Chest
Clinical Investigations: MiscellaneousThoracoabdominal Pattern of Breathing in Neuromuscular Disorders
Section snippets
Subjects
Fifty subjects (24 boys, 26 girls), mean age 9.7±3.1 y, mean height 123.8±16.9 cm, mean weight 21.6±8.6 kg with spinal muscular atrophy—or myopathy—participated in this study. Patient characteristics for each of the two protocols are given in Table 1. The diagnoses were based on clinical and laboratory examinations including nerve conduction studies, electromyograms, and muscle biopsies. All the patients underwent one or two 30-min daily sessions of IPPV for several years. Volume values were
Baseline Values
The subjects displayed high levels of LBI and phase angle during spontaneous breathing (Fig 1). The %RC during spontaneous breathing was low in patients with SMA, and within normal limits in patients with myopathy. However, this and all the remaining differences between groups were nonsignificant.
Breathing Pattern During IPPV Without Binding
The %RC increased up to 58% in the SMA group, and 65% in the myopathy group. The thoracic volume rose to 400 mL in patients with SMA and 300 mL in patients with myopathy. During IPPV, LBI and phase
DISCUSSION
The purpose of this study was to quantitatively assess the abnormalities in the TAPB during spontaneous breathing or mechanical ventilation in subjects with SMA or myopathy. The first protocol reproduced their habitual IPPV session, whereas in the second protocol, IPPV pressure was manipulated. In fact, these two protocols yielded similar results. Accordingly, the observations of the second protocol may be generalized to the normal IPPV sessions with constant pressure. We observed that the
References (27)
- et al.
Bilateral diaphragmatic paralysis with hypercapnic respiratory failure
Am J Med
(1978) - et al.
Inhibition of respiratory activity during passive ventilation: a role for intercostal afferents?
Respir Physiol
(1993) Mechanisms for respiratory insufficiency after cervical cord injury: a source of alveolar hypoventilation
Ann Intern Med
(1961)- et al.
Motion of the rib cage and the abdomen in tetraplegic patients
Clin Sci Mol Med
(1978) - et al.
Mechanics of breathing in tetraplegics
Am Rev Respir Dis
(1979) - et al.
Respiratory mechanics in quadriplegia: the respiratory function of the intercostal muscle
Am Rev Respir Dis
(1980) - et al.
Relationship between respiratory muscle electromyogram and rib cage motion in tetraplegia
Am Rev Respir Dis
(1985) - et al.
Changes in lung volumes and rib cage configuration with abdominal binding in quadriplegia
J Appl Physiol
(1986) - et al.
Amyotrophie spinale infantile
Rev Neurol (Paris)
(1989) Chest wall motion before and during mechanical ventilation in children with neuromuscular disease
Pediatr Pulmonol
(1993)
Abdominal wall movement in normals and patients with hemidiaphragmatic and bilateral diaphragmatic palsy
Thorax
Atelectasis and neuromuscular respiratory failure
Chest
Les problèmes thoraciques, rachidiens et respiratoires de l'enfant atteint d'amyotrophie spinale infantile à évolution prolongée
Arch Fr Pediatr
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This work was supported by the Association Française contre les Myopathies.