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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.
Affiliation
  • 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 in En | MEDLINE | ID: mdl-37786287
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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Full text: 1 Database: MEDLINE Main subject: Pierre Robin Syndrome / Hearing Loss, Sensorineural Limits: Child / Female / Humans / Infant Country/Region as subject: Europa Language: En Year: 2023 Type: Article

Full text: 1 Database: MEDLINE Main subject: Pierre Robin Syndrome / Hearing Loss, Sensorineural Limits: Child / Female / Humans / Infant Country/Region as subject: Europa Language: En Year: 2023 Type: Article