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Pediatric electrophysiology

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https://www.readbyqxmd.com/read/29915537/preclinical-animal-models-for-dravet-syndrome-seizure-phenotypes-comorbidities-and-drug-screening
#1
REVIEW
Aliesha Griffin, Kyla R Hamling, SoonGweon Hong, Mana Anvar, Luke P Lee, Scott C Baraban
Epilepsy is a common chronic neurological disease affecting almost 3 million people in the United States and 50 million people worldwide. Despite availability of more than two dozen FDA-approved anti-epileptic drugs (AEDs), one-third of patients fail to receive adequate seizure control. Specifically, pediatric genetic epilepsies are often the most severe, debilitating and pharmaco-resistant forms of epilepsy. Epileptic syndromes share a common symptom of unprovoked seizures. While some epilepsies/forms of epilepsy are the result of acquired insults such as head trauma, febrile seizure, or viral infection, others have a genetic basis...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29908370/gender-differences-in-patients-with-brugada-syndrome-and-arrhythmic-events-data-from-a-survey-on-arrhythmic-events-in-678-patients
#2
Anat Milman, Jean-Baptiste Gourraud, Antoine Andorin, Pieter G Postema, Frederic Sacher, Philippe Mabo, Giulio Conte, Carla Giustetto, Georgia Sarquella-Brugada, Aviram Hochstadt, Sung-Hwan Kim, Jimmy Jm Juang, Shingo Maeda, Yoshihide Takahashi, Tsukasa Kamakura, Takeshi Aiba, Eran Leshem, Yoav Michowitz, Michael Rahkovich, Yuka Mizusawa, Elena Arbelo, Zhengrong Huang, Isabelle Denjoy, Yanushi D Wijeyeratne, Carlo Napolitano, Ramon Brugada, Ruben Casado-Arroyo, Jean Champagne, Leonardo Calo, Jacob Tfelt-Hansen, Silvia G Priori, Masahiko Takagi, Christian Veltmann, Pietro Delise, Domenico Corrado, Elijah R Behr, Fiorenzo Gaita, Gan-Xin Yan, Josep Brugada, Antoine Leenhardt, Arthur A M Wilde, Pedro Brugada, Kengo F Kusano, Kenzo Hirao, Gi-Byoung Nam, Vincent Probst, Bernard Belhassen
BACKGROUND: There is limited information on gender differences in patients with Brugada syndrome (BrS) who experienced arrhythmic events (AEs). OBJECTIVES: To compare clinical, electrocardiographic (ECG), electrophysiologic (EP) and genetic characteristics between males and females in BrS-patients with their first AE. METHODS: The multicenter Survey on AE in BrS (SABRUS) collected data on first AE in 678 BrS-patients including 619 (91.3%) males and 59 (8...
June 13, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29903452/functional-topography-of-the-human-cerebellum
#3
Catherine J Stoodley, Jeremy D Schmahmann
Accumulating evidence points to a critical role for the human cerebellum in both motor and nonmotor behaviors. A core tenet of this new understanding of cerebellar function is the existence of functional subregions within the cerebellum that differentially support motor, cognitive, and affective behaviors. This cerebellar functional topography - based on converging evidence from neuroanatomic, neuroimaging, and clinical studies - is evident in both adult and pediatric populations. The sensorimotor homunculi in the anterior lobe and lobule VIII established in early tract tracing and electrophysiologic studies are evident in both task-based and resting-state human functional imaging studies...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29893416/clinical-and-electrophysiological-properties-of-atrial-tachycardia-after-pediatric-heart-transplantation
#4
Diana E Drogalis-Kim, Roberto G Gallotti, Benjamin A Blais, Greg Perens, Jeremy P Moore
BACKGROUND: Pediatric heart transplant recipients are at an elevated risk for development of atrial tachycardia (AT) however the underlying mechanisms and long-term outcomes are unclear. OBJECTIVE: We hypothesized that occurrence of AT in pediatric heart transplant recipients would be associated with a higher frequency of adverse events during follow-up. METHODS: A single center retrospective review of all pediatric heart transplant recipients with suspected AT between 1997 and 2017 was performed...
June 12, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29798793/delirium-cordis-an-arrhythmia-at-the-intersection-of-pediatric-and-adult-electrophysiology
#5
EDITORIAL
J Philip Saul, Martin J LaPage
No abstract text is available yet for this article.
May 2018: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/29742889/intraoperative-neurophysiological-monitoring-a-review-of-techniques-used-for-brain-tumor-surgery-in-children
#6
REVIEW
Keewon Kim, Charles Cho, Moon-Suk Bang, Hyung-Ik Shin, Ji-Hoon Phi, Seung-Ki Kim
Intraoperative monitoring (IOM) utilizes electrophysiological techniques as a surrogate test and evaluation of nervous function while a patient is under general anesthesia. They are increasingly used for procedures, both surgical and endovascular, to avoid injury during an operation, examine neurological tissue to guide the surgery, or to test electrophysiological function to allow for more complete resection or corrections. The application of IOM during pediatric brain tumor resections encompasses a unique set of technical issues...
May 2018: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/29692269/clinical-observation-of-electroencephalographic-changes-and-risk-of-convulsion-occurrence-in-children-receiving-neural-precursor-cell-transplantation
#7
Suqing Qu, Weipeng Liu, Hui Yang, Zhaoyan Wang, Yinxiang Yang, Fang Liu, Aruna Sharma, Hari Sharma, Zuo Luan
PURPOSE: This study was intended to observe electroencephalographic (EEG) changes and convulsion attacks in children receiving neural precursor cell transplantation, and to explore the possibility of electrophysiological changes and risk of convulsion occurrence after cell transplantation. METHOD: Included in this study were 227 children who received neural precursor cell transplantation in our hospital between March 2008 and July 2012. No history of convulsion attacks was elicited before cell transplantation...
April 24, 2018: CNS & Neurological Disorders Drug Targets
https://www.readbyqxmd.com/read/29685473/acute-pediatric-colchicine-toxicity-is-associated-with-marked-bradydysrhythmias
#8
Lamya Mubayed, Brie-Ann A Muller, Jessica L Jacobson, Howard A Hast, Hoang H Nguyen
BACKGROUND: Colchicine ingestion is rare but highly lethal. Patients usually die of multiorgan failure and cardiogenic shock. Colchicine is not only associated with depressed myocardial function but also with fatal heart rhythm disturbances, such as complete heart block, ventricular tachycardia, and asystole. While histologic changes of myocytes are well known, the mechanism by which colchicine affects cardiac impulse generation and conduction is not fully understood. CASE REPORT: We present a case of colchicine ingestion with sinus bradycardia, marked sinus arrhythmia, and first- and second-degree heart block...
April 20, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29604150/neurophysiological-study-of-critical-illness-polyneuropathy-and-myopathy-in-mechanically-ventilated-children-additional-aspects-in-paediatric-critical-illness-comorbidities
#9
A Thabet Mahmoud, M A M Tawfik, S A Abd El Naby, W M M Abo El Fotoh, N Y Saleh, N M S Abd El Hady
BACKGROUND AND PURPOSE: Critical illness polyneuropathy and myopathy (CIP/CIM) is being increasingly recognized as a significant clinical problem in critically ill children especially if they have spent long periods in the intensive care unit. So the aim was to determine the frequency of CIP/CIM amongst mechanically ventilated children and to analyse the associated risk factors and drawbacks frequently encountered in this cohort. METHODS: The study included 105 patients admitted to the paediatric intensive care unit who underwent mechanical ventilation for ≥7 days...
March 31, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29579186/arrhythmias-in-congenital-heart-disease-a-position-paper-of-the-european-heart-rhythm-association-ehra-association-for-european-paediatric-and-congenital-cardiology-aepc-and-the-european-society-of-cardiology-esc-working-group-on-grown-up-congenital-heart-disease
#10
Antonio Hernández-Madrid, Thomas Paul, Dominic Abrams, Peter F Aziz, Nico A Blom, Jian Chen, Massimo Chessa, Nicolas Combes, Nikolaos Dagres, Gerhard Diller, Sabine Ernst, Alessandro Giamberti, Joachim Hebe, Jan Janousek, Thomas Kriebel, Jose Moltedo, Javier Moreno, Rafael Peinado, Laurent Pison, Eric Rosenthal, Jonathan R Skinner, Katja Zeppenfeld
The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy...
March 20, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29574705/a-comprehensive-approach-to-identifying-repurposed-drugs-to-treat-scn8a-epilepsy
#11
Talia A Atkin, Chani M Maher, Aaron C Gerlach, Bryant C Gay, Brett M Antonio, Sonia C Santos, Karen M Padilla, JulieAnn Rader, Douglas S Krafte, Matthew A Fox, Gregory R Stewart, Slavé Petrovski, Orrin Devinsky, Matthew Might, Steven Petrou, David B Goldstein
OBJECTIVE: Many previous studies of drug repurposing have relied on literature review followed by evaluation of a limited number of candidate compounds. Here, we demonstrate the feasibility of a more comprehensive approach using high-throughput screening to identify inhibitors of a gain-of-function mutation in the SCN8A gene associated with severe pediatric epilepsy. METHODS: We developed cellular models expressing wild-type or an R1872Q mutation in the Nav 1.6 sodium channel encoded by SCN8A...
April 2018: Epilepsia
https://www.readbyqxmd.com/read/29564522/antibiotic-prophylaxis-practices-in-pediatric-cardiac-implantable-electronic-device-procedures-a-survey-of-the-pediatric-and-congenital-electrophysiology-society-paces
#12
Spenser Y Chen, Scott R Ceresnak, Kara S Motonaga, Anthony Trela, Debra Hanisch, Anne M Dubin
Cardiac implantable electronic device (CIED) infections are associated with significant morbidity in the pediatric device population, with a tenfold higher risk of infection in children compared to adults. The 2010 American Heart Association (AHA) guidelines recommend a single dose of systemic antibiotic (ABX) prophylaxis prior to CIED implantation and no post-operative (OP) ABX. However, there is limited data regarding adherence to this recommendation among the pediatric community. To assess current clinical practices for CIED ABX prophylaxis in pediatrics; whether the AHA guidelines are being followed; and if not, the reasons for non-adherence...
March 22, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29506771/electrophysiologic-features-of-radial-neuropathy-in-childhood-and-adolescence
#13
Ioannis Karakis, Sofia Georghiou, H Royden Jones, Basil T Darras, Peter B Kang
BACKGROUND: We analyzed the clinical and electrophysiologic patterns of nerve injury in pediatric patients with radial neuropathy. METHODS: This is a retrospective analysis of 19 children and adolescents with radial neuropathy. RESULTS: The mean subject age was 12 years (range one month to 19 years), 56% were female, and 53% had traumatic etiologies. Weakness in the finger and wrist extensors was the prevailing complaint (82%). Predominant localization was at the posterior interosseous nerve (37%), followed by the radial nerve below the spiral groove (32%), the radial nerve at the spiral groove (26%), and the radial nerve above the spiral groove (5%)...
April 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29500504/development-and-implementation-of-a-standardized-heparin-protocol-for-left-sided-pediatric-electrophysiology-procedures
#14
Emily F Moore, Jennifer Pak, Christa Jefferis-Kirk, Arlene Armatage, Richard A Kronmal, Jack C Salerno, Matthew D Files
Heparin is used to decrease the risk of thromboembolic complications during electrophysiology studies (EPS); however, there is wide practice variation and minimal evidence to guide heparin dosing, particularly in pediatric patients. This study retrospectively analyzed heparin dosing and response, measured via activated clotting time (ACT), in patients undergoing EPS and used these data (pre-protocol cohort, n = 40), as well as guidance from available literature to implement a standardized heparin protocol (phase 1, n = 43)...
March 2, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29476598/rhythm-disturbances-and-treatment-strategies-in-children-with-congenitally-corrected-transposition-of-the-great-arteries
#15
Taner Kasar, Pelin Ayyildiz, Gulhan Tunca Sahin, Erkut Ozturk, Selman Gokalp, Sertac Haydin, Alper Guzeltas, Yakup Ergul
BACKGROUND: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected transposition of the great arteries (ccTGA) and associated treatment strategies. PATIENTS AND METHODS: This retrospective cohort study included 65 pediatric patients with ccTGA who were admitted to the clinic between 2009 and 2017. The patients were divided into two groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electrophysiological data, and device implantation data on the two groups were compared...
May 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29415597/prediction-of-postnatal-arrhythmia-in-fetuses-with-cardiac-rhabdomyoma
#16
Tetsuya Ide, Takekazu Miyoshi, Shinji Katsuragi, Reiko Neki, Ken-Ichi Kurosaki, Isao Shiraishi, Jun Yoshimatsu, Tomoaki Ikeda
OBJECTIVES: Fetal cardiac rhabdomyomas are rare but well-known to be associated with arrhythmia or conduction abnormalities. However, since in utero electrophysiological information is quite limited, it remains unclear which type of rhabdomyoma will develop arrhythmia after birth. The aim of this study is to identify factors that predict postnatal arrhythmia requiring therapy in fetuses with cardiac rhabdomyoma. STUDY DESIGN: A retrospective review of infants prenatally diagnosed with cardiac rhabdomyoma was performed at our tertiary pediatric cardiac center between 1990 and 2016...
February 20, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29397525/coronary-sinus-morphology-in-pediatric-patients-with-supraventricular-tachycardia
#17
Matthew B Ambrose, Jennifer N Avari Silva, Michael Rudokas, Tammy M Bowman, Joshua Murphy, George F Van Hare
PURPOSE: The anatomic basis of atrioventricular node reentrant tachycardia (AVNRT) remains incompletely characterized in children. Differences in coronary sinus (CS) size and morphology have been observed in adults with AVNRT but have not been well characterized in children. METHODS: Children (< 18 years) brought to the EP lab with supraventricular tachycardia for ablation underwent CS venography. A blinded pediatric interventional cardiologist performed CS measurements, which were indexed to body surface area...
March 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/29376095/massive-cortical-reorganization-is-reversible-following-bilateral-transplants-of-the-hands-evidence-from-the-first-successful-bilateral-pediatric-hand-transplant-patient
#18
William Gaetz, Sudha K Kessler, Tim P L Roberts, Jeffrey I Berman, Todd J Levy, Michelle Hsia, Deborah Humpl, Erin S Schwartz, Sandra Amaral, Ben Chang, Lawrence Scott Levin
In this repeated measures case study, we show that sensory deafferentation after limb amputation leads to changes in cortical somatotopic maps which are reversible after restoration of sensory input. Using magnetoencephalography (MEG), we observed in a child with bilateral hand transplants large-scale shifts in somatosensory lip cortical representation from anatomic hand area to anatomic face region. After recovery of tactile sensation in the digits, responses to finger stimulation were localized to orthotopic sensory cortex, but with atypical electrophysiologic features (amplitude and frequencies)...
January 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29366866/international-consensus-icon-on-audiological-assessment-of-hearing-loss-in-children
#19
A Farinetti, A Raji, H Wu, B Wanna, C Vincent
The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Early diagnosis and rehabilitation of congenital hearing loss are mandatory in order to achieve a satisfactory linguistic and cognitive development. Without appropriate opportunities to learn language, these children will fall behind their normal hearing peers in communication, cognition, reading and socio-emotional development. After promising results, neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out...
February 2018: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/29333742/parvalbumin-fast-spiking-interneurons-are-selectively-altered-by-paediatric-traumatic-brain-injury
#20
Joshua Nichols, George Reed Bjorklund, Jason Newbern, Trent Anderson
KEY POINTS: Traumatic brain injury (TBI) in children remains a leading cause of death and disability and it remains poorly understood why children have worse outcomes and longer recover times. TBI has shown to alter cortical excitability and inhibitory drive onto excitatory neurons, yet few studies have directly examined changes to cortical interneurons. This is addressed in the present study using a clinically relevant model of severe TBI (controlled cortical impact) in interneuron cell type specific Cre-dependent mice...
April 1, 2018: Journal of Physiology
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