Developmental outcome of children with Robin sequence treated with the current Paris protocol.

Robin sequence continuous positive airway pressure psychomotor development stickler syndrome upper airway obstruction

Journal

Acta paediatrica (Oslo, Norway : 1992)
ISSN: 1651-2227
Titre abrégé: Acta Paediatr
Pays: Norway
ID NLM: 9205968

Informations de publication

Date de publication:
12 2023
Historique:
revised: 13 09 2023
received: 27 04 2023
accepted: 15 09 2023
medline: 9 11 2023
pubmed: 3 10 2023
entrez: 3 10 2023
Statut: ppublish

Résumé

We aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release upper airway obstruction. We included children with isolated RS or RS associated with Stickler syndrome who were aged 15 months to 6 years. We used the French version of the Child Development Inventory and calculated the developmental quotient (DQ) for eight different domains and the global DQ (DQ-global). We searched for determinants of risk of delay. Of the 87 children, for 71%, the developmental evolution was within the norm (DQ-global ≥86 or ≥-1 SD), 29% were at high risk of delay (DQ-global <86 or <-1 SD), and only 3% were at very high risk of delay (DQ-global <70 or <-2 SD). The DQs for expressive language and language comprehension were lower in our study population than the general population, but an improvement was noticed with the children's growth. Risk of a developmental delay was not greater for children with the most severe respiratory phenotype than the others. Children whose mothers had low education levels were more at risk than the others.

Identifiants

pubmed: 37786287
doi: 10.1111/apa.16979
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

2601-2610

Informations de copyright

© 2023 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.

Références

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Auteurs

Alix Fleurance (A)

General Paediatrics Unit, Necker University Hospital, Paris, France.
Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.

Dorothée Leunen (D)

Pedopsychiatric Unit, Necker University Hospital, Paris, France.

Nancy Végas (N)

General Paediatrics Unit, Necker University Hospital, Paris, France.
Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Malformation and Embryology Lab, IMAGINE Institute, Paris, France.
Paris Cité University, Paris, France.

Véronique Soupre (V)

Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Maxillo-Facial and Plastic Surgery Unit, Necker University Hospital, Paris, France.

Lucie Griffon (L)

Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Sleep and Non-Invasive Ventilation Unit, Paris, France.

Pauline Adnot (P)

General Paediatrics Unit, Necker University Hospital, Paris, France.
Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Paris Cité University, Paris, France.

Gaelle Malecot (G)

General Paediatrics Unit, Necker University Hospital, Paris, France.
Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.

Romain Luscan (R)

Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Ear Nose and Throat Unit, Paris, France.

Jeanne Amiel (J)

Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Malformation and Embryology Lab, IMAGINE Institute, Paris, France.
Paris Cité University, Paris, France.
Medical Genetics Department, Necker University Hospital, Paris, France.

Brigitte Fauroux (B)

Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Paris Cité University, Paris, France.
Sleep and Non-Invasive Ventilation Unit, Paris, France.

Véronique Abadie (V)

General Paediatrics Unit, Necker University Hospital, Paris, France.
Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux», Paris, France.
Paris Cité University, Paris, France.

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