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Pediatric electrophysiology

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https://www.readbyqxmd.com/read/27903347/-clinical-genetic-and-pathological-features-of-neuronal-ceroid-lipofuscinosis-in-5-chinese-patients
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/27810292/special-electrophysiological-characteristics-of-pediatric-idiopathic-ventricular-tachycardia
#5
Shuenn-Nan Chiu, Wei-Lun Wu, Chun-Wei Lu, Kun-Lang Wu, Wei-Chieh Tseng, Ming-Tai Lin, Chien-Chih Chang, Jou-Kou Wang, Mei-Hwan Wu
BACKGROUND: Idiopathic ventricular tachycardia (VT) is an important pediatric arrhythmia but relevant studies in Asian cohort are rare. We determined the electrophysiological characteristics and outcomes of this rhythm disturbance. METHODS: From an institutional database, patients aged <18years without structural heart disease who have sustained monomorphic VT (longer than 30s) during 1995-2016 were enrolled. RESULTS: We enrolled 57 idiopathic VT patients (male:female=31:26)...
October 29, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27793516/-overnight-polysomnography-versus-respiratory-polygraphy-in-the-diagnosis-of-pediatric-obstructive-sleep-apnea
#6
P Franco, H Bourdin, F Braun, J Briffod, I Pin, M-J Challamel
The French Society of Research and Sleep Medicine (SFRMS) organized a meeting on obstructive sleep apnea syndrome (OSAS) in children. A multidisciplinary group of specialists (pulmonologists, ENT surgeons, pediatricians, neurophysiologists, sleep specialists) drew up a consensus document on the value of electrophysiological recordings in the diagnosis of OSAS in children. Technical considerations and recommended sensors, respiratory event definitions, and scoring criteria are presented according to the 2012 and 2014 recommendations of the American Academy of Sleep Medicine (AASM)...
October 25, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27793469/challenges-in-pediatric-chronic-inflammatory-demyelinating-polyneuropathy
#7
Göknur Haliloğlu, Deniz Yüksel, Cağri Mesut Temoçin, Haluk Topaloğlu
Chronic inflammatory demyelinating neuropathy, a treatable immune-mediated disease of the peripheral nervous system is less common in childhood compared to adults. Despite different sets of diagnostic criteria, lack of a reliable biologic marker leads to challenges in diagnosis, follow-up and treatment. Our first aim was to review clinical presentation, course, response to treatment, and prognosis in our childhood patients. We also aimed to document diagnostic and therapeutic pitfalls and challenges at the bedside...
September 22, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27785341/sex-and-gender-medicine-in-physician-clinical-training-results-of-a-large-single-center-survey
#8
Shivani Dhawan, May Bakir, Erika Jones, Sarah Kilpatrick, C Noel Bairey Merz
BACKGROUND: "Sex and Gender Medicine" is a novel medical discipline that takes into account the effects of sex and gender on the health of women and men. The Institute of Medicine in the USA declared in its 2001 and 2010 statements that being a woman or a man significantly impacts the course of diseases, and therefore, this fact must be considered in diagnosis and therapy. We evaluated the representation of Sex and Gender Medicine in clinical training at Cedars-Sinai Medical Center, a large, tertiary, non-profit, academic medical training center in the Western United States...
2016: Biology of Sex Differences
https://www.readbyqxmd.com/read/27784736/characteristics-of-decremental-accessory-pathways-in-children
#9
Allison C Hill, Michael J Silka, Choo Phei Wee, Yaniv Bar-Cohen
BACKGROUND: Although retrograde decremental accessory pathways (DAPs) are thought to typically present as permanent junctional reciprocating tachycardia (permanent junctional reciprocating tachycardia), they may also be diagnosed unexpectedly during electrophysiology study. We aimed to compare the clinical and electrophysiological characteristics of patients with DAPs to an age-matched cohort with nondecremental accessory pathways. METHODS AND RESULTS: We retrospectively studied pediatric patients (<21 years of age) with retrograde DAPs and an age-matched control population with nondecremental accessory pathways who underwent electrophysiology study between 2005 and 2014...
November 2016: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/27782541/babymeg-a-whole-head-pediatric-magnetoencephalography-system-for-human-brain-development-research
#10
Yoshio Okada, Matti Hämäläinen, Kevin Pratt, Anthony Mascarenas, Paul Miller, Menglai Han, Jose Robles, Anders Cavallini, Bill Power, Kosal Sieng, Limin Sun, Seok Lew, Chiran Doshi, Banu Ahtam, Christoph Dinh, Lorenz Esch, Ellen Grant, Aapo Nummenmaa, Douglas Paulson
We developed a 375-channel, whole-head magnetoencephalography (MEG) system ("BabyMEG") for studying the electrophysiological development of human brain during the first years of life. The helmet accommodates heads up to 95% of 36-month old boys in the USA. The unique two-layer sensor array consists of: (1) 270 magnetometers (10 mm diameter, ∼15 mm coil-to-coil spacing) in the inner layer, (2) thirty-five three-axis magnetometers (20 mm × 20 mm) in the outer layer 4 cm away from the inner layer. Additionally, there are three three-axis reference magnetometers...
September 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27779344/dexmedetomidine-use-in-patients-undergoing-electrophysiological-study-for-supraventricular-tachyarrhythmias
#11
Christopher F Tirotta, Tuan Nguyen, Steven Fishberger, Evelio Velis, Melissa Olen, Lourdes Lam, Danielle R Madril, Jessica Hughes, Richard G Lagueruela
BACKGROUND: Dexmedetomidine is a selective alpha-2 adrenergic agonist with sedative, analgesic, and anxiolytic properties. Dexmedetomidine has not been approved for use in pediatrics. Dexmedetomidine has been reported to depress sinus node and atrioventricular nodal function in pediatric patients; it has been suggested that the use of dexmedetomidine may not be desirable during electrophysiological studies. AIM: We hypothesize that the use of dexmedetomidine does not inhibit the induction of supraventricular tachyarrhythmias (SVT) during electrophysiological studies and does not inhibit the ablation of such arrhythmias...
October 25, 2016: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27660579/catheter-ablation-to-treat-supraventricular-arrhythmia-in-children-and-adults-with-congenital-heart-disease-what-we-know-and-where-we-are-going
#12
REVIEW
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
https://www.readbyqxmd.com/read/27638585/clinical-electrophysiological-and-biochemical-markers-of-peripheral-and-central-nervous-system-disease-in-canine-globoid-cell-leukodystrophy-krabbe-s%C3%A2-disease
#13
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
https://www.readbyqxmd.com/read/27615598/electrophysiologic-features-of-fibular-neuropathy-in-childhood-and-adolescence
#14
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
https://www.readbyqxmd.com/read/27579988/electrically-evoked-auditory-event-related-responses-in-patients-with-auditory-brainstem-implants-morphological-characteristics-test-retest-reliability-effects-of-stimulation-level-and-association-with-auditory-detection
#15
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)...
November 2016: Ear and Hearing
https://www.readbyqxmd.com/read/27554253/variability-of-myocardial-repolarization-in-pediatric-patients-with-a-ventricular-septal-defect
#16
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...
December 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27518079/the-role-of-mr-imaging-in-investigating-isolated-pediatric-nystagmus
#17
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
https://www.readbyqxmd.com/read/27513419/transesophageal-echocardiography-experience-in-thepediatric-age-group-in-a-tertiary-cardiac-center
#18
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
https://www.readbyqxmd.com/read/27503213/internal-cardioverter-defibrillator-indications-and-therapies-after-atrial-baffle-procedure-for-d-transposition-of-the-great-arteries-a-multicenter-analysis
#19
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
https://www.readbyqxmd.com/read/27489404/the-role-of-the-world-health-organization-s-international-classification-of-functioning-health-and-disability-in-models-of-infant-cochlear-implant-management
#20
REVIEW
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
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