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Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease
- 1st Edition - April 13, 2023
- Editor: Oscar Henry Mayer
- Language: English
- Paperback ISBN:9 7 8 - 0 - 3 2 3 - 9 5 7 4 7 - 2
- eBook ISBN:9 7 8 - 0 - 3 2 3 - 9 5 7 4 8 - 9
Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease covers the broad medical problems and specific pulmonary conditions in patients with pediat… Read more
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Request a sales quotePulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease covers the broad medical problems and specific pulmonary conditions in patients with pediatric neuromuscular disease, ranging from the different types of neuromuscular disease, their pathophysiology, and assessment and management, including both novel disease modifying pharmacotherapies and state-of-the-art clinical management. This book provides evidence-based guidance for pediatric patients with neuromuscular disease, and is a valuable resource to pediatric pulmonologists, critical care physicians, and respiratory therapists who want an update and understanding on the cutting-edge therapies and standards of care for managing this population.
- Provides a single comprehensive source of information to properly guide pulmonary assessments of pediatric neuromuscular disease
- Discusses recent advancements in both medications and clinical respiratory management for pediatric patients
- Covers different types of neuromuscular disease, including spinal muscular atrophy, Duchenne muscular dystrophy, congenital muscular dystrophy, and more
Pediatric pulmonologists, critical care physicians, respiratory therapists, neurologists and general pediatricians
- Cover image
- Title page
- Table of Contents
- Copyright
- Dedication
- Contributors
- Preface
- References
- Section 1: Introduction
- Chapter 1: Types of neuromuscular disease
- Abstract
- Introduction
- Genetic neuromuscular disease
- Acquired neuromuscular disease
- Conclusion
- References
- Chapter 2: Pulmonary pathophysiology of neuromuscular disease
- Abstract
- Introduction
- Components of respiratory system and mechanisms of respiratory progression
- Clinical features of respiratory involvement in neuromuscular disease
- References
- Chapter 3: The natural history of pulmonary disease
- Abstract
- References
- Section 2: Assessment
- Chapter 4: Assessment of pulmonary function
- Abstract
- Introduction
- Clinical evaluation: History and physical examination
- Volume
- Flow
- Pressure
- Gas exchange
- Conclusion
- References
- Further reading
- Chapter 5: Assessment of sleep-disordered breathing
- Abstract
- Introduction
- Pathophysiology
- Sleep-disordered breathings events associated with NMD
- Definitions of SDB and hypoventilation
- Evaluation of SDB
- Conclusion
- References
- Chapter 6: Assessment of need for airway clearance
- Abstract
- Introduction
- History and physical examination
- Pulmonary function testing
- Summary
- References
- Section 3: Clinical management
- Chapter 7: Disease-modifying pharmacotherapies
- Abstract
- Introduction
- Duchenne muscular dystrophy
- Spinal muscular atrophy
- Other rare neuromuscular diseases
- Pharmacoeconomics
- Conclusion
- References
- Chapter 8: Airway clearance
- Abstract
- The need for airway clearance
- Cough assessment
- Classification of airway clearance techniques (Table 8.1)
- Proximal airway clearance techniques
- Distal airway clearance techniques
- Medications
- Co-morbid conditions that can impact cough efficacy
- Considerations with tracheostomy/endotracheal tubes
- Clinical care guidelines
- Conclusion
- References
- Chapter 9: Aerodigestive problems and management in children with neuromuscular disorders
- Abstract
- Aerodigestive management in children with neuromuscular disorders
- Normal swallow
- Evaluation
- Management
- Noninvasive and invasive ventilation and aspiration
- References
- Chapter 10: Chronic mechanical ventilation
- Abstract
- Introduction
- Role and benefits of mechanical ventilation in NMD
- Initiation of mechanical ventilation in NMD
- Interfaces and modes
- Tracheostomy
- Monitoring: Clinical
- Monitoring: Electronic
- Ventilator data collection methods
- Ventilator manufacturer-specific platforms
- Summary
- Vignettes
- References
- Chapter 11: Thoraco-spinal disease
- Abstract
- Introduction
- Respiratory function in children with scoliosis
- Respiratory function before and after treatment in children with neuromuscular weakness and scoliosis
- References
- Chapter 12: Challenges in managing hypertonic neuromuscular disease
- Abstract
- Aspiration
- Spinal and chest wall deformities
- Airway clearance
- Upper airway obstruction and sleep-disordered breathing
- Conclusions
- References
- Chapter 13: Management of acute respiratory failure in neuromuscular disease
- Abstract
- Introduction
- Baseline risk factors for acute respiratory failure
- Causes of acute respiratory failure in NMD
- Acute management of respiratory illness in children with NMD
- Airway clearance
- Weaning
- Tracheostomy and invasive ventilation
- Conclusion
- References
- Chapter 14: Home management
- Abstract
- Preparation
- Home monitoring
- Community resources
- Routine health maintenance
- Contingency planning
- Quality of life
- References
- Section 4: Patient focused
- Chapter 15: Transition from pediatric to adult care in neuromuscular disease
- Abstract
- Introduction
- Transition stakeholders
- Rationale for transition planning in NMD
- Transition challenges in patients NMD and chronic respiratory failure
- Components of a successful transition program
- Health care transition timeline
- Transition lessons from other chronic disease models
- Special considerations for individuals with NMD during transition
- Future research
- Conclusion
- References
- Section 5: Conclusion
- Chapter 16: The evolving course of neuromuscular disease
- Abstract
- Defining natural history
- Spinal muscular atrophy
- Duchenne muscular dystrophy
- Conclusion
- References
- Index
- No. of pages: 254
- Language: English
- Edition: 1
- Published: April 13, 2023
- Imprint: Academic Press
- Paperback ISBN: 9780323957472
- eBook ISBN: 9780323957489
OM
Oscar Henry Mayer
Dr Mayer is Attending Physician, Division of Pulmonology at the Children’s Hospital of Philadelphia and Professor of Clinical Pediatrics, at the Perelman School of Medicine, University of Pennsylvania. He has been managing patients with pediatric neuromuscular disease over the last 20 years and has become a recognized international expert in the field, has served on committees to determine standard of care practices and has lectured extensively on a variety of different topics in this field.
Affiliations and expertise
Professor of Clinical Pediatrics, The Perelman School of Medicine, University of Pennsylvania, USARead Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease on ScienceDirect