Predictors of severe chest infections in pediatric neuromuscular disorders
Introduction
Patients with neuromuscular disorders are often threatened by respiratory complications like peumonia and atelectasis which may cause acute respiratory failure [1], [2], [3]. Mortality due to pneumonia has been reported in 30% of patients with ALS and adult-onset-myotonic dystrophy [4], [5]. In spinal muscular atrophy type I respiratory complications [6], [7] lead to death during the first year, if patients were not treated by tracheotomy or recurrent invasive ventilation. Incidence of pneumonia in Duchenne patients was 0.8/year and in other myopathies 1/year [8].
Clearance of airway secretions is determined by mucociliary debris transport and a sufficient cough mechanism [9], [10]. This system may become overloaded in case of excessive bronchial secretions. Upper respiratory tract infections due to otherwise non-virulent viruses then lead to pneumonia and atelectasis.
In the literature respiratory complications have been related to different parameters of respiratory function testings. Respiratory failure occurred in patients with severe restrictive ventilatory defect [11] and impaired respiratory muscle strength [12]. ALS patients with peak expiratory cough flows <3 l/s had to undergo tracheotomy to prolong survival [13]. Chaudri et al. [14] showed a correlation between the absence of cough expiratory spikes and mortality in motor neuron disease.
In pediatric patients with neuromuscular disorders, spirometry was proven to discriminate between patients with nocturnal hypoventilation and those without [15]. A review of the current literature showed no description of the relation between respiratory function parameters and chest infections for patients at school age. Aim of the study was to investigate if simple bed side tests (spirometry, respiratory muscle function, peak cough flow) could identify pediatric patients at high risk for severe chest infections like pneumonia and/or atelectasis.
Section snippets
Patients
We performed a retrospective chart review of 46 patients with neuromuscular disorders who were referred to the Ruhrlandklinik in Essen and the University Hospital of Essen for respiratory assessment from years 2000 to 2004. Mean age was 12.7 ±3.6 years and ranged from 6 to 20 years. Twenty-eight male and 18 female patients were enrolled. Underlying diagnosis was Duchenne muscular dystrophy (DMD) in 16 patients, spinal muscular atrophy (SMA) in 14 patients (5 type Ib, 9 type II), congenital
Results
Mean lung function and respiratory muscle function values were as follows: IVC 1.08±0.68 l (37.6±21.6% pred.), FEV1 0.90±0.58 l (37.6±23.3% pred.), PCF 167.9±83.8 l/min, P01 0.29±0.17, PIP 3.19±1.71 kPa, P01/PIP 11.4±8.1% and PEP 2.72±1.17 kPa. PCF highly correlated with IVC (R=0.87), FEV1 (R=0.88), PEP (R=0.59) and respiratory muscle strain (R=−0.65); P<0.0001 for all. Weaker correlations were found between PCF and PIP (R=0.41) and PCF and P01 (R=−0.42); P<0.01 for all. PCF did not correlate with
Discussion
Our study is the first systematic analysis of respiratory assessment and its relation to incidence and risk of chest infections in children and adolescents with neuromuscular disorders. Not surprisingly, pediatric patients with neuromuscular disorders experience a rising risk of chest infections with progressive deterioration of lung function and peak cough flows. Therefore, patients at high risk can be easily identified by providing simple bed side tests measuring inspiratory vital capacitiy
References (27)
- et al.
Atelectasis and neuromuscular respiratory failure
Chest
(1984) Very severe spinal muscular atrophy (SMA type 0): an expanding clinical phenotype
Eur J Paediatr Neurol
(1999)Amyotrophic lateral sclerosis: predictors for prolongation of life by noninvasive respiratory aids
Arch Phys Med Rehabil
(1995)- et al.
Daytime predictors of sleep disordered breathing in children and adolescents with neuromuscular disorders
Neuromuscul Disord
(2003) Update and perspectives on noninvasive repiratory muscle aids. Part 2: the expiratory aids
Chest
(1994)Mechanical insufflation–exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques
Chest
(1993)- et al.
Maximum insufflation capacity
Chest
(2000) - et al.
Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning
Chest
(1996) - et al.
Prevention of pulmonary morbidity for patients with neuromuscular disease
Chest
(2000) - et al.
Migrating atelactesis in Werdnig–Hoffmann disease: pulmonary manifestations in two cases of spinal atrophy type I
Pediatr Pulmonol
(1999)
Respiratory failure in neuromuscular diseases
Thorax
Hospitalization in amyotrophic lateral sclerosis
Neurology
Age and cause of death in adult-onset myotonic dystrophy
Brain
Cited by (107)
Can postural changes in spirometry in children with Duchenne muscular dystrophy predict sleep hypoventilation?
2024, Paediatric Respiratory ReviewsManagement of acute respiratory failure in neuromuscular disease
2023, Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular DiseaseAssessment of need for airway clearance
2023, Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular DiseaseProposals from a French expert panel for respiratory care in ALS patients
2022, Respiratory Medicine and ResearchThe effect of nebulized antibiotics in children with tracheostomy
2021, International Journal of Pediatric Otorhinolaryngology