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Pediatric neuromuscular disease

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https://www.readbyqxmd.com/read/28780390/a-12-year-experience-with-tracheostomy-for-neonates-and-infants-in-northern-taiwan-indications-hospital-courses-and-long-term-outcomes
#1
Chia-Huei Chen, Jui-Hsing Chang, Chyong-Hsin Hsu, Nan-Chang Chiu, Chun-Chin Peng, Wai-Tim Jim, Hung-Yang Chang, Kuo-Sheng Lee
BACKGROUND: Tracheostomy is a valuable procedure in infants and neonates with chronic respiratory failure or severe airway obstruction. The aim of this study is to identify the indication, hospital course, and long-term outcome in a cohort of infants who required tracheostomy in a neonatal and pediatric tertiary care center in northern Taiwan. METHODS: Medical records of infants, who underwent tracheostomy between January 2002 and December 2013, were retrospectively reviewed...
July 24, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28738322/pediatric-obstructive-sleep-apnea-where-do-we-stand
#2
Yu-Shu Huang, Christian Guilleminault
Pediatric obstructive sleep apnea (OSA) was initially described in 1976. In 1981, Dr. Guilleminault emphasized that pediatric OSA was different from the clinical presentation reported in adults. It was characterized by more disturbed nocturnal sleep than excessive daytime sleepiness, and presented more behavioral problems, particularly school problems, hyperactivity, nocturnal enuresis, sleep terrors, depression, insomnia, and psychiatric problems. The underlying causes of pediatric OSA are complex. Such factors as adenotonsillar hypertrophy, obesity, anatomical and neuromuscular factors, and hypotonic neuromuscular disease are also involved...
2017: Advances in Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28713509/mechanical-ventilation-practice-in-egyptian-pediatric-intensive-care-units
#3
Bassant Salah Meligy, Sally Kamal, Seham Awad El Sherbini
INTRODUCTION: Mechanical ventilation is one of the indispensable tools in pediatric intensive care units. Few studies addressed the epidemiology of pediatric patients on mechanical ventilation and the frequently used modes of ventilation. This is the first study to describe the practice of mechanical ventilation (MV) in Egyptian pediatric intensive care units (PICUs). METHODS: This prospective observational study was conducted from January 2014 to December 2014 in two pediatric intensive care units at Cairo University Pediatric Hospital...
May 2017: Electronic Physician
https://www.readbyqxmd.com/read/28691974/peroral-endoscopic-myotomy-in-children-with-achalasia-a-relatively-long-term-single-center-study
#4
Miao Shijian, Wu Jie, Lu Junping, Wang Yuhuan, Tang Zifei, Zhou Ying, Huang Zhiheng, Ying Huang, Zhou Pinghong
BACKGROUND: Achalasia is a disease caused by neuromuscular dysfunction in the esophagogastric junction, with a relatively low incidence among children. Peroral endoscopic myotomy (POEM) is a new endoscopic procedure for the treatment of achalasia, however it is rarely applied in children. OBJECTIVES: We aim to study the feasibility, safety, and effectiveness of POEM as a treatment for pediatric achalasia. METHODS: A total of 21 pediatric patients (aged from 11 months to 18 years) diagnosed with achalasia and treated with POEM from October of 2014 to October of 2016 in our hospital were included in our study...
July 6, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28668199/chronic-respiratory-failure-utilization-of-a-pediatric-specialty-integrated-care-program
#5
Robert J Graham, Michael L McManus, Angie Mae Rodday, Ruth Ann Weidner, Susan K Parsons
OBJECTIVE: Describe utilization and satisfaction in a specialty integrated care program for children with severe, chronic respiratory insufficiency (CRI). SUBJECTS: Enrollees of the Critical Care, Anesthesia, Perioperative Extension (CAPE) and Home Ventilation Program. METHODS: Children with CRI received home visits, care coordination, and "on-demand" 24/7 access to physicians. Program activity and outcomes were recorded for 3 years using an adapted Care Coordination Measurement Tool© version...
March 2017: Healthcare
https://www.readbyqxmd.com/read/28662944/skeletal-muscle-channelopathies-rare-disorders-with-common-pediatric-symptoms
#6
Emma Matthews, Arpana Silwal, Richa Sud, Michael G Hanna, Adnan Y Manzur, Francesco Muntoni, Pinki Munot
OBJECTIVE: To ascertain the presenting symptoms of children with skeletal muscle channelopathies to promote early diagnosis and treatment. STUDY DESIGN: Retrospective case review of 38 children with a skeletal muscle channelopathy attending the specialist pediatric neuromuscular service at Great Ormond Street Hospital over a 15-year period. RESULTS: Gait disorder and leg cramps are a frequent presentation of myotonic disorders (19 of 29). Strabismus or extraocular myotonia (9 of 19) and respiratory and/or bulbar symptoms (11 of 19) are common among those with sodium channelopathy...
June 26, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28633151/age-based-normative-measurements-of-the-pediatric-pelvis
#7
Matthew E Oetgen, Steven Andelman, Benjamin D Martin
OBJECTIVES: Pediatric pelvic trauma is associated with high rates of morbidity and mortality. Current classifications do not take into account age-dependent quantitative differences of the pediatric pelvis making accurate diagnosis of pathologic diastasis of the pubic symphysis, sacroiliac (SI) joints, and triradiate cartilage difficult. The purpose of this study was to establish age-based normative values for the width of the pubic symphysis, SI joints, and the triradiate cartilage of the pediatric pelvis...
July 2017: Journal of Orthopaedic Trauma
https://www.readbyqxmd.com/read/28632945/developing-multidisciplinary-clinics-for-neuromuscular-care-and-research
#8
REVIEW
Sabrina Paganoni, Katie Nicholson, Fawn Leigh, Kathryn Swoboda, David Chad, Kristin Drake, Kellen Haley, Merit Cudkowicz, James D Berry
Multidisciplinary care is considered the standard-of-care for both adult and pediatric neuromuscular disorders and has been associated with improved quality of life, resource utilization, and health outcomes. Multidisciplinary care is delivered in multidisciplinary clinics that coordinate care across multiple specialties reducing travel burden and streamlining care. In addition, multidisciplinary care setting facilitates the integration of clinical research, patient advocacy, and care innovation (e.g., TeleHealth)...
June 20, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28546380/respiratory-implications-of-pediatric-neuromuscular-disease
#9
Howard B Panitch
Children with progressive neuromuscular weakness undergo a stereotypical progression of respiratory involvement, beginning with impaired airway clearance and progressing to nocturnal and then diurnal ventilatory failure. This review examines issues related to airway clearance and mucus mobilization, sleep problems, and use of assisted ventilation in children with neuromuscular diseases. Interventions for each of these problems have been created or adapted for the pediatric population. The use of airway clearance therapies and assisted ventilation have improved survival of children with neuromuscular weakness...
June 2017: Respiratory Care
https://www.readbyqxmd.com/read/28546128/predictive-factors-of-intraoperative-cell-salvage-during-pediatric-scoliosis-surgery-cell-saver-during-scoliosis-surgery-in-children
#10
Daphné Michelet, Florence Julien-Marsollier, Julie Hilly, Thierno Diallo, Christophe Vidal, Souhayl Dahmani
INTRODUCTION: blood saving strategy during spinal surgery in children often includes recombinant erythropoietin (rEPO) and antifibrinolytic therapapy (AFT). The aim of this study was to investigate the efficacy of intraoperative blood salvage in decreasing homologous blood transfusion. MATERIAL AND METHODS: using the prospective data from patients operated during a one year period for scoliosis correction, we calculate the predictable hematocrit at day postoperative 1 without the use of blood salvage and compare it to the target hematocrit transfusion according to patient's status...
May 22, 2017: Anaesthesia, Critical Care & Pain Medicine
https://www.readbyqxmd.com/read/28440921/pediatric-pulmonology-year-in-review-2016-part-1
#11
REVIEW
David J Birnkrant, Jane B Black, Ignacio E Tapia, Thomas Nicolai, William A Gower, Terry L Noah
Pediatric Pulmonology continues to publish research and clinical topics related to the entire range of children's respiratory disorders. As we have done annually in recent years, we here summarize the past year's publications in our major topic areas, as well as selected literature in these areas from other core journals relevant to our discipline. This review (Part 1) covers selected articles on sleep, diagnostic testing/endoscopy, respiratory complications of neuromuscular disorders, and rare lung diseases...
September 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28437378/risk-factors-for-pediatric-extubation-failure-the-importance-of-respiratory-muscle-strength
#12
Robinder G Khemani, Tro Sekayan, Justin Hotz, Rutger C Flink, Gerrard F Rafferty, Narayan Iyer, Christopher J L Newth
OBJECTIVE: Respiratory muscle weakness frequently develops during mechanical ventilation, although in children there are limited data about its prevalence and whether it is associated with extubation outcomes. We sought to identify risk factors for pediatric extubation failure, with specific attention to respiratory muscle strength. DESIGN: Secondary analysis of prospectively collected data. SETTING: Tertiary care PICU. PATIENTS: Four hundred nine mechanically ventilated children...
August 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28346272/influenza-in-children
#13
Janienne Kondrich, Michele Rosenthal
PURPOSE OF REVIEW: We review the current information and evidence available on the global burden of disease in the pediatric population, clinical presentation and complications, testing, treatment, and immunization. RECENT FINDINGS: In addition to multiple other risk factors for influenza complications, children with neurologic and neuromuscular disorders are significantly higher risk for serious complications. In practice, there is no lower age limit for children with influenza who can be treated with oseltamivir...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28338364/indications-risk-of-lower-airway-infection-and-complications-to-pediatric-tracheotomy-report-from-a-tertiary-referral-center
#14
Christian Grønhøj, Birgitte Charabi, Christian von Buchwald, Thomas Hjuler
BACKGROUND: Although pediatric tracheotomy is potentially life-saving, the procedure is associated with high risk of complications, and indications have changed the last decade. We report indications, complications, and lower airway infections (LAIs) to pediatric tracheotomy performed at a tertiary referral center. METHODS: We identified all children (<18 years) who underwent tracheotomy at our institution during 2008-2015. A review of hospital records was performed to extract data on indication of the procedure, complications, and information on pre- and postoperative LAI...
March 24, 2017: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/28325641/spectrum-of-nondystrophic-skeletal-muscle-channelopathies-in-children
#15
Fouad Al-Ghamdi, Basil T Darras, Partha S Ghosh
BACKGROUND: The nondystrophic skeletal muscle channelopathies are a group of disorders caused by mutations of various voltage-gated ion channel genes, including nondystrophic myotonia and periodic paralysis. METHODS: We identified patients with a diagnosis of muscle channelopathy from our neuromuscular database in a tertiary care pediatric center from 2005 to 2015. We then performed a retrospective review of their medical records for demographic characteristics, clinical features, investigations, treatment, and follow-up...
May 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28175989/predictors-of-health-related-quality-of-life-in-boys-with-duchenne-muscular-dystrophy-from-six-european-countries
#16
MULTICENTER STUDY
Christiane Otto, Birgit F Steffensen, Ann-Lisbeth Højberg, Claus Barkmann, Jes Rahbek, Ulrike Ravens-Sieberer, Annette Mahoney, Julia Vry, Kathrin Gramsch, Rachel Thompson, Sunil Rodger, Kate Bushby, Hanns Lochmüller, Janbernd Kirschner
Duchenne muscular dystrophy (DMD) is a progressive, genetically determined neuromuscular disease that affects males and leads to severe physical disability in early teenage years. Over the last decades, patient-reported outcomes such as Health-Related Quality of Life (HRQoL) gained great interest in clinical research. However, little is known about factors affecting HRQoL in boys with DMD. Data from the multi-center CARE-NMD project of boys with DMD from six European countries collected between 2011 and 2012 were analyzed (8-17 years old; n = 321)...
April 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28123622/full-outline-of-unresponsiveness-score-and-the-glasgow-coma-scale-in-prediction-of-pediatric-coma
#17
Atahar Jamal, Naveen Sankhyan, Murlidharan Jayashree, Sunit Singhi, Pratibha Singhi
BACKGROUND: This study was done to compare the admission Full Outline of Unresponsiveness (FOUR) score and Glasgow Coma Scale (GCS) as predictors of outcome in children with impaired consciousness. METHODS: In this observational study, children (5-12 years) with impaired consciousness of <7 days were included. Children with traumatic brain injury, on sedatives or neuromuscular blockade; with pre-existing cerebral palsy, mental retardation, degenerative brain disease, vision/hearing impairment; and seizure within last 1 hour were excluded...
2017: World Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28109487/utility-of-polysomnography-in-determination-of-laryngomalacia-severity
#18
Jacqueline E Weinstein, Claire M Lawlor, Eric L Wu, Kimsey H Rodriguez
OBJECTIVE: To examine the efficacy of polysomnography in determining the severity of laryngomalacia in pediatric patients. METHODS: Prospective cohort study. Pediatric patients referred to our pediatric otolaryngology department with a polysomnogram already performed for a presumptive diagnosis of laryngomalacia were enrolled in the study. Patients with concurrent airway lesions or neuromuscular disorders were excluded. Patients underwent history, physical exam, and flexible fiberoptic laryngoscopy...
February 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28097856/-transition-experience-of-patients-with-neuromuscular-disease
#19
Valeria Greif, Florencia Ugo, M Fernanda de Castro Pérez, Julieta Mozzoni, Verónica Aguerre, Milagros Saldías, M Soledad Monges
Neuromuscular diseases are mostly genetic disorders, with chronic and progressive course. Affected people are at high risk of developing physical and emotional disabilities. In the last decades, the advance in technology and science has increased chronic pediatric patients survival rate, thus requiring an ongoing assistance in adult hospitals, making the transition a necessity and a challenge. This article reports the clinical practice designed between Hospital Garrahan and Hospital Ramos Mejía for the transition of 27 adolescents during 2015, setting achievements, findings and challenges resulting from this experience...
February 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27989332/neuropathic-and-myopathic-pain
#20
REVIEW
Anthony C Rodrigues, Peter B Kang
The evaluation and management of childhood pain syndromes of neuromuscular origin have distinct challenges, as the patterns of disease presentation and the ability of a child to describe symptoms may differ from that of an adult. Advances in scientific and clinical knowledge are leading to significant progress in the care of affected children. The genetic origins of Fabry disease and the inherited form of erythromelalgia are better understood. The increasing interest in neuroimmunology among pediatric neurologists has led to more sophisticated diagnostic and therapeutic approaches...
August 2016: Seminars in Pediatric Neurology
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