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Developmental outcome of children with Robin sequence treated with the current Paris protocol.
Fleurance, Alix; Leunen, Dorothée; Végas, Nancy; Soupre, Véronique; Griffon, Lucie; Adnot, Pauline; Malecot, Gaelle; Luscan, Romain; Amiel, Jeanne; Fauroux, Brigitte; Abadie, Véronique.
Afiliación
  • Fleurance A; General Paediatrics Unit, Necker University Hospital, Paris, France.
  • Leunen D; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Végas N; Pedopsychiatric Unit, Necker University Hospital, Paris, France.
  • Soupre V; General Paediatrics Unit, Necker University Hospital, Paris, France.
  • Griffon L; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Adnot P; Malformation and Embryology Lab, IMAGINE Institute, Paris, France.
  • Malecot G; Paris Cité University, Paris, France.
  • Luscan R; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Amiel J; Maxillo-Facial and Plastic Surgery Unit, Necker University Hospital, Paris, France.
  • Fauroux B; Reference Centre for Rare Disease-Centre de Référence Maladies Rares «Syndromes de Pierre Robin et troubles de succion-déglutition congénitaux¼, Paris, France.
  • Abadie V; Sleep and Non-Invasive Ventilation Unit, Paris, France.
Acta Paediatr ; 112(12): 2601-2610, 2023 12.
Article en En | MEDLINE | ID: mdl-37786287
ABSTRACT

AIM:

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.

METHODS:

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.

RESULTS:

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.

CONCLUSION:

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.
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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Síndrome de Pierre Robin / Pérdida Auditiva Sensorineural Límite: Child / Female / Humans / Infant País/Región como asunto: Europa Idioma: En Revista: Acta Paediatr Año: 2023 Tipo del documento: Article País de afiliación: Francia

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Síndrome de Pierre Robin / Pérdida Auditiva Sensorineural Límite: Child / Female / Humans / Infant País/Región como asunto: Europa Idioma: En Revista: Acta Paediatr Año: 2023 Tipo del documento: Article País de afiliación: Francia