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Pediatric neuromuscular disease

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https://www.readbyqxmd.com/read/29049016/skeletal-muscle-ultrasonography-in-nutrition-and-functional-outcome-assessment-of-critically-ill-children-experience-and-insights-from-pediatric-disease-and-adult-critical-care-studies-formula-see-text
#1
Chengsi Ong, Jan Hau Lee, Melvin K S Leow, Zudin A Puthucheary
Evidence suggests that critically ill children develop muscle wasting, which could affect outcomes. Muscle ultrasound has been used to track muscle wasting and association with outcomes in critically ill adults but not children. This review aims to summarize methodological considerations of muscle ultrasound, structural findings, and possibilities for its application in the assessment of nutrition and functional outcomes in critically ill children. Medline, Embase, and CINAHL databases were searched up until April 2016...
September 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28947640/clinical-definition-of-respiratory-viral-infections-in-young-children-and-potential-bronchiolitis-misclassification
#2
Rosemary Megalaa, Geovanny F Perez, Sasikumar Kilaikode-Cheruveettara, Nidhi Kotwal, Carlos E Rodriguez-Martinez, Gustavo Nino
Viral respiratory infections are often grouped as a single respiratory syndrome named 'viral bronchiolitis', independently of the viral etiology or individual risk factors. Clinical trials and guidelines have used a more stringent definition of viral bronchiolitis, including only the first episode of wheezing in children less than 12 months of age without concomitant respiratory comorbidities. There is increasing evidence suggesting that this definition is not being followed by pediatric care providers, but it is unclear to what extent viral respiratory infections are currently misclassified as viral bronchiolitis using standard definitions...
September 24, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28889642/treating-pediatric-neuromuscular-disorders-the-future-is-now
#3
REVIEW
James J Dowling, Hernan D Gonorazky, Ronald D Cohn, Craig Campbell
Pediatric neuromuscular diseases encompass all disorders with onset in childhood and where the primary area of pathology is in the peripheral nervous system. These conditions are largely genetic in etiology, and only those with a genetic underpinning will be presented in this review. This includes disorders of the anterior horn cell (e.g., spinal muscular atrophy), peripheral nerve (e.g., Charcot-Marie-Tooth disease), the neuromuscular junction (e.g., congenital myasthenic syndrome), and the muscle (myopathies and muscular dystrophies)...
September 10, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28864264/a-self-reported-needs-assessment-survey-of-pediatric-orthopaedic-education-in-haiti
#4
Rameez A Qudsi, Heather J Roberts, Abhiram R Bhashyam, Elena Losina, Donald S Bae, Francel Alexis, George S Dyer
OBJECTIVE: The burden of musculoskeletal disease remains high in low-income countries, with a high rate of pediatric disease. Efforts continue for orthopedic education, but there is little guidance on local needs and desires. Our aim was to determine the specific content and modalities that would be most useful for pediatric orthopedic educational programs abroad, and we demonstrate a practical method of identifying country-specific educational deficits through a self-reported needs survey in Haiti...
August 29, 2017: Journal of Surgical Education
https://www.readbyqxmd.com/read/28838934/management-of-cardiac-involvement-associated-with-neuromuscular-diseases-a-scientific-statement-from-the-american-heart-association
#5
Brian Feingold, William T Mahle, Scott Auerbach, Paula Clemens, Andrea A Domenighetti, John L Jefferies, Daniel P Judge, Ashwin K Lal, Larry W Markham, W James Parks, Takeshi Tsuda, Paul J Wang, Shi-Joon Yoo
For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic...
September 26, 2017: Circulation
https://www.readbyqxmd.com/read/28830650/basics-of-bone-metabolism-and-osteoporosis-in-common-pediatric-neuromuscular-disabilities
#6
REVIEW
Evren Yaşar, Emre Adigüzel, Mutluay Arslan, Dennis J Matthews
Bone modeling is a process that starts with fetal life and continues during adolescence. Complex factors such as hormones, nutritional and environmental factors affect this process. In addition to these factors, physical conditioning and medications that have toxic effects on bony tissue should be carefully considered in patient follow-up. Osteoporosis is a significant problem in pediatric population because of ongoing growth and development of skeletal system. Two types of osteoporosis are primary and secondary types and children with neuromuscular disabilities constitute a major group with secondary osteoporosis...
August 10, 2017: European Journal of Paediatric Neurology: EJPN
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
#7
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
#8
REVIEW
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
#9
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
#10
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
#11
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
#12
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...
September 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28633151/age-based-normative-measurements-of-the-pediatric-pelvis
#13
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
#14
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 by reducing travel burden and streamlining care. In addition, the multidisciplinary care setting facilitates the integration of clinical research, patient advocacy, and care innovation (e.g...
November 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28546380/respiratory-implications-of-pediatric-neuromuscular-disease
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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