
Respiratory Disorders in Neuromuscular Disease: Management and Practice Principles
Beschreibung
Weitere Details
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Inhalt
- Intro
- Contents
- Preface
- Introduction
- Overview of Neuromuscular Disorders (NMDs) Affecting Respiratory Function
- Abstract
- Abbreviations
- Upper Motoneuron Lesions/Pyramidal Insufficiency
- Stroke
- Diseases of the Spinal Cord (SCI)
- Parkinson's Disease
- Amyotrophic Lateral Sclerosis (ALS)
- Anterior Horn Cell Disorders
- Brachial Plexitis
- Peripheral Neuropathies
- Other Neuropathies Which Can Cause Acute Respiratory Impairment
- Disorders of Peripheral Nerves
- Diaphragm Paralysis
- Bilateral Diaphragm Paralysis
- Critical Illness Polyneuropathy (CIP)
- Disorder of the Neuromuscular Junction
- Myasthenia Gravis (MG)
- Botulism
- Congenital Myopathies
- Duchenne Muscular Dystrophies
- Facioscapulohumeral Dystrophy (FSH)
- Limb-Girdle Dystrophy (LGD)
- Mitochondrial Myopathy (MM)
- Inflammatory Myopathy
- Other Myopathies
- Inclusion-Body Myopathy (IBM)
- Titinopathy and Myofibrillar Myopathy
- Necrotizing Autoimmune Myopathies (NAMs)
- Conclusion
- References
- Section 1. Physiology Neuromuscular Disorders
- Chapter 1
- Respiratory Phenotypes of Pediatric Patients
- Abstract
- Introduction
- Pathophysiology of the Respiratory Complications of NMD
- Major Congenital NMDS Occurring in Pediatric Patients
- NMD Affecting the Anterior Horn Cells
- Spinal Muscular Atrophy
- SMA Linked to SMN1 Gene
- Other, NON-SMN1 Linked SMAs
- NMDs Affecting the Peripheral Nerve
- NMD Affecting the Neuromuscular Junction
- Congenital Myasthenic Syndromes (CMS)
- NMD Affecting the Muscle
- Muscular Dystrophies (MD)
- Congenital Myopathies
- Myotonic Dystrophies
- Metabolic Myopathies
- Disorders of Glycogen Metabolism
- Disorders of Lipid Metabolism
- Mitochondrial Defects (MD)
- Conclusion
- References
- Chapter 2
- Respiratory Muscle Strength
- Abstract
- Abbreviations
- Introduction
- Anatomy and Physiology of the Respiratory Muscles
- Assessment of Respiratory Muscle Strength
- History and Physical Examination
- Imaging
- Radiographs
- Ultrasound
- Volumetric Imaging
- Objective Measurements of Respiratory Muscle Strength
- Volitional Tests of Respiratory Muscle Strength
- Static and Dynamic Lung Function
- Pressure Measurement
- Transdiaphragmatic Pressure
- Mouth and Nasal Pressure Measurements
- Maximum Inspiratory and Expiratory Pressure
- Nasal Pressure
- Non-Volitional Tests of Respiratory Muscle Strength
- Phrenic Nerve Stimulation
- Abdominal Muscle Stimulation
- Clinical Application of Respiratory Muscle Strength Assessment Tools
- Sleep Disordered Breathing in Neuromuscular Patients
- Prognostication
- Conclusion
- Key Messages
- References
- Chapter 3
- Cough Function Abnormalities
- Abstract
- Abbreviations
- Introduction
- Physiology of Cough
- Clinical and Functional Assessment of Cough
- Cough Reflex in Neuromuscolar Diseases
- References
- Chapter 4
- Diurnal Pulmonary Function Testing in Neuromuscular Disorders
- Abstract
- Introduction
- Pulmonary Function Testing
- Spirometry
- Lung Volumes
- Respiratory Muscle Strength Testing (MIP, MEP and SNIP)
- Peak Cough Flow
- Diurnal Blood Gas Analysis
- References
- Section 2. Methodology/Non Invasive Ventilation
- Chapter 5
- Sleep Disorders in Neuromuscular Diseases
- Abstract
- Abbreviations
- Introduction
- Normal Sleep Respiratory Physiology
- Sleep-Disordered Breathing in Patients with Neuromuscular Diseases
- Symptoms and Signs
- Types of Sleep-Disordered Breathing Events in Patients with Neuromuscular Diseases
- Sleep-Disordered Breathing Events Associated with Disease Pathophysiology
- Sleep-Disordered Breathing Events Associated with Noninvasive Ventilation
- Non-Respiratory Sleep Disorders in Patients with Neuromuscular Diseases
- Conclusion
- References
- Chapter 6
- Initiation of Non-Invasive Ventilation in Children with Neuromuscular Disease
- Abstract
- Introduction
- Identifying the Need for NIV
- Interfaces and Ventilation Modes
- Pediatric Interfaces
- Ventilator Modes
- Pressure-Targeted Ventilation
- Spontaneous Mode
- Spontaneous/Timed Mode Ventilation
- Pressure Control Mode
- Volume Assured Pressure Support
- Volume Targeted Ventilation
- Initial Ventilator Settings
- Alarms and Monitoring
- Benefits of NIV in Childhood Neuromuscular Disease
- NIV Complications
- Relative Contraindications to NIV
- Monitoring NIV in Children
- Long-Term Goals of NIV
- References
- Chapter 7
- Initiation of NIV in Adults
- Abstract
- Purpose of Review
- Recent Findings
- Summary
- Main Summary Results
- Key Messages
- References
- Chapter 8
- Modes of Ventilation
- Abstract
- Introduction
- Pathophisiology of Respiratory Failure in Neuromuscular Diseases
- Alveolar Hypoventilation
- Decreased Respiratory Drive
- Increased Mechanical Load
- Upper Airway Obstruction
- Impaired Chest Wall and Lung Mechanics
- Disminished Respiratory Muscle Performance
- Impaired Cardiovascular Performance
- Impaired Gas Exchange
- Diffuse Microatelectasis
- Ineffective Cough/Bronchopneumonia
- Decreased Cardiac Function
- Mechanisms of Action in NIV
- Clinical and Functional Effects of NIV
- Assited/Controlled Ventilation
- Pressure-Support Ventilation
- Assist Pressure-Controlled Ventilation
- Discussion
- Trigger
- Rise Time
- Cycle
- Ramp
- References
- Chapter 9
- Interface, Mouthpiece, Nasal Face/Alternative Interface
- Abstract
- Abbreviations
- Introduction
- Interfaces
- Types of Interfaces
- Nasal Mask
- Oro-Nasal Mask
- Oral Mask
- Mouthpiece
- Nasal Pillow Mask
- Total Face Mask
- Helmet
- IAPV
- Cuirass
- Different Interfaces in the Acute Setting
- MPV
- Amyotrophic Lateral Sclerosis (ALS)
- Duchenne Dystrophy
- Other Neuromuscular Disease
- COPD
- Intermittent Abdominal Compression Ventilation (IAPV)
- Negative Interface
- Problems Related to the Interface
- Interface Type and Upper Airway Obstruction
- Air Leaks
- Nasal or Oral Dryness and Nasal Congestion
- Decubitus
- Conclusion
- Key Messages
- References
- Chapter 10
- Negative Mechanical Ventilation
- Abstract
- Background
- Ventilation Mechanics
- Machines for Non-Invasive Extra-Thoracic Negative Ventilation
- Iron Lung
- Poncho
- Cuirass
- Extra-Toracic Negative Mechanical Ventilation Mode
- Intermittent Negative Pressure Ventilation (NPV)
- Negative and Positive Pressure Ventilation (NPV)
- Extra-Thoracic Continuous Negative Pressure Ventilation (CNPV)
- Ventilation at Two Levels of Negative Pressure (NPV-NEEP)
- Applications of NPV in Various Pathologies
- Neuromuscular Disorders
- Extra-Thoracic Ventilation in Adults with Respiratory Failure
- Extra-Thoracic Ventilation in Rehabilitation
- Extra-Thoracic Ventilation in Children and Infants
- Advantages and Disadvantages of Negative Extra-Thoracic Mechanical Ventilation
- References
- Chapter 11
- Intermittent Abdominal Mechanical Ventilation
- Abstract
- Introduction
- Advances over the Years
- IAPV Mechanism of Action
- Clinical Applications
- Conclusion
- References
- Chapter 12
- MPV and Other Emergent Therapies
- Abstract
- Abbreviations
- Introduction
- MPV Ventilation
- In What Diseases?
- Amyotrophic Lateral Sclerosis (ALS)
- Duchenne Muscular Distrophy
- Myotonic Dystrophy
- Other Neuromuscular Disease
- Conclusion
- Key Messages
- References
- Chapter 13
- Noninvasive Ventilation
- Abstract
- Introduction
- Work of Breathing and Noninvasive Ventilation
- Benefits of Noninvasive Ventilation
- Assessing Need for Noninvasive Ventilation
- Noninvasive Ventilation Basics
- Ensuring Adequate Ventilation Overtime
- References
- Chapter 14
- Continuous Noninvasive Ventilator Support
- Abstract
- Introduction
- Historical Background
- Pathophysiology
- Long-Term Continuous NIV and Survival
- Indications and Methods of Use
- Criteria to Start Daily NIV (Table 1)
- Necessary Instruments and Settings
- Ventilator
- Ventilation Modality and Circuit
- Interfaces (Figure 1)
- Alternative Daily Ventilation Systems
- Monitoring
- Tele-Monitoring
- The Caregiver's Home Management
- Twenty-Four Hour Ventilation Limits and Transition to Invasive Ventilation
- Key Messages
- References
- Chapter 15
- Risk of Unsuccessful Non-Invasive Ventilation in Neuromuscular Patients
- Abstract
- NIV Failure in the Acute Setting
- Introduction
- Definition
- Timing
- Reasons for Failure
- NIV Failure in the Chronic Setting
- Introduction
- Timing for NIV Initiation
- The Choice of Interface
- Ventilator Setting
- Patient-Ventilator Asynchrony
- Patient Related Issues
- Ventilator Related Issues
- Residual Sleep Respiratory Events
- Adherence to Non-Invasive Ventilation
- Management of Secretion and Airflow Humidification
- Follow-Up
- Conclusion
- References
- Section 3. Monitoring
- Chapter 16
- Monitoring and Synchrony Ventilator Patients
- Abstract
- Clinical Monitoring
- Monitoring of Gas Exchange
- Tidal Volume
- Air Leaks
- Asynchronies
- Ineffective Effort
- Autotriggering
- Cycling Asynchrony
- Patient Ventilatory Asynchrony and Sleep Alteration
- References
- Section 4. Airways and Clearance
- Chapter 17
- Laryngeal Response Patterns during NIV and Mechanically Assisted Cough
- Abstract
- Laryngeal Anatomy and Function
- Cough at the Laryngeal Level
- Bulbar Innervated Muscle Weakness
- Evaluation of the Laryngeal Responses during Respiratory Therapy
- Laryngeal Responses to Non-Invasive Ventilation
- In Health
- In Patients
- Laryngeal Responses to Mechanically Assisted Cough
- In Health
- In Patients
- Clinical Implications
- Key Messages
- References
- Chapter 18
- Tracheostomy Timing and Choice of Cannula
- Abstract
- Introduction
- Indications to Tracheostomy in Neuromuscular Patients
- Timing of Tracheotomy
- Tracheostomy Technique
- Choice of Cannula
- Fenestration
- Inner Cannula
- Cuff Type and Management
- References
- Chapter 19
- Cough Management
- Abstract
- Background
- Purpose
- Methods
- Results
- Conclusions
- Introduction
- Evaluation of Cough Function
- Peak Cough Flow (PCF)
- Vital Capacity (VC) / Insufflation Capacity (IC)
- Maximum Expiratory Pressure (MEP)
- Cough Augmentation Techniques
- Assisted Inspiration
- Manually Assisted Cough (MAC)
- Mechanical Insufflation-Exsufflation (MI-E)
- Conclusion
- Key Messages
- References
- Section 5. Methodology/Invasive Ventilation
- Chapter 20
- Invasive Ventilation
- Abstract
- Introduction
- History of Invasive Ventilation in NMD
- Indications of Invasive Ventilation
- Management of Invasive Ventilation
- Mode of Ventilation
- Choice of Ventilator
- Plan for Patients
- Tracheostomy Management
- Conclusion
- References
- Chapter 21
- Weaning from Mechanical Ventilation and Extubation
- Abstract
- Phases of Weaning
- Weaning Failure and Failed Extubation
- Critical Weaning Issues
- Assessment of Readyness to Wean
- How to Perform the SBT
- Management of Difficult Weaning
- Emerging Weaning Issues
- The Role of Tracheotomy in Weaning
- Use of High-Flow Nasal Cannula (HFNC)
- Weaning from Prolonged Mechanical Ventilation
- References
- Chapter 22
- Ventilatory Dependent Patient's Unweanable Conditions
- Abstract
- Introduction
- Ventilatory Dependent
- References
- Section 6. Perioperative Respiratory Management
- Chapter 23
- Perioperative Respiratory Management of Pediatric Patients with NMD
- Abstract
- Preoperative Management
- Respiratory Evaluation
- Cardiovascular Evaluation
- Nutritional Evaluation
- Evaluation of the Consent
- Intraoperative Management
- Post-Operative Management
- Respiratory Management
- Pain Management
- Cardiovascular Management
- Nutritional Management
- References
- Chapter 24
- Anesthetic Considerations for Neuromuscular Diseases
- Abstract
- Purpose of Review
- Recent Findings
- Preoperative
- Intraoperative
- Postoperative
- Pain Control Management
- Postoperative Nausea and Vomiting (PONV)
- Respiratory Management
- Conclusion
- Key Messages
- References
- Chapter 25
- Management of Acute Respiratory Failure in Pregnancy
- Abstract
- Abbreviations
- Introduction
- Physiologic Respiratory Changes in Pregnancy
- Causes of Respiratory Failure in Pregnancy
- Pregnancy-Specific
- Pulmonary Oedema
- Amniotic Fluid Embolism
- Aggravated by Pregnancy
- Gastric Aspiration
- Pulmonary Thromboembolic Disease
- Asthma
- Non-Specific Conditions
- Pulmonary Infection
- Acute Respiratory Distress Syndrome
- Restrictive Lung Disease
- Neuromuscular Disease
- Labour
- Postpartum
- Management of Pregnancy in Neuromuscular and Chest Wall Disease
- Monitoring during Pregnancy
- Respiratory Muscle Aids
- Ventilatory Management
- Noninvasive Ventilation
- Invasive Mechanical Ventilation
- References
- Chapter 26
- Cardiac Manifestations of Neuromuscular Disease
- Abstract
- Introduction
- Myotonic Dystrophy Type 1
- Cardiac Involvement and Sudden Death in DM1
- Sudden Cardiac Death
- Conduction Defects
- Tachyarrhythmias
- Myocardial Impairment and Heart Failure
- Implantable Cardiac Electrical Devices
- Cardiac Resynchronization Therapy in DM1 Patients
- Dystrophinopathies
- Duchenne Muscular Dystrophy
- Becker Muscular Dystrophy
- Dystrophinopathic Cardiomyopathy: Stages and Evolution
- Emery-Dreifuss Muscular Dystrophy
- Cardiac Involvement in EDMD
- Facio-Scapulo-Humeral-Dystrophy
- References
- Chapter 27
- Therapy of Scoliosis in Neuromuscular Pathology
- Abstract
- Neuromuscular Scoliosis Classification
- Primary Neuropathies
- Lower Motor Neuron
- Primary Myopathies
- Etiopathogenesis
- Physical Examination
- Medical Considerations
- Radiographic Assessment
- Conservative Treatment
- Surgical Treatment
- Conclusion
- References
- Section 7. Diagnosis Approach in Neuromuscular Disorders
- Chapter 28
- Imaging Techniques in NMD
- Abstract
- Introduction
- Anatomic/Physiological Background and VFSS Technique
- Pathophysiological Mechanisms of Dysphagia in NMD Patients
- Pathological VFSS Findings in NMD Patients
- Lateral (LL) and Anteroposterior (AP) Direct Neck Radiograph
- VFSS on LL Projection in NMD Patients
- Aspiration-Induced Lung Diseases (AILD) in NMD
- Aspiration Pneumonia (with or without Abscess Formation) (AILD-Parenchymal Lung Manifestation)
- Aspiration Pneumonitis and Mendelson Syndrome (AILD-Parenchymal Lung Manifestation)
- Bronchiectasis (AILD-Airway Manifestation)
- Diffuse Aspiration Bronchiolitis (DAB) (AILD-Airway Manifestation)
- Tracheobronchial Foreign Body (AILD-Airway Manifestation)
- Near-Drowning
- Chronic Exogenous Lipoid Pneumonia (AILD-Parenchymal Lung Manifestation)
- Chronic Interstitial Lung Disease (Chronic-ILD) and Organizing Pneumonia (AILD-Parenchymal Lung Manifestations)
- Multidisciplinary Team
- References
- Chapter 29
- Sonography in the Evaluation of the Disease
- Abstract
- Anatomy and Physiology of the Diaphragm
- Causes of Diaphragm Dysfunction
- Ultrasound Assessment of Diaphragmatic Function
- Diaphragm Dome Motion
- Diaphragm Muscle Thickening during Inspiration
- Clinical Applications of Diaphragm Ultrasound
- Ultrasound Diagnosis and Monitoring of Pleuropulmonary Complications
- Limitations of Diaphragm and Chest Ultrasound
- Future Directions
- References
- Chapter 30
- Vocal Cord Dysphonia and Sialorrhea
- Abstract
- Vocal Cord Dysphonia
- Motor Neuron Diseases
- Disease of the Neuromuscular Junction
- Diseases of the Peripheral Nerves
- Myopathy
- Extrapyramidal Diseases
- Spasmodic Dysphonia (SD)
- Aetiology and Pathogenesis
- Diagnosis
- Auditory-Perceptual Measures
- Vocal Fold Imaging
- Acoustic Measures
- Aerodynamic Measures
- Therapy
- Sialorrea
- Diagnosis
- Therapy
- References
- Chapter 31
- Screening and Evaluation Tools of Dysphagia in Neuromuscular Disorders
- Abstract
- Introduction
- Instrumental Examinations
- Non Instrumental Examinations
- Conclusion
- References
- Chapter 32
- Aspiration Treatment and Decisional Algorithm
- Abstract
- Abbreviations
- Overview of Dysphagia Associated with Neuromuscular Disease
- Patient Evaluation Overview
- The Intervention for Dysphagia Associated with Neuromuscular Disease
- Conclusion
- References
- Chapter 33
- Infections in Neuromuscolar Disease Patients: A Point in the Right Direction
- Abstract
- Pneumonia and Pneumonia in NMD
- Microbiology
- Diagnosis
- Treatment
- Community-Acquired Pneumonia
- Aspiration Community Acquired Pneumonia (ACAP)
- Virus Interstizial Pneumonia
- Pulmunary Fungai Infection
- Other Infections
- Prevention
- References
- Chapter 34
- Nutritional Support in Neuromuscular Diseases
- Abstract
- Introduction
- Malnutrition
- Nutritional Assessment
- Nutritional Support Planning
- Nutritional Requirements
- Routes of Administration of Artificial Nutrition
- Nutritional Mixtures
- Monitoring Artificial Nutrition
- References
- Section 8. Pulmonary Rehabilitation
- Chapter 35
- Respiratory Muscle Training in Children and Adults with Neuromuscular Diseases
- Abstract
- Introduction
- Respiratory Muscle Training
- Evidence
- Conclusion
- References
- Chapter 36
- Prevention of Pulmonary Morbidity
- Abstract
- Introduction
- A Protocol to Manage Respiratory Tract Infections
- Principles of Chest Physiotherapy
- Peripheral Airway Clearance Techniques
- Manual Techniques
- HFCWO and HFCWC
- Intrapulmonary Percussive Ventilation (IPV)
- Chest Wall Strapping
- Expiratory Flow Acceleration
- References
- Section 9. Home Mechanical Ventilation
- Chapter 37
- Discharge Planning from Hospital to Home Mechanical Ventilation
- Abstract
- Organization of the Plan of Care
- References
- Chapter 38
- Home Mechanical Ventilation
- Abstract
- Introduction
- Non-Invasive Ventilation (NIV) or Mask Ventilation
- Continuous Positive Airway Pressure (CPAP)
- Indication
- Telemonitoring
- Bilevel Positive Airway Pressure
- Indication
- Different Non-Invasive Techniques
- Ethical Approach
- References
- Section 10. Outcome
- Chapter 39
- Cause and Outcome of Acute Neuromuscular Respiratory Failure
- Abstract
- Introduction
- Causes of Acute Neuromuscular Respiratory Failure
- Outcomes of Acute Neuromuscular Respiratory Failure
- References
- Section 11. Health Care and Ethical Aspects
- Chapter 40
- The Caregiver Formation
- Abstract
- Introduction
- The Caregiver Figure: Definition and Role
- Caregiver and Patient Formation: A State of the Art
- Why
- Where
- When
- What
- Rationale for Ventilatory Support
- The Ventilator
- Ventilatory Parameters Surveillance
- Alarms and Devices Technical Malfunctioning
- Airway Patency and Secretion Removal
- Emergency Management
- How
- Evaluating the Caregiver Formation
- Future Perspectives: High Fidelity Simulation Training
- The Caregiver's Burden
- Conclusion
- References
- Chapter 41
- The Role of Non Invasive Ventilation in Quality of Life in Neuromuscular Diseases
- Abstract
- Abbreviations
- Introduction
- A Definition of Neuromuscular Disease
- What is Quality of Life?
- Exploring Quality of Life in Neuromuscular Diseases
- A Brief Analysis of Quality of Life in Neuromuscular Diseases
- The Link between Quality of Life and Non Invasive Ventilation
- The Controversial Sides of Measuring Quality of Life in Neuromuscular Diseases
- General Discussion
- What's Still Missing?
- References
- Chapter 42
- Telemonitoring
- Abstract
- Introduction
- Background
- Economic Considerations
- Critical Issues
- The Telemonitoring in the Context of Neuromuscular Patients
- General Considerations
- Telemedicine in the Long-Term Disease Monitoring
- Telemedicine in the Monitoring and Setting of Mechanical Ventilation
- Telemedicine in Rehabilitative Context
- Conclusion
- References
- Chapter 43
- Palliative Care
- Abstract
- Introduction
- End-Stage Management
- Pharmacological Treatment
- Oxygen Therapy and Mechanical Ventilation
- Pulmonary Physiotherapy
- Nutritional Assessment
- Communication Support
- Communication and Emotional Support
- The Family
- Decisions at the End of Life (The Good Death)
- References
- Chapter 44
- End-of-Life Care
- Abstract
- Introduction
- Spinal Muscular Atrophy (SMA)
- Duchenne Muscular Dystrophy (DMD)
- Amyotrophic Lateral Sclerosis (ALS)
- Conclusion
- References
- Chapter 45
- Ethical and Medico-Legal Aspects
- Abstract
- Abbreviations
- Introduction
- NIV Prolongs Survival, but Improves the Quality of Life Too?
- Tracheotomy is the Best Solution for Progression of Disease. and the Patient?
- When to Say "Stop?
- Conclusion
- References
- Editors' Contact Information
- Index
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