Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review

Voulgaris, Athanasios; Αντωνιάδου, Μαρία; Agrafiotis, Michalis; Steiropoulos, Paschalis

doi:10.1155/2019/2734054

Cited by 37 publications

(25 citation statements)

References 136 publications

(175 reference statements)

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“…However, episodic attacks of acute respiratory failure (ARF) may further aggravate the already existed respiratory compromises (7). Factors posing a risk of ARF in children with NMD are usually multifactorial and occur simultaneously (8)(9)(10)(11). Table 1 summarizes the risk levels of various NMD potentially affected by the acute respiratory compromise.…”

Section: At Birth or Within The First Year Of Lifementioning

confidence: 99%

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Noninvasive Ventilation and Mechanical Insufflator-Exsufflator for Acute Respiratory Failure in Children With Neuromuscular Disorders

Chen

Hsu

2020

Front. Pediatr.

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Children with neuromuscular disorder (NMD) usually have pulmonary involvement characterized by weakened respiratory muscles, insufficient coughing, and inability to clear airway secretions. When suffering from community-acquired pneumonia, these patients are more likely to develop acute respiratory failure (ARF). Therefore, recurrent pneumonias leading to acute on chronic respiratory failure accounts for a common cause of mortality in children with NMD. For many years, noninvasive ventilation (NIV) has been regarded as a life-prolonging tool and has been used as the preferred intervention for treating chronic hypoventilation in patients with advanced NMD. However, an increasing number of studies have proposed the utility of NIV as first-line management for acute on chronic respiratory failure in NMD patients. The benefits of NIV support in acute settings include avoiding invasive mechanical ventilation, shorter intensive care unit or hospital stays, facilitation of extubation, and improved overall survival. As the difficulty in clearing respiratory secretions is considered a significant risk factor attributing to NIV failure, combined coughing assistance of mechanical insufflator-exsufflator (MI-E) with NIV has been recommended the treatment of acute neuromuscular respiratory failure. Several recent studies have demonstrated the feasibility and effectiveness of combined NIV and MI-E in treating ARF of children with NMD in acute care settings. However, to date, only one randomized controlled study has investigated the efficacy of NIV in childhood ARF, but subjects with underlying NMD were excluded. It reflects the need for more studies to elaborate evidence-based practice, especially the combined NIV and MI-E use in children with acute neuromuscular respiratory failure. In this article, we will review the feasibility, effectiveness, predictors of outcome, and perspectives of novel applications of combined NIV and MI-E in the treatment of ARF in NMD children.

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Section: At Birth or Within The First Year Of Lifementioning

confidence: 99%

“…Humidification of the ventilator is often beneficial in reducing sputum viscosity and mobilizing secretions. Therapies of nebulized bronchodilator or systemic steroid may be considered if evidence of asthma or asthmatic bronchitis (10,60).…”

Section: Combined Niv and Mi-e In Arf Treatment Of Nmd Childrenmentioning

confidence: 99%

Noninvasive Ventilation and Mechanical Insufflator-Exsufflator for Acute Respiratory Failure in Children With Neuromuscular Disorders

Chen

Hsu

2020

Front. Pediatr.

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Section: Introductionmentioning

confidence: 99%

“…In children with neuromuscular disease (NMD), respiratory muscle weakness is frequently not obvious and is difficult to estimate 1 . Muscle weakness predisposes to repetitive pneumonia and atelectasis, leading ultimately to respiratory failure 1,2 . Clinical assessment of respiratory function in myopathic patients is a poor predictor of sleep-disordered breathing (SDB) and objective respiratory diagnostic tests are essential 2,3 .…”

Section: Introductionmentioning

confidence: 99%

Assessment of respiratory function and sleep disorders in children with myopathy

El-Baroudy

Falaki

Afify

et al. 2021

Sri Lanka J Child Health

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Background: Impaired activity of respiratory muscles and poor lung mechanisms predispose to sleep disordered breathing in neuromuscular disorders. Although it may lead to major morbidity, its relation to respiratory function is poorly defined. Objectives:To evaluate respiratory muscle function and sleep disorders in children with myopathy and their relationship to daytime and nocturnal symptoms and oxygen saturation. Method:A cross-sectional study was carried out on 30 children, 20 males and 10 females, diagnosed with myopathy at Abou El Reesh Paediatric Hospital, Cairo University. Arterial blood gases, creatine kinase (CK), aspartate transaminase (AST) and alanine transaminase (ALT) levels were measured. All subjects underwent respiratory function tests using spirometry, overnight polysomnography and diaphragmatic ultrasound. Results:Patients were assigned into 2 groups based on respiratory function tests assessed by spirometry. Group A included 14 patients with normal respiratory function tests and Group B included 16 patients with abnormal respiratory function tests. A significant difference was noted as regards symptoms suggestive of poor sleep quality, including somnolence, waking unrested and frequent awakening (p=0.005). Apnoea hypopnoea index (AHI) was significantly higher in group B patients (p=0.04). AHI was abnormal in 43% of patients in group A and 69% of patients in group B. Obstructive apnoeic and hypopnoeic events were detected in all patients with abnormal AHI. No significant difference was noted regarding sleep staging, sleep efficiency or total sleep test (TST).

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“…Similarly, the institution of intensive mechanical respiratory and nutritional support reduced risk of death in spinal muscular atrophy (SMA) type 1 patients by 70% 9 . Despite the use of NIV, however, progressive respiratory insufficiency remains the main cause of morbidity and mortality for individuals with NMD 10 . While NIV has been a major advance for individuals with NMD, survival without dependence on respiratory technology would represent a huge improvement in quality of life.…”

Section: Introductionmentioning

confidence: 99%

Sleep disordered breathing: Assessment and therapy in the age of emerging neuromuscular therapies

Gurbani

Pascoe

Katz

et al. 2020

Pediatric Pulmonology

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The term neuromuscular disease (NMD) encompasses a large variety of disorders that result in abnormal muscle function. Although it may be conventional to relate the use of this term to the most common muscular diseases (Duchenne muscular dystrophy [DMD], spinal muscular atrophy [SMA], and amyotrophic lateral sclerosis, etc), it is important to extend the term to pathologies manifested by severe neurologic (brain and spinal cord) malformations and injuries. In many of these scenarios, there are common mechanisms that contribute to sleep disordered breathing (SDB) and respiratory insufficiency although comorbidities may be somewhat different. Advances in the understanding of these diseases and their natural history, and increasing availability of mechanical ventilation to these patients have improved survival. The development of novel genetic and molecular therapies (as in the cases of DMD, SMA, and X‐linked myotubular myopathy) provides an opportunity to use SDB as a reasonable outcome measure while also allowing the use of polysomnography as a validation tool in the assessments of effectiveness of therapies. We seek to provide an understanding of SDB in NMDs, and in the same light, would like to begin the conversation of thinking about weaning respiratory support when possible.

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Respiratory Involvement in Patients with Neuromuscular Diseases: A Narrative Review

Cited by 37 publications

References 136 publications

Noninvasive Ventilation and Mechanical Insufflator-Exsufflator for Acute Respiratory Failure in Children With Neuromuscular Disorders

Noninvasive Ventilation and Mechanical Insufflator-Exsufflator for Acute Respiratory Failure in Children With Neuromuscular Disorders

Assessment of respiratory function and sleep disorders in children with myopathy

Sleep disordered breathing: Assessment and therapy in the age of emerging neuromuscular therapies

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