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Pediatric electrophysiology

Patricia E Thomas, Scott L Macicek
BACKGROUND: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. METHODS: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD...
2016: Ochsner Journal
Allison M Bradbury, Jessica H Bagel, Xuntian Jiang, Gary P Swain, Maria L Prociuk, Caitlin A Fitzgerald, Patricia A O'Donnell, Kyle G Braund, Daniel S Ory, Charles H Vite
Globoid cell leukodystrophy (GLD), or Krabbe's disease, is a debilitating and always fatal pediatric neurodegenerative disease caused by a mutation in the gene encoding the hydrolytic enzyme galactosylceramidase (GALC). In the absence of GALC, progressive loss of myelin and accumulation of a neurotoxic substrate lead to incapacitating loss of motor and cognitive function and death, typically by 2 years of age. Currently, there is no cure. Recent convincing evidence of the therapeutic potential of combining gene and cell therapies in the murine model of GLD has accelerated the requirement for validated markers of disease to evaluate therapeutic efficacy...
November 2016: Journal of Neuroscience Research
Ioannis Karakis, Mohammad Khoshnoodi, Wendy Liew, Elizabeth S Nguyen, H Royden Jones, Basil T Darras, Peter B Kang
OBJECTIVE: To analyze patterns of nerve injury in pediatric common fibular neuropathy (CFN). METHODS: A retrospective analysis was performed on data from 53 children with CFN at a pediatric electromyography laboratory. RESULTS: Conduction block at the fibular head was present in 35% of patients. Deep fibular axonal loss was identified in 77%, while superficial fibular axonal loss was identified in 45%. The pathophysiology was predominantly axonal in 72%, mostly demyelinating in 6%, and mixed in 22%...
September 12, 2016: Muscle & Nerve
Shuman He, Tyler C McFayden, Holly F B Teagle, Matthew Ewend, Lillian Henderson, Craig A Buchman
OBJECTIVE: This study aimed to (1) characterize morphological characteristics of the electrically evoked cortical auditory event-related potentials (eERPs) and explore the potential association between onset eERP morphology and auditory versus nonauditory stimulation; (2) assess test-retest reliability of onset eERPs; (3) investigate effects of stimulation level on onset eERPs; and (4) explore the feasibility of using the onset eERP to estimate the lowest stimulation level that can be detected for individual stimulating electrodes in patients with auditory brainstem implants (ABIs)...
August 30, 2016: Ear and Hearing
Hidetoshi Uchida, Miki Nishio, Yumi Omeki, Yuka Takeuchi, Rina Nagata, Shota Oikawa, Arisa Nagatani, Yoshihiko Eryu, Tadayoshi Hata, Tetsushi Yoshikawa
: In patients with a ventricular septal defect, left-to-right shunting increases the left ventricular preload. This pathological change affects myocardial depolarization and repolarization and has the potential to evoke arrhythmogenic substrates. We examined the effect of ventricular septal defects on myocardial repolarization by investigating the variability in the repolarization interval. This retrospective study included 19 patients (mean age, 1.8 ± 2.1 years) who underwent surgical closure (mean left-to-right shunt ratio, 2...
August 23, 2016: Pediatric Cardiology
Vaishnavi Batmanabane, Elise Heon, Tianyang Dai, Prakash Muthusami, Shiyi Chen, Arun Reginald, Shilpa Radhakrishnan, Manohar Shroff
BACKGROUND: The use of MRI in isolated pediatric nystagmus remains a gray area in clinical management. Many clinicians prefer to order an MRI to rule out intracranial pathology despite the lack of clinically significant findings in most cases. OBJECTIVE: To assess the yield of MR imaging in isolated pediatric nystagmus and define a management algorithm to minimize avoidable MRI referrals and streamline MRI protocols. MATERIALS AND METHODS: We reviewed the charts of 148 children who underwent neuro MRI for isolated nystagmus between January 2008 and September 2014...
August 12, 2016: Pediatric Radiology
Pelin Ayyildiz, Alper Güzeltaş, İbrahim Cansaran Tanidir, Taner Kasar, Erkut Öztürk, Yakup Ergül
BACKGROUND/AIM: The aim of this study was to evaluate the transesophageal echocardiography (TEE) findings of pediatric patients in a tertiary center where complex congenital heart surgery and interventional procedures have been performed. MATERIALS AND METHODS: All TEE studies performed between December 2009 and December 2014 were reviewed retrospectively. Patients were divided into 3 groups: perioperative, during interventional procedures, and due to other reasons...
2016: Turkish Journal of Medical Sciences
David Backhoff, Gunter Kerst, Andrea Peters, Monika Lüdemann, Christian Frische, Michaela Horndasch, Gabriele Hessling, Thomas Paul, Ulrich Krause
BACKGROUND: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure. METHODS: Demographic and clinical data as well as data on device implantation, programming, ICD discharges, and complications after atrial baffle procedure for d-TGA from four German centers were analyzed retrospectively...
October 2016: Pacing and Clinical Electrophysiology: PACE
Colleen Psarros, Sarah Love
Newborn hearing screening has led to the early diagnosis of hearing loss in neonates and early device fitting is common, based primarily on electrophysiologic and radiologic information, with some supplementary behavioral measures. Such early fitting of hearing devices, in particular cochlear implants (CIs), has been beneficial to the majority of children implanted under the age of 12 months who meet the cochlear implant candidacy criteria. Comorbidities are common in children with hearing loss, although they may not be evident in neonates and may not emerge until later in infants...
August 2016: Seminars in Hearing
Katarzyna Kurdziel, Barbara Skowronek-Bała, Aleksandra Gergont
Tetany is the abnormal state of increased neuromuscular excitability. It is manifested with muscle cramps and spasms, usually associated with abnormal calcium metabolism. This state can be devided into two main types: tetany with clinical manifestaton (hypocalcemic) and occurred more frequently latent tetany (normocalcemic). In this study was presented the case of a child with electrophysiological and clinical manifestation of latent tetany. We report a case of a female patient who was admitted to the Pediatric Neurology Department in the year 2015...
2016: Przegla̧d Lekarski
Sławomir Kroczka, Katarzyna Stasiak, Marek Kaciński
BACKGROUND: Myasthenia gravis (MG) is an autoimmunologic disorder. It is characterized by various clinical symptoms and their dependency upon the exertion and the rest as well. MATERIAL AND METHODS: Between 2002-20014 in the Neurophysiology Laboratory at the Chair of Pediatric and Adolescent Neurology, Jagiellonian University in Krakow, the electrophysiological repetitive nerve stimulation study were performed in 44 children. The clinical picture and positive electrophysiological test were the ground to diagnose MG in 19 of them (12 girls and 7 boys)...
2016: Przegla̧d Lekarski
Marek Kaciński
The author reminded preclinical years and the work of children's neu- rologists at that time. And next he de- scribed the work of people which from creating the Department of Pediatric Neurology in 1996 created structures of the Department, its 6 Laboratories and the Outpatients Clinic. From 2008 the Department was a part a Chair of Pediatric and Adolescent Neurology, from here the Author also checked teaching and scientific activity of the Chair, the Department, and 2 academic Laboratories of the Neurophysiology and the Clinical Electrophysiology...
2016: Przegla̧d Lekarski
Maria Louraki, Marina Katsalouli, Christina Kanaka-Gantenbein, Nikolitsa Kafassi, Eleni Critselis, Dimitra Kallinikou, Charalampos Tsentidis, Kyriaki Karavanaki
AIM: To evaluate the prevalence of early somatic neuropathy in children and adolescents with Type 1 diabetes mellitus (Type 1 DM) and its association with the presence of glutamic acid decarboxylase and islet antigen-2 autoantibodies (GADA and IA-2A). METHODS: A cross-sectional study was conducted in a hospital-based cohort of pediatric Type 1 DM patients (n=85, mean(±SD) age: 13.5±3.4years, mean(±SD) disease duration 5.5±3.4years). Peripheral neuropathy was assessed with nerve conduction studies (NCS)...
July 2016: Diabetes Research and Clinical Practice
Sherrie Joy A Baysa, Melissa Olen, Ronald J Kanter, Steven B Fishberger
BACKGROUND: Recently, there has been an increasing number of internal medicine-trained electrophysiologists who choose not to test for acute defibrillation efficacy during implantable cardioverter defibrillator (ICD) implantation. It is not known whether this same trend is seen in pediatric electrophysiologists, who care primarily for patients with congenital heart disease or primary electrical problems. METHODS: Through a 14-question survey created on Survey Monkey, we asked the members of the Pediatric and Adult Congenital Electrophysiology Society (PACES) for their approach to ICD implantation...
August 2016: Pacing and Clinical Electrophysiology: PACE
Jan Janoušek, Peter Kubuš
Cardiac resynchronization therapy (CRT) is an established treatment option for adult patients suffering heart failure due to idiopathic or ischemic cardiomyopathy associated with electromechanical dyssynchrony. There is limited evidence suggesting similar efficacy of CRT in patients with congenital heart disease (CHD). Due to the heterogeneity of structural and functional substrates, CRT implantation techniques are different with a thoracotomy or hybrid approach prevailing. Efficacy of CRT in CHD seems to depend on the anatomy of the systemic ventricle with best results achieved in systemic left ventricular patients upgraded to CRT from conventional pacing...
June 2016: Herzschrittmachertherapie & Elektrophysiologie
Duoru Lin, Jingjing Chen, Haotian Lin, Weirong Chen
Visual electrophysiology is widely used in clinical ophthalmology. It is also of significant value in the objective assessment of visual function in adult and pediatric cataract patients and for the diagnosis of and research on retinal and visual pathway diseases. This article systematically reviews visual electrophysiology techniques, their applications in the diagnosis and treatment of adult and pediatric cataracts, and factors influencing the application of visual electrophysiology during surgical treatment for cataracts...
December 2015: Eye Science
Sidney J Segalowitz
Exercise has long been reputed to have beneficial effects on cognitive processes and emotional stability, with obvious applications to lifespan developmental issues in mental health. Over the last 20 years, animal studies have provided potential mechanisms for this link in neurophysiological terms. Only a very small number of studies have applied this model to well-controlled child and adolescent studies of psychological development, with such studies necessarily requiring collaboration across the fields of exercise science and developmental psychology...
May 2016: Pediatric Exercise Science
Rupa Radhakrishnan, James L Leach, Francesco T Mangano, Michael J Gelfand, Leonid Rozhkov, Lili Miles, Hansel M Greiner
BACKGROUND: Cortical dysplasia is the most common cause of pediatric refractory epilepsy. MRI detection of epileptogenic lesion is associated with good postsurgical outcome. Additional electrophysiological information is suggested to be helpful in localization of cortical dysplasia. Educational measures were taken to increase the awareness of cortical dysplasia at our institution in the context of a recent International League Against Epilepsy (ILAE 2011) classification of cortical dysplasia...
September 2016: Pediatric Radiology
Mitchell Cohen, Shubhayan Sanatani, Elizabeth Stephenson, Jon Skinner, Fabrizio Drago, Andrew Davis, Jan Janousek, Eric Rosenthal, Kathryn K Collins, John Triedman
The Pediatric and Congenital Electrophysiology Society (PACES) is a non-profit organisation comprised of individuals dedicated to improving the care of children and young adults with cardiac rhythm disturbances. Although PACES is a predominantly North American-centric organisation, international members have been a part of PACES for the last two decades. This year, PACES expanded its North American framework into a broadly expansive international role. On May 12, 2015, paediatric electrophysiology leaders from within the United States of America and Canada met with over 30 international paediatric electrophysiologists from 17 countries and five continents discussing measures to (1) expand PACES' global vision, (2) address ongoing challenges such as limited resource allocation that may be present in developing countries, (3) expand PACES' governance to include international representation, (4) promote joint international sessions at future paediatric EP meetings, and (5) facilitate a global multi-centre research consortium...
May 2016: Heart Rhythm: the Official Journal of the Heart Rhythm Society
R B Clark, T Schmidt, F B Sachse, D Boyle, Gary S Firestein, W R Giles
Rheumatoid arthritis (RA) is a progressive disease that affects both pediatric and adult populations. The cellular basis for RA has been investigated extensively using animal models, human tissues and isolated cells in culture. However, many aspects of its etiology and molecular mechanisms remain unknown (McInnes & Schett, 2011). Some of the electrophysiological principles that regulate secretion of essential lubricants (hyaluronan and lubricin) and cytokines from synovial fibroblasts (FLS) have been identidied...
April 15, 2016: Journal of Physiology
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