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.

Study Overview

• Status: Recruiting
• Conditions: Lung Diseases; Neuromuscular Diseases; Scoliosis; Congenital Diaphragmatic Hernia; Cardiac Diseases; Diaphragmatic Impairment; Respiratory Muscle Impairment
• Intervention / Treatment: Other: Esogastric pressure measurement

Detailed Description

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.

• Study Type: Interventional
• Enrollment (Estimated): 550
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Hélène Morel; Phone Number: +33 1 71 19 63 46; Email: helene.morel@aphp.fr
• Study Contact Backup: Name: Brigitte Fauroux, MD, PhD; Phone Number: +33 1 71 19 60 92; Email: brigitte.fauroux@aphp.fr

Study Locations

• France
  • Paris, France, 75015
    • Recruiting
    • Hôpital Necker-Enfants Malades
    • Contact: Brigitte Fauroux, MD, PhD; Phone Number: +33 1 71 19 60 92; Email: brigitte.fauroux@aphp.fr
    • Sub-Investigator: Lucie Griffon, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 1 second to 17 years (Child)
• Accepts Healthy Volunteers: No

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Diagnostic
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Respiratory muscle impairment; Minor patients with primary or secondary impairment of respiratory muscles and followed at Necker Hospital	Other: Esogastric pressure measurement; 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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Esophageal pressure-time product (PTPoes)	The integral of the esophageal pressure signal over inspiratory time	Day 0
Diaphragmatic pressure-time product (PTPdi)	The integral of the transdiaphragmatic pressure signal over inspiratory time	Day 0
Work of breathing (WOB)	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.	Day 0

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Vital capacity	Vital capacity is the maximum amount of air a person can expel from the lungs after a maximum inhalation, using spirometry	Day 0
Maximal respiratory static pressures	Maximal static inspiratory and expiratory airway pressures	Day 0
Maximal sniff pressure	Airway pressure during a sniff test	Day 0
Maximal whistle pressure	Airway pressure during a whistle test	Day 0
Peak expiratory flow	Maximal flow during expiratory maneuver	Day 0
Peak cough flow	Maximal flow during cough	Day 0
Esophageal pressure during sniff	Esophageal pressure measurement during a sniff test	Day 0
Gastric pressure during sniff	Gastric pressure measurement during a sniff test	Day 0
Transdiaphragmatic pressure during sniff	Transdiaphragmatic pressure measurement during a sniff test	Day 0
Crying transdiaphragmatic pressure	Transdiaphragmatic pressure measurement during crying in young children	Day 0
Gastric pressure during cough	Gastric pressure measurement during a maximal cough maneuver	Day 0
Twitch transdiaphragmatic pressure	Transdiaphragmatic pressure measurement during cervical magnetic stimulation	Day 0
Inspiratory muscles tension-time index	Measurement of the tension-time index of the inspiratory muscles	Day 0
Diaphragmatic tension-time index	Measurement of the tension-time index of the diaphragm	Day 0
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	Day 0
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	Day 0
Esophageal pressure-time products in patients with any treatment and in patients with no treatment	The esophageal pressure-time product will be compared between patients with any treatment and patients receiving no treatment	Day 0
The total work of breathing in patients with any treatment and in patients with 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	Day 0
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	Day 0
Measurement of the esophageal pressure-time product before and after pharmacological treatment	The esophageal pressure-time product will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation	6 months and 1 year after treatment initiation
The total work of breathing before and after pharmacological 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, will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation, using the ANOVA on repeated measures	6 months and 1 year after treatment initiation
Measurement of the esophageal pressure during a sniff before and after pharmacological treatment	The esophageal pressure during a sniff will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation	6 months and 1 year after treatment initiation
Measurement of transdiaphragmatic pressure during a sniff before and after pharmacological treatment	The transdiaphragmatic pressure measurement during a sniff will be compared before treatment, and 6 months and 1 year after pharmacological treatment initiation	6 months and 1 year after treatment initiation
Adaptation of the respiratory support according to the esophageal pressure-time product values	The esophageal pressure-time product will be measured to guide the respiratory support adaptation	Day 0
Adaptation of the respiratory support according to the total work of breathing values	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	Day 0
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	Day 0
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	Day 0
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	Day 0
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	Day 0

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Collaborators: URC-CIC Paris Descartes Necker Cochin
• Investigators: Principal Investigator: Brigitte Fauroux, MD, PhD, Assistance Publique - Hôpitaux de Paris; Study Director: Sonia Khirani, PhD, Assistance Publique - Hôpitaux de Paris

Study record dates

Study Major Dates

• Study Start (Actual): January 19, 2022
• Primary Completion (Estimated): January 1, 2028
• Study Completion (Estimated): January 1, 2028

Study Registration Dates

• First Submitted: June 14, 2021
• First Submitted That Met QC Criteria: September 9, 2021
• First Posted (Actual): September 21, 2021

Study Record Updates

• Last Update Posted (Actual): April 3, 2026
• Last Update Submitted That Met QC Criteria: March 30, 2026
• Last Verified: March 1, 2026

More Information

Terms related to this study

• Keywords: Respiratory muscle strength; Work of breathing; Diaphragmatic dysfunction; Diaphragmatic paralysis; Respiratory muscle testing; Esogastric pressures
• Additional Relevant MeSH Terms: Internal Hernia; Neurologic Manifestations; Bone Diseases; Musculoskeletal Diseases; Nervous System Diseases; Cardiovascular Diseases; Pathological Conditions, Anatomical; Respiratory Tract Diseases; Respiration Disorders; Spinal Diseases; Congenital Abnormalities; Hernia; Hernia, Diaphragmatic; Respiratory Insufficiency; Paralysis; Spinal Curvatures; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathological Conditions, Signs and Symptoms; Signs and Symptoms; Lung Diseases; Heart Diseases; Hernias, Diaphragmatic, Congenital; Scoliosis; Neuromuscular Diseases; Respiratory Paralysis
• Other Study ID Numbers: APHP210261; ID-RCB 2020-A03393-36 (Other Identifier: ID RCB Number)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No