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Pediatric electrophysiology

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https://www.readbyqxmd.com/read/28319875/electrophysiologic-features-of-ulnar-neuropathy-in-childhood-and-adolescence
#1
Ioannis Karakis, Wendy Liew, Heather Szelag Fournier, H Royden Jones, Basil T Darras, Peter B Kang
OBJECTIVE: To analyze patterns of nerve injury in pediatric ulnar neuropathy (PUN). METHODS: Retrospective analysis of 49 children with PUN. RESULTS: Sensory loss in digit V was the prevailing complaint (89%). Predominant localization was at the elbow (55%). Diminished ulnar SNAP was the most common abnormality (71%) with median axon loss estimate (MAXE) of 62%. Dorsal ulnar cutaneous (DUC) sensory nerve action potential (SNAP) was reduced in 55% with MAXE of 43%...
February 20, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28274505/limited-fluoroscopy-catheter-ablation-of-accessory-pathways-in-children
#2
Moshe Swissa, Einat Birk, Tamir Dagan, Sody Abby Naimer, Michal Fogelman, Tom Einbinder, Elchanan Bruckheimer, Rami Fogelman
BACKGROUND: Limited fluoroscopy ablation using 3D electro-anatomical system (3DS) has been used for arrhythmias in children, however it is not a common practice. We aimed to facilitate a fluoroscopy limited approach for ablation of accessory pathways (AP) in children. METHODS: Following electrophysiologic (EP) catheter placement a single dual-plane fluoroscopic image (right anterior oblique-30° and left anterior oblique-60° views) was acquired and the 3DS views were rotated to be a perfect match to the fluoroscopy...
March 5, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28273405/heart-rhythm-society-expert-consensus-statements-part-2
#3
REVIEW
Susie Sennhauser, Rishi Anand, Fred Kusumoto, Nora Goldschlager
One of the most important roles for professional societies in medicine is assembling multiple stakeholders and experts to develop documents that can help guide and define policies and strategies for best medical care. Each year the Heart Rhythm Society (HRS) develops several consensus documents that address critical clinical subjects that have been identified by input from HRS members and HRS committees. Over the past 5 years, HRS has produced documents with multiple professional societies from around the world, and although the topics chosen for exploration center around arrhythmia management, the reviews and recommendations made in the documents are important for clinical cardiologists and generalists who are not arrhythmia specialists...
March 8, 2017: Clinical Cardiology
https://www.readbyqxmd.com/read/28273360/heart-rhythm-society-expert-consensus-statements-part-1
#4
REVIEW
Susie Sennhauser, Rishi Anand, Fred Kusumoto, Nora Goldschlager
One of the most important roles for professional societies in medicine is assembling multiple stakeholders and experts to develop documents that can help guide and define policies and strategies for best medical care. Each year the Heart Rhythm Society (HRS) develops several consensus documents that address critical clinical subjects that have been identified by input from HRS members and HRS committees. Over the past 5 years, HRS has produced documents with multiple professional societies from around the world, and although the topics chosen for exploration center around arrhythmia management, the reviews and recommendations made in the documents are important for clinical cardiologists and generalists who are not arrhythmia specialists...
March 8, 2017: Clinical Cardiology
https://www.readbyqxmd.com/read/28265208/effectiveness-of-early-invasive-therapy-for-atrial-tachycardia-in-adult-atrial-baffle-survivors
#5
Elisa A Bradley, Ali N Zaidi, Justin Morrison, Curt J Daniels, Steven Kalbfleisch, Naomi J Kertesz
Adults who underwent complex atrial baffling as children via Mustard or Senning procedures are at heightened risk for atrial arrhythmias. Antiarrhythmic therapies are typically ineffective in this population. Accordingly, our team of pediatric and adult electrophysiologists investigated the effectiveness of early invasive transbaffle-access techniques to perform early radiofrequency ablation at the source of these clinically significant arrhythmias. For this retrospective study, we selected 11 adult survivors of atrial baffling (mean age, 34 ± 9 yr) who underwent clinically indicated electrophysiologic study after no more than one trial of antiarrhythmic therapy...
February 2017: Texas Heart Institute Journal
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
#6
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/28228960/early-onset-hirayama-disease-in-a-female
#7
Matthias Baumann, Josef Finsterer, Elke R Gizewski, Wolfgang N Löscher
OBJECTIVES: Hirayama disease is a rare myelopathy, occurring predominantly in males with onset in the teens. METHODS AND RESULTS: Here, we report a young female patient who developed the first signs of Hirayama disease at 10.5 years of age. Prior to onset, she had experienced a growth spurt and grew about 8 cm. The disease progressed over 3 years and the typical clinical, electrophysiological, and neuroimaging signs of Hirayama disease were found. After this period and achievement of her final height, no further progression was noticed...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28225736/effects-of-long-term-musical-training-on-cortical-auditory-evoked-potentials
#8
Carolyn J Brown, Eun-Kyung Jeon, Virginia Driscoll, Bruna Mussoi, Shruti Balvalli Deshpande, Kate Gfeller, Paul J Abbas
OBJECTIVE: Evidence suggests that musicians, as a group, have superior frequency resolution abilities when compared with nonmusicians. It is possible to assess auditory discrimination using either behavioral or electrophysiologic methods. The purpose of this study was to determine if the acoustic change complex (ACC) is sensitive enough to reflect the differences in spectral processing exhibited by musicians and nonmusicians. DESIGN: Twenty individuals (10 musicians and 10 nonmusicians) participated in this study...
March 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28224647/electrical-impedance-myography-eim-in-individuals-with-col6-and-lama2-congenital-muscular-dystrophy-a-cross-sectional-and-two-year-analysis
#9
Carmel Nichols, Minal S Jain, Katherine G Meilleur, Tianxia Wu, James Collins, Melissa R Waite, Jahannaz Dastgir, Anam Salman, Sandra Donkervoort, Tina Duong, Katherine Keller, Meganne E Leach, Donovan J Lott, Michelle N McGuire, Leslie Nelson, Anne Rutkowski, Carole Vuillerot, Carsten G Bönnemann, Tanya J Lehky
INTRODUCTION: Electrical impedance myography (EIM) is a non-invasive electrophysiological technique that characterizes muscle properties through bioimpedance. We compared EIM measurements to function, strength, and disease severity in a population with congenital muscular dystrophy (CMD). METHODS: Forty-one patients with CMD, either collagen 6 related disorders (COL6-RD) (n = 21) or laminin alpha 2-related disorders (LAMA2-RD) (n = 20) and 21 healthy pediatric controls underwent 2 yearly EIM exams...
February 22, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28217155/acute-bulbar-palsy-plus-syndrome-a-rare-variant-of-guillain-barre-syndrome
#10
Sanghamitra Ray, Prakash Chand Jain
Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide both in adult and pediatric population. Although flaccid paralysis is the hallmark of this disease, there are some rare variants which may be easily missed unless suspected. Here, we present a very rare variant of GBS - acute bulbar palsy plus syndrome in a pediatric patient. A 13-year-old female child presented with right-sided lower motor neuron type of facial palsy and palsy of bilateral glossopharyngeal and vagus nerve of 2 weeks duration...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28213194/subcortical-mapping-using-an-electrified-cusa-in-pediatric-supratentorial-surgery
#11
Jonathan Roth, Akiva Korn, Yifat Bitan-Talmor, Rivka Kaufman, Margaret Ekstein, Shlomi Constantini
BACKGROUND: Intraoperative electrophysiology is increasingly used for various lesion resections, both in adult and pediatric brain surgery. Subcortical mapping is often used in adult surgery when lesions lie in proximity to the corticospinal tract (CST). We describe a novel technique of continuous subcortical mapping using an electrified Cavitron UltraSonic Aspirator (CUSA) in children with supratentorial lesions. METHODS: We evaluated the method of subcortical mapping using a CUSA as a stimulation probe...
February 14, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28191656/utility-and-safety-of-combined-interventional-catheterization-and-electrophysiology-procedures-in-a-children-s-hospital
#12
S Yukiko Asaki, Jeffrey W Orcutt, Christina Y Miyake, Henri Justino, Caridad M DE LA Uz, Jeffrey J Kim, Santiago O Valdes, Athar M Qureshi
BACKGROUND: Interventional cardiac catheterization (cath) and electrophysiology (EP) procedures are not routinely performed together. There are several perceived barriers affecting this practice, though there are also advantages for both the patient and practitioner to a combined approach. METHODS: This was a single center retrospective study reviewing combined cath and EP procedures with a pre-procedural intention to intervene at Texas Children's Hospital from 2001 to 2014...
February 13, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28148335/a-pediatric-cardiology-fellowship-boot-camp-improves-trainee-confidence
#13
Catherine K Allan, Paul Tannous, Elizabeth DeWitt, Michael Farias, Laura Mansfield, Christina Ronai, David Schidlow, Stephen P Sanders, James E Lock, Jane W Newburger, David W Brown
Introduction New paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children's Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice. METHODS: The PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28108086/long-term-risk-of-wolff-parkinson-white-pattern-and-syndrome
#14
REVIEW
Susan S Kim, Bradley P Knight
For years, conventional wisdom has held that patients with asymptomatic ventricular pre-excitation (asymptomatic WPW or WPW pattern) were at low risk for adverse outcomes. This assumption has been challenged more recently in a number of observational/natural history studies as well as in prospective trials in which patients were more aggressively studied via invasive electrophysiology study (EPS) and more aggressively treated, in some cases, with pre-emptive catheter ablation, despite the lack of symptoms. In sum, the data do not definitively support one approach (early, up-stream EPS and/or ablation) vs...
December 5, 2016: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28091832/utility-and-safety-of-the-safesept%C3%A2-transseptal-guidewire-for-electrophysiology-studies-with-catheter-ablation-in-pediatric-and-congenital-heart-disease
#15
Joseph J Knadler, Jeffrey B Anderson, Ahmad S Chaouki, Richard J Czosek, Chad Connor, Timothy K Knilans, David S Spar
PURPOSE: The atrial transseptal procedure is used in catheter ablation of left-sided arrhythmias. Studies in adult patients have shown the SafeSept™ transseptal guidewire (SSTG) to be effective in atrial transseptal procedures. We analyzed our 5-year experience with SSTG use in pediatric and congenital heart disease patients undergoing catheter ablation. METHODS: This is a single-center retrospective analysis of patients undergoing catheter ablation from 2009 to 2014...
January 14, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/28011839/patient-specific-modeling-of-left-ventricular-electromechanics-as-a-driver-for-haemodynamic-analysis
#16
Christoph M Augustin, Andrew Crozier, Aurel Neic, Anton J Prassl, Elias Karabelas, Tiago Ferreira da Silva, Joao F Fernandes, Fernando Campos, Titus Kuehne, Gernot Plank
AIMS: Models of blood flow in the left ventricle (LV) and aorta are an important tool for analysing the interplay between LV deformation and flow patterns. Typically, image-based kinematic models describing endocardial motion are used as an input to blood flow simulations. While such models are suitable for analysing the hemodynamic status quo, they are limited in predicting the response to interventions that alter afterload conditions. Mechano-fluidic models using biophysically detailed electromechanical (EM) models have the potential to overcome this limitation, but are more costly to build and compute...
December 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/27903347/-clinical-genetic-and-pathological-features-of-neuronal-ceroid-lipofuscinosis-in-5-chinese-patients
#17
S C Ren, B Q Gao, Y J Wang, X J Wu, Z X Tian, Y L Sun
Objective: To report the clinical, genetic and ultrastructural pathological features of neuronal ceroid lipofuscinosis (NCLs) in 5 Chinese patients. Methods: A total of 5 patients with NCLs were collected from 2013 to 2015 diagnosed by the department of pediatrics of Beijing Tian Tan Hospital. Their clinical, electrophysiological and neuroimaging data of the patients were reviewed.A total of 9 underlying genes of NCLs were tested in 4 cases and their parents.Ultrastructural pathology by skin biopsy was performed in 3 cases respectively...
November 22, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27882734/novel-mutations-in-sh3tc2-in-a-young-japanese-girl-with-charcot-marie-tooth-disease-type-4c
#18
Kazushi Ichikawa, Keita Numasawa, Saoko Takeshita, Akihiro Hashiguchi, Hiroshi Takashima
Charcot-Marie-Tooth disease type 4C (CMT4C) is an autosomal recessive demyelinating form of CMT characterized clinically by early onset and severe spinal deformities, and is caused by mutations in SH3TC2. We describe the case of a 10-year-old Japanese girl diagnosed with CMT4C. The patient developed progressive foot deformities such as marked pes cavus and ankle contracture, with mild muscle weakness in both legs, and generalized areflexia. On electrophysiological studies, motor nerve conduction velocity ranged from 22...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27874252/pediatric-cardiac-readmissions-an-opportunity-for-quality-improvement
#19
Jeffrey H Sacks, Michael Kelleman, Courtney McCracken, Michelle Glanville, Matthew Oster
OBJECTIVE: Hospital readmissions are increasingly becoming a metric for quality in the current landscape of changing and cost effective medicine. However, no 30-d readmission rates have been established for pediatric cardiac medical patients in the United States. Thus, the objective was to determine 30 d readmission rates and risk factors associated with readmission for pediatric cardiac patients, hypothesizing that pediatric cardiac patients would have significantly higher readmission rates than their general pediatric counterparts...
November 22, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27861220/hereditary-neuropathy-with-liability-to-pressure-palsies-diverse-phenotypes-in-childhood
#20
Yohei Harada, Araya Puwanant, David N Herrmann
Hereditary neuropathy with liability to pressure palsies (HNPP) is a rare autosomal-dominant disorder that most commonly produces recurrent painless focal sensory and motor neuropathies often preceded by minor, mechanical stress, or minor trauma. Herein, we report 2 pediatric cases of HNPP with atypical presentations; isolated muscle cramping and toe walking. Electrophysiologic testing disclosed multifocal sensorimotor polyneuropathy with slowing of sensory conduction velocities in both cases, which prompted PMP 22 gene deletion testing...
December 2016: Journal of Clinical Neuromuscular Disease
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