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Pediatrics neurology

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https://www.readbyqxmd.com/read/28329394/intermediate-follow-up-of-pediatric-patients-with-hemolytic-uremic-syndrome-during-the-2011-outbreak-caused-by-e-coli-o104-h4
#1
Sebastian Loos, Wiebke Aulbert, Bernd Hoppe, Thurid Ahlenstiel-Grunow, Birgitta Kranz, Charlotte Wahl, Hagen Staude, Alexander Humberg, Kerstin Benz, Martin Krause, Martin Pohl, Max C Liebau, Raphael Schild, Johanna Lemke, Ortraud Beringer, Dominik Müller, Christoph Härtel, Marianne Wigger, Udo Vester, Martin Konrad, Dieter Haffner, Lars Pape, Jun Oh, Markus J Kemper
Background.: In 2011 Escherichia coli O104:H4 caused an outbreak with over 800 cases of hemolytic uremic syndrome (HUS) in Germany, including 90 children. Data on the intermediate outcome in children after HUS due to E. coli O104:H4 have been lacking. Methods.: Follow-up data were gathered retrospectively from the medical records of patients who had been included in the German Pediatric HUS Registry during the 2011 outbreak. Results.: A total of 72/89 (81%) of the patients was included after a median follow-up of 3...
March 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28328788/early-presence-of-sleep-spindles-on-electroencephalography-is-associated-with-good-outcome-after-pediatric-cardiac-arrest
#2
Laurence Ducharme-Crevier, Craig A Press, Jonathan E Kurz, Michele G Mills, Joshua L Goldstein, Mark S Wainwright
OBJECTIVES: The role of sleep architecture as a biomarker for prognostication after resuscitation from cardiac arrest in children hospitalized in an ICU remains poorly defined. We sought to investigate the association between features of normal sleep architecture in children after cardiac arrest and a favorable neurologic outcome at 6 months. DESIGN: Retrospective review of medical records and continuous electroencephalography monitoring. SETTING: Cardiac and PICU of a tertiary children's hospital...
March 21, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28327376/maternal-m%C3%A3-llerian-anomalies-and-future-health-of-the-offspring
#3
Adva Cahen-Peretz, Asnat Walfisch, Michael Friger, Eyal Sheiner
OBJECTIVE: To evaluate whether offspring of women with müllerian anomalies are at an increased risk for long-term pediatric morbidity. STUDY DESIGN: A population-based cohort study compared the incidence of long-term (up to the age of 18 years for offspring) hospitalizations due to cardiovascular, endocrine, neurological, hematological, respiratory and urinary morbidity of offspring to mothers diagnosed with uterine anomalies. Deliveries occurred between the years 1991 and 2013 in a tertiary medical center...
March 3, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28326208/a-child-as-a-donor-for-hematopoietic-stem-cell-transplantation-bioethical-justification-a-case-study-on-sickle-cell-disease
#4
Andrea Z Pereira, Ricardo Hellman, Nelson Hamerschlak, Andrea Kondo, Polianna Mara Rodrigues de Souza, Wilson Leite Pedreira, Luiz Fernando Alves Lima Mantovani, Eduardo Juan Troster, Henrique Grunspun, Marco Aurélio Scarpinella Bueno
Hematopoietic stem cell transplantation (HSCT) is an important treatment option for children with severe and refractory sickle cell disease (SCD) with debilitating clinical complications. HSCT with cells from the bone marrow of a HLA-identical sibling used in SCD has a low mortality risk, high cure rate, and high event-free survival rate after a median follow-up of 5-6 years. However, matched donors are found in only about 20% of the patients. A boy aged 8 years with SCD had a sister, <2 years old, a fully compatible donor...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28324682/gene-expression-analysis-in-untreated-absence-epilepsy-demonstrates-an-inconsistent-pattern
#5
Markus von Deimling, Robert Häsler, Verena Steinbach, Paul-Martin Holterhus, Sarah von Spiczak, Ulrich Stephani, Ingo Helbig, Hiltrud Muhle
OBJECTIVE: Childhood and Juvenile Absence Epilepsy account for 30% of all genetic generalized epilepsies with a strong genetic contribution. At the current state the genetic background remains to be resolved. The aim of this study was to identify disease associated transcripts pinpointing potential underlying disease mechanisms in patients with CAE and JAE. METHODS: We performed gene expression analysis from peripheral blood mononuclear cells (PBMCs) in 30 patients with newly-diagnosed absence epilepsy prior to initiating treatment and 30 healthy age - and gender-matched pediatric controls...
February 28, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28322606/cognitive-impairment-in-pediatric-onset-multiple-sclerosis-is-detected-by-the-brief-international-cognitive-assessment-for-multiple-sclerosis-and-computerized-cognitive-testing
#6
Leigh E Charvet, Michael Shaw, Ariana Frontario, Dawn Langdon, Lauren B Krupp
BACKGROUND: Cognitive impairment is a common and troubling feature of pediatric-onset multiple sclerosis (POMS). Brief cognitive assessment in the outpatient setting can identify and longitudinally monitor cognitive involvement so that early intervention is possible. OBJECTIVES: The goal of this study was to measure the sensitivity of two cognitive assessment approaches that are brief, repeatable, and suitable for clinical practice and for multicenter investigation...
March 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28322177/prevalence-estimate-of-cerebral-palsy-in-northern-alberta-births-2008-2010
#7
Charlene M T Robertson, M Florencia Ricci, Kathleen O'Grady, Maryam Oskoui, Helly Goez, Jerome Y Yager, John C Andersen
OBJECTIVES: The objectives of this study were to determine prevalence estimates of cerebral palsy (CP) among 5-year-old children in northern Alberta; to provide congenital, gestational age- and birth weight-specific, and postneonatal CP rates; and to describe motor subtypes and function. METHODS: This population-based prevalence estimate study, part of the Canadian Cerebral Palsy Registry, reports confirmed CP diagnoses at age 5 years made by pediatric rehabilitation and child neurology specialists...
March 21, 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/28322114/predictive-value-of-gjb2-mutation-status-for-hearing-outcomes-of-pediatric-cochlear-implantation
#8
Yasin Abdurehim, Alexandre Lehmann, Anthony G Zeitouni
Objective To systematically review and quantify current evidence regarding the association of GJB2 mutation status with outcomes of pediatric cochlear implantation. Data Sources PubMed, Embase, and the Cochrane Library were searched for "GJB2,"pediatric hearing loss," and "cochlear implantation" and their synonyms, with no language restrictions, until December 2, 2015. Review Methods Studies were included that investigated the status of GJB2 mutation and its predictive value for outcomes of pediatric cochlear implantation...
March 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28321388/association-of-atlanto-occipital-dislocation-retroclival-hematoma-and-hydrocephalus-management-and-survival-in-a-pediatric-patient
#9
Isaac L Lee, Luis F Vasquez, Alan H Tyroch, Todd T Trier
Atlanto-occipital dislocation (AOD) is an injury with high morbidity and mortality. We present a case of survival of a pediatric patient with the diagnoses of AOD, retroclival hematoma, and resulting hydrocephalus. The patient's cervical spine was stabilized until occipital-cervical fusion provided definitive treatment, and the hydrocephalus was treated with a ventriculostomy. The patient survived with no neurological deficits. A better understanding and awareness of the radiologic criteria of AOD will lead to earlier recognition of AOD and improved outcomes, even in the presence of complications from AOD...
January 2017: Journal of Neurological Surgery Reports
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/28318500/mutations-in-tmem260-cause-a-pediatric-neurodevelopmental-cardiac-and-renal-syndrome
#11
Asaf Ta-Shma, Tahir N Khan, Asaf Vivante, Jason R Willer, Pavle Matak, Chaim Jalas, Ben Pode-Shakked, Yishay Salem, Yair Anikster, Friedhelm Hildebrandt, Nicholas Katsanis, Orly Elpeleg, Erica E Davis
Despite the accelerated discovery of genes associated with syndromic traits, the majority of families affected by such conditions remain undiagnosed. Here, we employed whole-exome sequencing in two unrelated consanguineous kindreds with central nervous system (CNS), cardiac, renal, and digit abnormalities. We identified homozygous truncating mutations in TMEM260, a locus predicted to encode numerous splice isoforms. Systematic expression analyses across tissues and developmental stages validated two such isoforms, which differ in the utilization of an internal exon...
March 11, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28316855/central-nervous-system-involvement-in-henoch-schonlein-purpura-in-children-and-adolescents
#12
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/28306631/thermoregulate-autoregulate-and-ventilate-brain-directed-critical-care-for-pediatric-cardiac-arrest
#13
Jonathan E Kurz, Craig M Smith, Mark S Wainwright
PURPOSE OF REVIEW: Cardiac arrest in childhood is associated with a high risk for mortality and poor long-term functional outcome. This review discusses the current evidence for neuroprotective therapies and goals for postarrest care in the context of the pathophysiology of hypoxic-ischemic injury, modalities for neurologic prognostication in these children and potential future monitoring paradigms for maximizing cerebral perfusion in the postarrest period. RECENT FINDINGS: The recent publication of the in-hospital and out-of-hospital Therapeutic Hypothermia After Cardiac Arrest trials demonstrated a lack of statistically significant benefit for the use of postarrest therapeutic hypothermia...
March 16, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28306628/pediatric-spinal-cord-injury-without-radiographic-abnormality-in-the-era-of-advanced-imaging
#14
Caitlin A Farrell, Megan Hannon, Lois K Lee
PURPOSE OF REVIEW: The current review describes the current evidence on pediatric spinal cord injury without radiographic abnormality (SCIWORA) with attention to the definition, epidemiology, and clinical presentation of the condition, as well as common MRI findings, management strategies, and outcomes. RECENT FINDINGS: Recent literature demonstrates that with more widespread MRI use, our understanding of SCIWORA has improved. The new literature, although still limited, provides a more granular conceptualization of patterns of injury as well as potential prognostic stratification of patients based on MRI findings...
March 16, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28304316/perspectives-and-attitudes-of-pediatricians-concerning-post-discharge-care-practice-of-premature-infants
#15
A Gad, E Parkinson, N Khawar, A Elmeki, P Narula, D Hoang
OBJECTIVE: Survival rates of premature infants are at a historical high and increasingly more pediatricians are caring for former premature infants. The goal of this study was to describe the perspectives and attitudes of pediatricians, as well as, the challenges of rendering post-neonatal intensive care unit (NICU) discharge care for premature infants. METHODS: An anonymous 22-question web-based survey was emailed to pediatricians who are current members of the American Academy of Pediatrics (AAP) and practicing in Kings County, New York...
March 16, 2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/28302372/experiences-during-newborn-screening-for-glutaric-aciduria-type-1-diagnosis-treatment-genotype-phenotype-and-outcomes
#16
Fang-Chih Tsai, Han-Jui Lee, An-Guor Wang, Shu-Chen Hsieh, Yung-Hsiu Lu, Ming-Che Lee, Ju-Shan Pai, Tzu-Hung Chu, Chia-Feng Yang, Ting-Rong Hsu, Chih-Jou Lai, Ming-Tzu Tsai, Ping-Hsun Ho, Min-Chieh Lin, Ling-Yee Cheng, Ya-Chin Chuang, Dau-Ming Niu
BACKGROUND: Glutaric aciduria type 1 (GA-1) is an organic acidemia with potentially severe neurological sequelae. In Taiwan, newborn screening (NBS) for GA-1 began in 2001, but large-scale reporting is lacking. This study describes Taiwan's largest newborn screening population to date. METHODS: Between 2001 and 2015, 1,490,636 newborns were screened for GA-1. Confirmatory examinations included the carnitine loading test. Confirmed patients were treated with a low lysine diet, carnitine, and high-energy intake during illness...
March 13, 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28301267/hypotonic-hyporesponsive-episodes-after-administration-of-hexavalent-dtp-based-combination-vaccine-a-description-of-12-cases
#17
Alessandro Vigo, Giulia Costagliola, Elisa Ferrero, Silvia Noce
Hypotonic-hyporesponsive (HHE) episodes are known and recognized phenomena, which typically occur within 48 hours of immunisation..Our aim is to describe 12 cases of HHE brought to the Center of Pediatric Sleep Medicine, with attention to the clinical features of the episode and their follow-up. Medical charts of infants visited between 2005-2015 were reviewed. Twelve infants showed a HHE using HHE using Brighton Collaboration Criteria. All infants received a Hexavalent diphtheria -tetanus -pertussis acellular component -hepatitis B-,inactivated poliovirus- Haemophilus influenzae type-b conjugate vaccine...
February 21, 2017: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/28295547/practitioner-perceptions-of-the-anesthetic-needs-for-magnetic-resonance-imaging-in-pediatric-patients
#18
Glenn E Mann, Adam Canter, Singh Nair, Donna LaMonica, Madelyn Kahana, Jennifer Yoo, Ellise Delphin
There is currently an increasing use of magnetic resonance imaging (MRI) as a diagnostic tool in the pediatric population(1). MRI is often viewed as an ideal diagnostic modality because it does not require exposure to ionizing radiation, provides superior soft tissue image quality compared with computed tomography (CT) and is thus well-suited for neurological, musculoskeletal, cardiac, thoracic, and abdominal studies(2). However, MRIs require patient cooperation and extended time periods of immobility, as the studies are often long, loud, and may induce claustrophobia (2-7)...
March 12, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28291425/long-term-outcomes-of-epilepsy-surgery-in-85-pediatric-patients-followed-up-for-over-10-years-a-retrospective-survey
#19
Hiroshi Hosoyama, Kazumi Matsuda, Tadahiro Mihara, Naotaka Usui, Koichi Baba, Yushi Inoue, Takayasu Tottori, Toshiaki Otsubo, Yumi Kashida, Koji Iida, Hirofumi Hirano, Ryosuke Hanaya, Kazunori Arita
OBJECTIVE The aim of this study was to investigate the treatment outcomes and social engagement of patients who had undergone pediatric epilepsy surgery more than 10 years earlier. METHODS Between 1983 and 2005, 110 patients younger than 16 years underwent epilepsy surgery at the National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders. The authors sent a questionnaire to 103 patients who had undergone follow-up for more than 10 years after surgery; 85 patients (82.5%) responded. The survey contained 4 categories: seizure outcome, use of antiepileptic drugs, social participation, and general satisfaction with the surgical treatment (resection of the epileptic focus, including 4 hemispherectomies)...
March 3, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28288328/case-control-exploratory-study-of-cerebrospinal-fluid-chemokines-cytokines-and-lymphocyte-subsets-in-childhood-tourette-syndrome-with-positive-streptococcal-markers
#20
Michael R Pranzatelli, Elizabeth D Tate, Tyler J Allison
A longstanding question is whether neuroinflammation is present in children symptomatic for Tourette syndrome (TS) with positive streptococcal serology and throat cultures. The objective was to directly test for it using modern hypothesis-driven approaches. Profiling studies for 14 immune cell types (flow cytometry), 7 chemokines/cytokines (ELISA), oligoclonal bands, and other immunoglobulins were performed in this IRB-approved study of 5 children with TS and streptococcal markers compared to data from 26 non-inflammatory pediatric neurological controls...
March 10, 2017: Cytokine
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