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Pediatric neuromuscular disease

Pedro Taffarel, German Bonetto, Facundo Jorro Barón, Claudia Meregalli
INTRODUCTION: Children in pediatric intensive care units (PICUs) are exposed to experiencing pain, stress and anxiety due to their disease, treatment or care setting. Adequate sedation and analgesia are key to their care, particularly in patients requiring mechanical ventilation (MV). OBJECTIVE: To determine the usual practice in sedation and analgesia management in patients requiring MV in PICUs in Argentina. MATERIAL AND METHODS: Descriptive, crosssectional, multi-center study conducted by means of e-mailed surveys...
April 1, 2018: Archivos Argentinos de Pediatría
Ashley Gionfriddo, Mika L Nonoyama, Peter C Laussen, Peter N Cox, Megan Clarke, Alejandro A Floh
OBJECTIVES: To promote standardization, the Centers for Disease Control and Prevention introduced a new ventilator-associated pneumonia classification, which was modified for pediatrics (pediatric ventilator-associated pneumonia according to proposed criteria [PVAP]). We evaluated the frequency of PVAP in a cohort of children diagnosed with ventilator-associated pneumonia according to traditional criteria and compared their strength of association with clinically relevant outcomes. DESIGN: Retrospective cohort study...
March 15, 2018: Pediatric Critical Care Medicine
Fiachra E Rowan, Biju Benjamin, Jurek R Pietrak, Fares S Haddad
BACKGROUND: Prevention of dislocation after primary total hip arthroplasty (THA) begins with patient preoperative assessment and planning. METHODS: We performed a literature search to assess historical perspectives and current strategies to prevent dislocation after primary THA. The search yielded 3458 articles, and 154 articles are presented. RESULTS: Extremes of age, body mass index >30 kg/m2 , lumbosacral pathology, surgeon experience, and femoral head size influence dislocation rates after THA...
March 7, 2018: Journal of Arthroplasty
Rambod Amirnovin, Sara Aghamohammadi, Carley Riley, Marlyn S Woo, Sylvia Del Castillo
BACKGROUND: The population of children requiring home mechanical ventilation has evolved over the years and has grown to include a variety of diagnoses and needs that have led to changes in the care of this unique population. The purpose of this study was to provide a descriptive analysis of pediatric patients requiring home mechanical ventilation after hospitalization and how the evolution of this technology has impacted their care. METHODS: A retrospective, observational, longitudinal analysis of 164 children enrolled in a university-affiliated home mechanical ventilation program over 26 years was performed...
March 6, 2018: Respiratory Care
Holly L Peay, Barbara B Biesecker, Benjamin S Wilfond, Jill Jarecki, Kendall L Umstead, Diana M Escolar, Aad Tibben
BACKGROUND/AIMS: Pediatric rare disease presents a challenging situation of high unmet need and a limited pool of potential clinical trial participants. Understanding perspectives of parents of children who have not participated in trials may facilitate approaches to optimize participation rates. The objective of this study was to explore factors associated with parental interest in enrolling children with pediatric neuromuscular disorders in clinical trials. METHODS: Parents of individuals with Duchenne or Becker muscular dystrophy and spinal muscular atrophy were recruited through advocacy organizations, a registry, and clinics...
January 1, 2018: Clinical Trials: Journal of the Society for Clinical Trials
Xiaobin Ge, Lei Zhang, Gonglei Yue, Xuewei Xiao, Zhenjie Yang, Bin Tang, Shumei Wu, Yan Li, Guangzhong Du
OBJECTIVE: To summarize the 2491 acupuncture inpatient consultations in Qilu Hospital of Shandong University during 2014, and analyze the application of acupuncture in comprehensive hospital, hoping to provide direction and methods for clinical practice and scientific research of acupuncture. METHODS: The consultation record and hospitalization information during 2014 in Department of Acupuncture-Moxibustion and Tuina, Qilu Hospital of Shandong University, were collected and summarized through Inpatient Information Management System...
February 12, 2018: Zhongguo Zhen Jiu, Chinese Acupuncture & Moxibustion
Akmer Mutlu, Halil Alkan, Tuzun Fırat, Aynur A Karaduman, Oznur T Yilmaz
OBJECTIVE: To compare the fatigue levels and energy expenditure of children with Duchenne Muscular Dystrophy (DMD) at different functional levels with healthy children. METHODS: The cross-sectional study was carried out in the Unit of Pediatric Neuromuscular Diseases in the Department of Physiotherapy and Rehabilitation, Faculty of Health Science, Hacettepe University between March 2015 and January 2016. Fifty two children diagnosed with DMD in Level I-III according to the Brooke Functional Classification Scale and 17 healthy children were included in the study...
January 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
Marina Boido, Elena De Amicis, Valeria Valsecchi, Marco Trevisan, Ugo Ala, Markus A Ruegg, Stefan Hettwer, Alessandro Vercelli
Spinal muscular atrophy (SMA) is a pediatric genetic disease, characterized by motor neuron (MN) death, leading to progressive muscle weakness, respiratory failure, and, in the most severe cases, to death. Abnormalities at the neuromuscular junction (NMJ) have been reported in SMA, including neurofilament (NF) accumulation at presynaptic terminals, immature and smaller than normal endplates, reduced transmitter release, and, finally, muscle denervation. Here we have studied the role of agrin in SMAΔ7 mice, the experimental model of SMAII...
2018: Frontiers in Cellular Neuroscience
Robert J Graham, Michael L McManus, Angie Mae Rodday, Ruth Ann Weidner, Susan K Parsons
OBJECTIVE: To describe program design, costs, and savings implications of a critical care-based care coordination model for medically complex children with chronic respiratory failure. DESIGN: All program activities and resultant clinical outcomes were tracked over 4 years using an adapted version of the Care Coordination Measurement Tool. Patient characteristics, program activity, and acute care resource utilization were prospectively documented in the adapted version of the Care Coordination Measurement Tool and retrospectively cross-validated with hospital billing data...
February 3, 2018: Pediatric Critical Care Medicine
M Girabent-Farres, L Monne-Guasch, C Bagur-Calafat, J Fagoaga
INTRODUCTION: Paediatric neuromuscular disorders, which negatively impact on children's health-related quality of life (HRQoL), are a frequent cause of parental consultation with a physiotherapist. Parents' stress overload triggers a poor perception of their children's HRQoL. For this reason, it is essential to have psychometrically sound instrument to measure parent's perceptions such as the Pediatric Quality of Life Inventory (PedsQL) Neuromuscular Module. AIM: To assess the validity and reliability of the Spanish version of PedsQL Neuromuscular Module for parent perceived quality of life of children aged 2-4 with neuromuscular disorders...
February 1, 2018: Revista de Neurologia
Amanda Dexter, Neil McNinch, Destiny Kaznoch, Teresa A Volsko
OBJECTIVE: This study sought to validate pediatric models with normal and altered pulmonary mechanics. METHODS: PubMed and CINAHL databases were searched for studies directly measuring pulmonary mechanics of healthy infants and children, infants with severe bronchopulmonary dysplasia and neuromuscular disease. The ASL 5000 was used to construct models using tidal volume (VT), inspiratory time (TI), respiratory rate, resistance, compliance, and esophageal pressure gleaned from literature...
January 17, 2018: Simulation in Healthcare: Journal of the Society for Simulation in Healthcare
Rebecca R Moultrie, Megan A Lewis, Ryan S Paquin, Ann Lucas, Jill Jarecki, Holly L Peay
Duchenne/Becker muscular dystrophy (DBMD) and spinal muscular atrophy (SMA) are rare neuromuscular disorders that present challenges to therapeutic and clinical trial decision making. We developed an interactive, evidence-based online tool designed to encourage thoughtful deliberation of the pros and cons of trial participation and to inform meaningful discussions with healthcare providers. Prior research demonstrates the importance of tool availability at the time each family is considering trial participation, which may be prior to the informed consent process...
December 20, 2017: Journal of Genetic Counseling
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
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
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
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
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
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
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
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
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