24 pediatric patients, affected by hemato-oncological diseases, with a neuroradiological diagnosis consistent with PRES identified from 2006 to 2013. Ten of them developed PRES after hematopoietic stem cell transplantation. We analyzed the sequence of clinical, radiological, and electrophysiological data...
April 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28173649/-a-novel-mutation-in-kcnb1-gene-in-a-child-with-neuropsychiatric-comorbidities-with-both-intellectual-disability-and-epilepsy-and-review-of-literature
#14
P Miao, J Peng, C Chen, N Gai, F Yin
Objective: To explore the association between the phenotype and KCNB1 gene mutation. Method: Clinical information including physical features, laboratory and genetic data of one patient of mental retardation with refractory epilepsy from Department of Pediatrics, Xiangya Hospital in January 2016 was analyzed. This patient was discovered to have KCNB1 gene mutations through whole exome sequencing. Relevant information about KCNB1 gene mutation was searched and collected from Pubmed, CNKI, Human Gene Mutation Database(HGMD) and Online Mendelian Inheritance in Man(OMIM)...
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28166364/interrater-agreement-in-the-interpretation-of-neonatal-electroencephalography-in-hypoxic-ischemic-encephalopathy
#15
Courtney J Wusthoff, Joseph Sullivan, Hannah C Glass, Renée A Shellhaas, Nicholas S Abend, Taeun Chang, Tammy N Tsuchida
OBJECTIVE: Research using neonatal electroencephalography (EEG) has been limited by a lack of a standardized classification system and interpretation terminology. In 2013, the American Clinical Neurophysiology Society (ACNS) published a guideline for standardized terminology and categorization in the description of continuous EEG in neonates. We sought to assess interrater agreement for this neonatal EEG categorization system as applied by a group of pediatric neurophysiologists. METHODS: A total of 60 neonatal EEG studies were collected from three institutions...
March 2017: Epilepsia
https://www.readbyqxmd.com/read/28157839/establishment-of-a-method-for-evaluating-endothelial-cell-injury-by-tnf-%C3%AE-in-vitro-for-clarifying-the-pathophysiology-of-virus-associated-acute-encephalopathy
#16
Kyohei Miyazaki, Koichi Hashimoto, Masatoki Sato, Masahiro Watanabe, Naoki Tomikawa, Shuto Kanno, Yukihiko Kawasaki, Nobuo Momoi, Mitsuaki Hosoya
BACKGROUND: Virus-associated acute encephalopathy (VAE) is a severe central nervous system complication caused by common viral infections in children. The pathophysiology of VAE is thought to be endothelial injury. This study was designed to establish an in vitro VAE model for evaluating endothelial injury caused by the proinflammatory cytokine TNF-α. METHODS: Transwell-grown human umbilical vein endothelial cells (HUVECs) monolayers were incubated with serially diluted TNF-α...
February 3, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28145649/clinical-characteristics-of-transplant-associated-encephalopathy-in-children
#17
Yun Jeong Lee, Mi Sun Yum, Eun Hee Kim, Min Jee Kim, Kyung Mo Kim, Ho Joon Im, Young Hwue Kim, Young Seo Park, Tae Sung Ko
We aimed to analyze characteristics of encephalopathy after both hematopoietic stem cell and solid organ pediatric transplantation. We retrospectively reviewed medical records of 662 pediatric transplant recipients (201 with liver transplantation [LT], 55 with heart transplantation [HT], and 67 with kidney transplantation [KT], 339 with allogeneic hematopoietic stem cell transplantation [HSCT]) who received their graft organs at Asan Medical Center between January 2000 and July 2014. Of the 662 patients, 50 (7...
March 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28141916/justifying-nonstandard-exception-requests-for-pediatric-liver-transplant-candidates-an-analysis-of-narratives-submitted-to-the-united-network-for-organ-sharing-2009-2014
#18
E R Perito, H J Braun, J L Dodge, S Rhee, J P Roberts
Nonstandard exception requests (NSERs), for which transplant centers provide patient-specific narratives to support a higher Model for End-stage Liver Disease/Pediatric End-stage Liver Disease score, are made for >30% of pediatric liver transplant candidates. We describe the justifications used in pediatric NSER narratives 2009-2014 and identify justifications associated with NSER denial, waitlist mortality, and transplant. Using United Network for Organ Sharing data, 1272 NSER narratives from 1138 children with NSERs were coded for analysis...
January 31, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28141793/sex-specific-associations-between-cerebrovascular-blood-pressure-autoregulation-and-cardiopulmonary-injury-in-neonatal-encephalopathy-and-therapeutic-hypothermia
#19
Raul Chavez-Valdez, Matthew O'Connor, Jamie Perin, Michael Reyes, Jillian Armstrong, Charlamaine Parkinson, Maureen Gilmore, Jacky Jennings, Frances J Northington, Jennifer K Lee
BACKGROUND: Cardiopulmonary injury is common in neonatal encephalopathy, but the link with cerebrovascular dysfunction is unknown. We hypothesized that alterations of cerebral autoregulation are associated with cardiopulmonary injury in neonates treated with therapeutic hypothermia (TH) for neonatal encephalopathy. METHODS: The cerebral hemoglobin volume index (HVx) from near-infrared spectroscopy was used to identify the mean arterial blood pressure (MAP) with optimal autoregulatory vasoreactivity (MAPOPT)...
March 1, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28141600/preventing-pressure-injuries-in-neonates-undergoing-therapeutic-hypothermia-for-hypoxic-ischemic-encephalopathy-an-interprofessional-quality-improvement-project
#20
Alexandra Luton, Jae Hernandez, Clive Robert Patterson, Jill Nielsen-Farrell, Anita Thompson, Jeffrey R Kaiser
BACKGROUND: Hospital-acquired pressure injuries (HAPIs) can be caused by multiple factors including pressure, shear, friction, moisture/incontinence, device-related pressure, immobility, inactivity, and nutritional deficits. Along with immobility, medical device-related (MDR) HAPIs are a primary cause of pressure injury in neonates, as the clinical practice setting has become increasingly technologically advanced. It is estimated that up to 50% of HAPIs are MDR in pediatric patients. Neonates are at particular risk for HAPI because of their specific anatomical, physiological, and developmental vulnerabilities...
January 30, 2017: Advances in Neonatal Care: Official Journal of the National Association of Neonatal Nurses
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