Predictors of severe chest infections in pediatric neuromuscular disorders

doi:10.1016/j.nmd.2006.02.003

Neuromuscular Disorders

Volume 16, Issue 5, May 2006, Pages 325-328

https://doi.org/10.1016/j.nmd.2006.02.003 Get rights and content

Abstract

Chest infections are serious complications in neuromuscular disorders. The predictive values of lung and respiratory muscle function including peak cough flow still remain unclear.

We performed retrospective analysis of 46 children and adolescents (12.7±3.7 years) in whom lung function, respiratory muscle function and peak cough flows had been obtained. Data were related to: (1). number of chest infections and days of antibiotic treatment the year prior to the study and (2). history of severe chest infection requiring hospital admission.

The number of chest infections and the number of days treated with antibiotics correlated with Inspiratory Vital Capacity IVC, peak cough flow PCF and Peak Expiratory Pressure PEP. Twenty-two patients were hospitalized at least once due to severe chest infection. IVC (0.65 vs. 1.44 l; P<0.0001) and PCF (116 vs. 211 l/min; P<0.0005) in these patients were significantly lower than in the non-hospitalized group. IVC<1.1 l and PCF<160 l/min were specific and sensitive thresholds to discriminate between patients who had already suffered severe chest infections and those who had not. Therefore, spirometry and peak cough flow are reliable tests to identify patients at high risk for severe chest infections.

Patients with IVC below 1.1 l and/or PCF below 160 l/min should be well monitored and introduced to assisted coughing techniques.

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

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Predictors of severe chest infections in pediatric neuromuscular disorders

Abstract

Introduction

Section snippets

Patients

Results

Discussion

Chest

Eur J Paediatr Neurol

Arch Phys Med Rehabil

Neuromuscul Disord

Chest

Chest

Chest

Chest

Chest

Migrating atelactesis in Werdnig–Hoffmann disease: pulmonary manifestations in two cases of spinal atrophy type I

Pediatr Pulmonol

Respiratory failure in neuromuscular diseases

Thorax

Hospitalization in amyotrophic lateral sclerosis

Neurology

Age and cause of death in adult-onset myotonic dystrophy

Brain