Abstract

Neuromuscular diseases may involve all major respiratory muscles groups including inspiratory, expiratory, and bulbar muscles. Respiratory complications are the major cause of morbidity and mortality. Pneumonia represents a frequent cause of morbidity in children with neuromuscular disease. The aim of this review is to collect knowledge about pneumonia in children with neuromuscular diseases. Pneumonia usually follows viral respiratory infections of the upper respiratory tract, due to the combination of an increased amount of nasal and oral secretions and an impairment of the cough efficiency and of the clearance of secretions due to the muscle weakness, further compromised by the infection itself. The accumulation of bronchial secretions leads to atelectasis and promote bacterial infection. Moreover, dysfunction of swallowing mechanism exposes these children to the risk of developing aspiration pneumonia. However, etiology of viral and bacterial respiratory infection in these patients is still poorly studied.

Highlights

  • Neuromuscular diseases (NMD) may involve most respiratory muscles groups including inspiratory, expiratory, and bulbar

  • Pneumonia represents a frequent cause of morbidity in children with NMD; inhalation, impaired cough and atelectasis are the main risk factors

  • Few data come from a cohort study conducted on 28 children with NMD admitted to PICU (13) which found both viruses and bacteria (Mycoplasma pneumoniae, Staphylococcus aureus, Haemophilus influenzae) as etiological agents associated to acute respiratory infections, defined by a positive culture from airways within 48 h of admission or signs of upper respiratory tract, respiratory symptoms, fever, and/or leukocytosis (12)

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Summary

INTRODUCTION

Neuromuscular diseases (NMD) may involve most respiratory muscles groups including inspiratory, expiratory, and bulbar. Respiratory complications are the major cause of morbidity and mortality. The natural course of NMD is characterized by ineffective cough and swallowing disorders leading to chronic aspiration, poor secretion clearance, pneumonia and hypercapnic respiratory failure. Pneumonia represents a frequent cause of morbidity in children with NMD; inhalation, impaired cough and atelectasis are the main risk factors. Scarce literature investigating on pneumonia in children with NMD is available. This review is part of the research topic “Emerging Pneumonia in Children” and its aim is to summarize knowledge about pneumonia in children with neuromuscular diseases and to encourage research in this neglected field

RESPIRATORY INVOLVEMENT IN NEUROMUSCULAR DISEASE
PATHOPHYSIOLOGY OF PNEUMONIA IN NEUROMUSCULAR DISEASES
Aspiration Pneumonia
DISCUSSION AND FUTURE
Findings
AUTHOR CONTRIBUTIONS
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