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| Name | Class |
|---|---|
| German Federal Ministry of Education and Research | OTHER_GOV |
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In a longitudinal cohort study, we want to assess the effect of the pre-epiglottic baton plate, the main treatment approach currently used in Germany for Pierre-Robin-Like phenotype, on upper airway obstruction and failure to thrive using objective criteria (mixed-obstructive apnea index assessed by polysomnography, standard deviation score for body weight) upon admission and 3 months after hospital discharge.
Pierre-Robin sequence consists of a small lower jaw, a retropositioned tongue (glossoptosis), and optionally cleft palate, leading to severe upper airway obstruction and failure to thrive; occasionally even to sudden infant death. Treatment approaches are heterogeneous, some are considerably invasive; few have been evaluated by objective tests.
In a longitudinal cohort study, we want to assess the effect of the pre-epiglottic baton plate on upper airway obstruction and failure to thrive using objective criteria (mixed-obstructive apnea index assessed by polysomnography, standard deviation score for body weight) upon admission and 3 months after hospital discharge. The pre-epiglottic baton plate is the main treatment approach currently used in Germany for this condition and practised standard care in the three participating study centers.
These data will help to compare the effect of the main treatment currently used in Germany with international data on more invasive treatments used in this rare condition.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Pre-epiglottic baton plate (PEBP) | Device | modified acrylic palatal plate in which a velar extension resembling a spur or baton shifts the base of the tongue forward. This treatment is the standard care for infants with Pierre-Robin-Like Phenotype in the participating study centers and not assigned by protocol. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in mixed-obstructive apnea index (MOAI) | Change in mixed-obstructive apnea index (MOAI) assessed by polygraphy from pre- to post-intervention as a marker of Pierre-Robin-sequence-related breathing disorder | upon admission within the first year of life (before start of treatment) and 3 months after hospital discharge |
| Measure | Description | Time Frame |
|---|---|---|
| Change in standard deviation score for body weight | Body weight will be obtained by electronic scales and the standard deviation score for weight calculated | upon admission within the first year of life (before start of treatment) and 3 months after hospital discharge |
| Duration of hospital stay |
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Inclusion Criteria:
Exclusion Criteria:
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Infants <1 year admitted with Pierre-Robin-like phenotype to participating centres
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| Name | Affiliation | Role |
|---|---|---|
| Christian F Poets, MD | Dept. of Neonatology and Interdisciplinary Centre forCraniofacial Malformations, University Hospital Tuebingen | Principal Investigator |
| Siegmar Reinert, MD, PhD | Dept. of Craniofacial Surgery, University Hospital Tuebingen | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Centre of Competence for Oro- and Craniofacial Malformations and Department of Neonatology, Univ. Hospital of Cologne | Cologne | 50924 | Germany |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 28274235 | Derived | Poets CF, Maas C, Buchenau W, Arand J, Vierzig A, Braumann B, Muller-Hagedorn S. Multicenter study on the effectiveness of the pre-epiglottic baton plate for airway obstruction and feeding problems in Robin sequence. Orphanet J Rare Dis. 2017 Mar 9;12(1):46. doi: 10.1186/s13023-017-0602-8. |
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| ID | Term |
|---|---|
| D010855 | Pierre Robin Syndrome |
| D000402 | Airway Obstruction |
| ID | Term |
|---|---|
| D007569 | Jaw Abnormalities |
| D007571 | Jaw Diseases |
| D009140 | Musculoskeletal Diseases |
| D019767 | Maxillofacial Abnormalities |
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| admission within the first year of life for initiation of study intervention |
| Treatment failure | 3 months after admission for initiation of study intervention |
| Dept. of Neonatology and Interdisciplinary Centre for Craniofacial Malformations, University Hospital Tuebingen | Tübingen | 72076 | Germany |
| Dept. of Orthodontics and University Children's Hospital, University Hospital of Wuerzburg | Würzburg | 97080 | Germany |
| D019465 |
| Craniofacial Abnormalities |
| D009139 | Musculoskeletal Abnormalities |
| D009057 | Stomatognathic Diseases |
| D018640 | Stomatognathic System Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D012131 | Respiratory Insufficiency |
| D012120 | Respiration Disorders |
| D012140 | Respiratory Tract Diseases |