Respiratory Muscles and Work of Breathing in Children (WOB&MR_Ped)

Respiratory muscle testing allows a quantitative assessment of inspiratory and expiratory muscles in children of any age with primary or secondary respiratory muscle impairment, in order to better understand the pathophysiology of respiratory impairment and guide therapeutic management. The use of an invasive technique (esogastric probe) makes it possible to specifically explore the diaphragm, the accessory inspiratory muscles and the expiratory muscles in order to detect dysfunction or paralysis of these muscles, and to estimate the work of breathing in order to better guide the respiratory management. The primary objective of the study is to evaluate the respiratory effort in children with primary or secondary impairment of the respiratory muscles during spontaneous breathing or during mechanical ventilation.

Respiratory muscle testing allows a quantitative assessment of inspiratory and expiratory muscles in children of any age with primary or secondary respiratory muscle impairment, in order to better understand the pathophysiology of respiratory impairment and guide therapeutic management. The use of an invasive technique (esogastric probe) makes it possible to specifically explore the diaphragm, the accessory inspiratory muscles and the expiratory muscles in order to detect dysfunction or paralysis of these muscles, and to estimate the work of breathing in order to better guide the respiratory management. Respiratory muscle testing by means of esogastric measurements may allow assessing the effect of pharmacological treatment by comparing respiratory muscle strength before and after a few months with treatment. Moreover, esogastric measurements can be used to better adapt mechanical ventilation or to determine the possibility of weaning from the respiratory support. The primary objective of the study is to evaluate the respiratory effort in children with primary or secondary impairment of the respiratory muscles during spontaneous breathing or during mechanical ventilation.

Neuromuscular Diseases, Congenital Diaphragmatic Hernia, Lung Diseases, Cardiac Diseases, Scoliosis, Diaphragmatic Impairment, Respiratory Muscle Impairment

Respiratory muscle testing, Respiratory muscle strength, Work of breathing, Esogastric pressures, Diaphragmatic dysfunction, Diaphragmatic paralysis

Minor patients with primary or secondary impairment of respiratory muscles and followed at Necker Hospital

Measurement of work of breathing and respiratory muscles strength using an esogastric catheter. One measurement or before and after (6 months and 1 year) the initiation of a pharmacological treatment in order to assess the effect of the treatment on respiratory muscle function.

Total work of breathing (WOBt) is calculated (in Joules/L) as the area under the pressure-volume curve, using the Campbell diagram. Elastic (WOBe) and resistive (WOBr) are estimated as the 2/3 and 1/3 of WOBt value, respectively.

Vital capacity is the maximum amount of air a person can expel from the lungs after a maximum inhalation, using spirometry

Correlation between the pressure-time product, the work of breathing and the respiratory muscle strength with the disease severity

The pressure-time product of the respiratory muscles, the work of breathing and the respiratory muscle strength will be correlated with disease severity

Correlation between the pressure-time product, the work of breathing and the respiratory muscle strength with polysomnography and gas exchange results

The pressure-time product of the respiratory muscles, the work of breathing and the respiratory muscle strength will be correlated with the polysomnography and gas exchange results

The esophageal pressure-time product will be compared between patients with any treatment and patients receiving no treatment

The total work of breathing (WOBt), which is calculated (in Joules/L) as the area under the pressure-volume curve using the Campbell diagram, be compared between patients with any treatment and patients receiving no traeatment, using the Student t test

Measurement of esophageal pressure during a sniff in patients with any treatment and patients with no treatment

The esophageal pressure during a sniff will be compared between patients with any treatment and patients receiving no treatment

The esophageal pressure-time product will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation

The total work of breathing (WOBt), which is calculated (in Joules/L) as the area under the pressure-volume curve using the Campbell diagram, will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation, using the ANOVA on repeated measures

The esophageal pressure during a sniff will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation

The transdiaphragmatic pressure measurement during a sniff will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation

The total work of breathing (WOBt), which is calculated (in Joules/L) as the area under the pressure-volume curve using the Campbell diagram, will be measured to guide the respiratory support adaptation

Measurement of the esophageal pressure-time product in patients with weaning success and in patients with weaning failure

The esophageal pressure-time product will be compared between patients with weaning success and weaning failure

Measurement of the total work of breathing in patients with weaning success and in patients with weaning failure

The total work of breathing (WOBt), which is calculated (in Joules/L) as the area under the pressure-volume curve using the Campbell diagram, will be compared between patients with weaning success and weaning failure, using the Student t test

Measurement of the esophageal pressure during a sniff in patients with weaning success and in patients with weaning failure

The esophageal pressure during a sniff will be compared between patients with weaning success and weaning failure

Correlation between the pressure-time product, the work of breathing and the respiratory muscle strength with the gas exchange during weaning trial

The pressure-time product, the work of breathing and the respiratory muscle strength will be correlated with the gas exchange during a weaning trial

Inclusion Criteria: Patients aged less than 18 years old with primary or secondary impairment of respiratory muscles and followed at Necker Hospital Patients under spontaneous breathing or noninvasive or invasive mechanical ventilation Written informed consent Exclusion Criteria: No social insurance Significant psychomotor retardation Absence of cooperation Significant agitation Hemodynamic instability Acute condition or temporary drug treatments that may interfere with the results of the respiratory muscle explorations