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Child neurology

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https://www.readbyqxmd.com/read/28934891/quality-of-life-among-boys-with-adrenoleukodystrophy-following-hematopoietic-stem-cell-transplant
#1
Nicole B Beckmann, Weston P Miller, Mary S Dietrich, Paul J Orchard
Hematopoietic stem cell transplant (HSCT) is the only accepted treatment capable of halting the progression of X-linked cerebral adrenoleukodystrophy (CALD). While survival and neurological outcomes have been described, there is little information regarding the quality of life (QoL) of transplanted patients with CALD. This analysis is a cross-sectional study of QoL in 16 males diagnosed with CALD who underwent HSCT at a single institution. Each child or parent proxy completed subscales from the Neuro-QoL and the PROMIS Pediatric Profile Instrument representing physical, mental, and social health domains...
September 21, 2017: Child Neuropsychology: a Journal on Normal and Abnormal Development in Childhood and Adolescence
https://www.readbyqxmd.com/read/28933983/addition-of-kinesio-taping-of-the-orbicularis-oris-muscles-to-speech-therapy-rapidly-improves-drooling-in-children-with-neurological-disorders
#2
Denise Lica Yoshimura Mikami, Cristina Lemos Barbosa Furia, Alexis Fonseca Welker
OBJECTIVE: To evaluate the effects of Kinesio Taping (KT) of the orbicularis oris muscles as an adjunct to standard therapy for drooling. METHODS: Fifteen children with neurological disorders and drooling received speech therapy and twice-weekly KT of the orbicularis muscles over a 30-day period. Drooling was assessed by six parameters: impact on the life of the child and caregiver; severity of drooling; frequency of drooling; drooling volume (estimated by number of bibs used); salivary leak; and interlabial gap...
September 21, 2017: Developmental Neurorehabilitation
https://www.readbyqxmd.com/read/28932983/prevention-and-management-of-vascular-complications-in-middle-ear-and-cochlear-implant-surgery
#3
Filippo Di Lella, Maurizio Falcioni, Silvia Piccinini, Ilaria Iaccarino, Andrea Bacciu, Enrico Pasanisi, Davide Cerasti, Vincenzo Vincenti
The objective of this study is to illustrate prevention strategies and management of vascular complications from the jugular bulb (JB) and internal carotid artery (ICA) during middle ear surgery or cochlear implantation. The study design is retrospective case series. The setting is tertiary referral university hospital. Patients were included if presented pre- or intraoperative evidence of high-risk anatomical anomalies of ICA or JB during middle ear or cochlear implant surgery, intraoperative vascular injury, or revision surgery after the previous iatrogenic vascular lesions...
September 20, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28932485/incontinentia-pigmenti-in-a-child-with-suspected-retinoblastoma
#4
Stephanie J Weiss, Archana Srinivasan, Michael A Klufas, Carol L Shields
BACKGROUND: Incontinentia pigmenti is a rare X-linked dominant syndrome caused by mutation in the NEMO/IKKgamma gene, and characterized by a spectrum of cutaneous, ocular, neurologic and dental abnormalities. In the eye, findings include retinal vascular non-perfusion, occasionally with traction retinal detachment, retinal fibrosis, and retinal pigment epithelium defects. These findings can resemble retinoblastoma, especially when vitreoretinal fibrosis produces leukocoria. CASE REPORT: A 2-month-old girl born full-term presented with leukocoria, suspicious for retinoblastoma...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/28929415/pediatric-neurocysticercosis
#5
REVIEW
Pratibha Singhi, Arushi Gahlot Saini
Neurocysticercosis is the most common helminth infection of nervous system in humans caused by the encysted larvae of Taenia solium. It is a major cause of epilepsy in tropical areas and the most common cause of focal-onset seizures in North Indian children. Children with neurocysticercosis have pleomorphic manifestations depending on the location, number and viability of the cysts and host response. In endemic areas, neurocysticercosis should be clinically suspected in any child with recent-onset seizures, headache or focal motor deficits where there is no other suggestion of an underlying neurological disorder...
September 20, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28928544/role-of-anesthesiologist-in-the-management-of-a-child-with-cerebral-palsy
#6
REVIEW
Safiya Imtiaz Shaikh, Ganapati Hegade
Cerebral palsy (CP) refers to a spectrum of nonprogressive neurological disorders with disturbances in posture and movement, resulting from perinatal intrauterine insult to developing infant brain. Many conditions associated with CP require surgery. Such cases pose important gastrointestinal, respiratory, and other perioperative considerations. Anesthetic management in these cases is delicate. Intraoperative complications including hypovolemia, hypothermia, muscle spasms, seizures, and delayed recovery might complicate the anesthetic management...
July 2017: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/28927775/-pasteurella-multocida-meningoencephalitis-in-a-14-year-old-child
#7
L Kuoch, F Villedieu, G Favaretto, J Toulouse, L Mignot, M Jokic
Pasteurella multocida is generally responsible for local infections through animal bites. It can be a cause of meningitis, which tends to affect people at the extreme ages of life or suffering from immunodeficiency. A 14-year-old teenager was hospitalized with typical signs of bacterial meningitis. P. multocida was evidenced in the cerebrospinal fluid. Progression was marked by a degradation on the 4th day of treatment, despite intravenous antibiotic therapy with third-generation cephalosporin, followed by a single seizure on the 7th day of treatment...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28922945/aav-gene-therapy-in-a-sheep-model-of-tay-sachs-disease
#8
Heather Gray-Edwards, Ashley N Randle, Stacy Maitland, Hector Benatti, Spencer Hubbard, Peter Canning, Matthew Vogel, Brandon Brunson, Misako Hwang, Lauren Ellis, Allison M Bradbury, Atoska Gentry, Amanda Taylor, Anne Wooldridge, Dewey Wilhite, Randoplh Winter, Brain Whitlock, Jacob A Johnson, Merrilee Holland, Nouha Salibi, Ronald Beyers, James Sartin, Thomas Denney, Nancy R Cox, Miguel Sena-Esteves, Douglas R Martin
Tay-Sachs disease (TSD) is a fatal neurodegenerative disorder caused by a deficiency of the enzyme hexosaminidase A (HexA). Tay-Sachs disease also occurs in sheep, the only experimental model of TSD that has clinical signs of disease. The natural history of sheep TSD was characterized using serial neurological evaluations, 7 tesla MRI, echocardiograms, electrodiagnostics and cerebrospinal fluid (CSF) biomarkers. Intracranial gene therapy was also tested using AAVrh8 monocistronic vectors encoding the α subunit of Hex (TSD α) or a mixture of two vectors encoding both the α and β subunits separately (TSD α+β) injected at high (1...
September 19, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28921731/inter-observer-agreement-in-pediatric-cervical-spine-injury-assessment-between-prehospital-and-emergency-department-providers
#9
Lorin R Browne, Hamilton Schwartz, Fahd A Ahmad, Michael Wallendorf, Nathan Kuppermann, E Brooke Lerner, Julie C Leonard
BACKGROUND: Investigators have derived cervical spine injury (CSI) decision support tools from physician observations. There is a need to demonstrate that prehospital emergency medical services (EMS) providers can use these tools to appropriately determine the need for spinal motion restrictions and make field disposition decisions. OBJECTIVE: To determine the inter-observer agreement between EMS and emergency department (ED) providers for CSI risk assessment variables and overall gestalt for CSI in children after blunt trauma...
September 18, 2017: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
https://www.readbyqxmd.com/read/28914199/clinical-applications-of-intravenous-immunoglobulins-in-child-neurology
#10
Maria Gogou, Efimia Papadopoulou-Alataki, Martha Spilioti, Sofia Alataki, Athanasios Evangeliou
BACKGROUND: While there are guidelines for the use of intravenous immunoglobulins in children with Guillain-Barre syndrome and myasthenia gravis based on high-level evidence studies, data is fewer for the majority of neurologic disorders in this age group. Neuronal antibodies are detected in children with seizures of autoimmune etiology. Intravenous immunoglobulins with their broad immunomodulatory mechanism of action could be ideally effective in different forms of immune-dysregulated intractable epilepsies such as autoimmune epilepsy and autoimmune Rasmussen encephalitis...
September 15, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28910997/paediatric-nuclear-medicine-imaging
#11
Lorenzo Biassoni, Marina Easty
Background: Nuclear medicine imaging explores tissue viability and function by using radiotracers that are taken up at cellular level with different mechanism. This imaging technique can also be used to assess blood flow and transit through tubular organs. Nuclear medicine imaging has been used in paediatrics for decades and this field is continuously evolving. Sources of data: The data presented comes from clinical experience and some milestone papers on the subject...
September 1, 2017: British Medical Bulletin
https://www.readbyqxmd.com/read/28904469/hyper-acute-demyelinating-encephalomyelitis-of-childhood-a-rare-entity
#12
Suman Kushwaha, Ashutosh Gupta, Neha Agarwal, Sujata Chaturvedi, Deepak Jha
A young child with catastrophic neurological illness diagnosed as a rare variant of acute demyelinating encephalomyelitis (ADEM). She succumbed to her illness despite of aggressive and appropriate management. Malignant demyelinating encephalomyelitis should be considered in children who are refractory to the treatment of ADEM.
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28901887/microcytosis-is-associated-with-low-cognitive-outcomes-in-healthy-2-year-olds-in-a-high-resource-setting
#13
Elaine K McCarthy, Mairead E Kiely, Geraldine Hannon, Caroline Ahearne, Louise C Kenny, Jonathan O'B Hourihane, Alan D Irvine, Deirdre M Murray
Fe deficiency in early childhood is associated with long-term consequences for cognitive, motor and behavioural development; however explorations in healthy children from low risk, high-resource settings have been limited. We aimed to explore associations between Fe status and neurodevelopmental outcomes in low risk, healthy 2-year-olds. This study was a secondary analysis of a nested case-control subgroup from the prospective, maternal-infant Cork Babies after Screening for Pregnancy Endpoints: Evaluating the Longitudinal Impact using Neurological and Nutritional Endpoints (BASELINE) Birth Cohort Study...
September 2017: British Journal of Nutrition
https://www.readbyqxmd.com/read/28901029/successful-venoarterial-extracorporeal-membrane-oxygenation-for-postoperative-septic-shock-in-a-child-with-liver-transplantation-a-case-report
#14
Michiko Abe, Kentaro Ide, Nao Nishimura, Satoshi Nakagawa, Akinari Fukuda, Seisuke Sakamoto, Mureo Kasahara
Refractory septic shock after LT is a life-threatening complication. VA ECMO is used to treat refractory cardiorespiratory failure. We present herein the case of a 5-year-old girl with post-Kasai biliary atresia, who underwent a living donor LT and suffered refractory septic shock. VA ECMO was indicated due to progressive cardiac deterioration. After full recovery of her EF, she has been steadily improving and has shown good liver function and no neurological sequelae. This is the first report of successful VA ECMO in a post- LT patient with refractory septic shock...
September 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28888092/neurodevelopmental-outcome-at-the-age-of-4-years-according-to-the-planned-mode-of-delivery-in-term-breech-presentation-a-nationwide-population-based-record-linkage-study
#15
Georg Macharey, Mervi Väisänen-Tommiska, Mika Gissler, Veli-Matti Ulander, Leena Rahkonen, Mika Nuutila, Seppo Heinonen
PURPOSE: To evaluate whether a trial of planned vaginal breech labor affects neurologic development in children. METHODS: This is a nationwide, Finnish, population-based record linkage study. An odds ratio with 95% confidence intervals was used to estimate the relative risk that a child delivered by planned vaginal breech labor would be diagnosed with adverse neurodevelopmental outcome (cerebral palsy, epilepsy, intellectual disability, sensor neural developmental outcome, hyperactivity, speech and language problems) at the age of 4 years...
September 9, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28871588/primary-pleuropulmonary-synovial-sarcoma-with-brain-metastases-in-a-paediatric-patient-an-unusual-presentation
#16
Pushpak Chandrakant Chirmade, Sonia Parikh, Asha Anand, Harsha Panchal, Apurva Patel, Sandip Shah
Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously...
2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/28870494/health-related-quality-of-life-outcomes-of-a-telehealth-care-coordination-intervention-for-children-with-medical-complexity-a-randomized-controlled-trial
#17
Wendy S Looman, Robyn L Hullsiek, Lyndsay Pryor, Michelle A Mathiason, Stanley M Finkelstein
The purpose of this study was to explore health-related quality of life (HRQL) and family impact in the context of an advanced practice registered nurse-delivered telehealth care coordination intervention for children with medical complexity (CMC). This was a secondary outcomes analysis of a randomized controlled trial with 163 families of CMC in an existing medical home. HRQL and family impact were measured using the PedsQL measurement model. Bivariate and analysis of covariance analyses were conducted to explore associations at baseline and the intervention effect over 2 years...
September 1, 2017: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/28870493/incontinentia-pigmenti-a%C3%A2-summary-review-of-this-rare-ectodermal-dysplasia-with-neurologic-manifestations-including-treatment-protocols
#18
Carol Greene-Roethke
Incontinentia pigmenti is a rare neuroectodermal dysplasia caused by a defect in the IKBKG gene (formerly known as NEMO). There are 27.6 new cases per year worldwide; 65% to 75% are sporadic mutations, and 25% to 35% are familial. It is usually lethal in males, but females survive because of X-inactivation mosaicism. The disorder is typically identified by unique skin findings, a series of four stages that emerge throughout the first year of life. The central nervous system manifestations in the eye and in the brain cause the most disability...
September 1, 2017: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/28868307/atlanto-axial-langerhans-cell-histiocytosis-in-a-child-presented-as-torticollis
#19
Miniar Tfifha, Mehdi Gaha, Nadia Mama, Mohamed Taher Yacoubi, Saoussen Abroug, Hela Jemni
Langerhans cell histiocytosis (LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma (EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery...
August 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28863007/intraoperative-neurophysiologic-monitoring-for-prediction-of-postoperative-neurological-improvement-in-a-child-with-chiari-type-i-malformation
#20
Yukari Kawasaki, Susumu Uchida, Kouhei Onishi, Masako Toyokuni, Kazuo Okanari, Minoru Fujiki
INTRODUCTION: Although many surgical treatment strategies for Chiari malformation type I (CM-I) have been reported, the most appropriate surgical technique remains controversial. It is wholly ascribable to the complicacy of pathological condition in CM-I. Recently, intraoperative neurophysiologic monitoring (INM) is becoming prevalent in spinal surgery. Indeed, motor-evoked potentials (MEPs) monitoring and somatosensory-evoked potentials (SSEPs) monitoring are standard tools to minimize the risk of neurologic injury and postoperative deficits...
August 31, 2017: Journal of Craniofacial Surgery
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