Brainstem Dysfunction Involvement in the Pathogenesis of Pierre Robin Sequence

Status: Completed
Location: See all (2) locations...
Intervention Type: Other
Study Type: Interventional
Study Phase: Not Applicable
SUMMARY

Introduction Pierre Robin Sequence, PRS, incidence is about one hundred births per year in France. The main neonatal clinical manifestations are secondary to airway obstruction and food difficulties related to swallowing disorders. Despite recent progress, the pathogenesis of PRS is not fully understood. The hypothesis is that brainstem dysfunction, BSD, plays a central role in the pathogenesis of PRS. The purpose of the study is to achieve a complete evaluation of BSD to specify its role in the pathogenesis of PRS. The primary objective is to compare central apnea index (CAI) of infants with PRS with those of infants with isolated airway obstruction (AWO) and those of healthy infants in order to clarify the direct role of BSD. Material and Methods This prospective interventional study will be carried out in Lyon at the Hôpital Femme-Mère-Enfant and in Paris at the Hôpital Necker-Enfants Malades for 2 years. 3 groups of patients will be studied: PRS, 50 patients, AWO, 50 patients and healthy, 30 patients, included before 2 months of life. Infants will be followed for a maximum of 10 months. The evaluations will be carried out for 48 hours between birth and 2 months of life and then for 24 hours between 6 and 10 months of life for PRS and AWO group. Concerning the healthy group, the evaluation will be carried out during 48h during a single hospitalization before 2 months. Polysomnography, holter-ECG, 24h gas exchange, impedance-pH monitoring and mental region EEG will be performed. The central apnea index (mean number per hour), obstructive apnea index, non-nutritive swallowing index (NNS), gastroesophageal reflux and NNS-respiration coordination will be assessed for each stage of sleep and compared between the three groups of patients.

Eligibility
Participation Requirements
Sex: All
Minimum Age: 1 day
Maximum Age: 2 months
Healthy Volunteers: f
View:

⁃ \- infants with PRS

⁃ \- healthy infant : siblings of sudden unexpected death of the infant or with any known pathology

• During one hospitalization or program for one hospitalization

• Parental consent

• Social safety affiliation

Locations
Other Locations
France
Hopital Femme Mère Enfant
Bron
APHP-Necker
Paris
Time Frame
Start Date: 2018-05-24
Completion Date: 2023-03-23
Participants
Target number of participants: 113
Treatments
Other: Pierre Robin sequence
Infants with PRS : retrognathism, glossoptosis, cleft palate~Group 1a : isolated PRS Group 1b : PRS with bone disease or collagen disease (Stickler) Group 1c : syndromic PRS or associated PRS without bone disease or collagen disease
Other: Superior airway obstruction, AWO
Infants with AWO : laryngomalacia, tracheal stenosis, laryngeal stenosis, others etiology
Other: Healthy infants
Healthy infants : siblings of sudden unexpected death of the infant
Sponsors
Leads: Hospices Civils de Lyon

This content was sourced from clinicaltrials.gov

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