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Hypoventilation in patients with Prader-Willi syndrome across the pediatric age.
Chen, Catherine; Ioan, Iulia; Thieux, Marine; Nicolino, Marc; Franco, Patricia; Coutier, Laurianne.
Afiliación
  • Chen C; Service de pneumologie pédiatrique, Hôpital Femme Mère Enfant, Hospices, Civils de Lyon, Bron, France.
  • Ioan I; Université Claude-Bernard Lyon 1, Lyon, France.
  • Thieux M; Service d'explorations fonctionnelles pédiatriques, Hôpital d'Enfants, CHRU de Nancy; Unité DevAH, Université de Lorraine, Nancy, France.
  • Nicolino M; Université Claude-Bernard Lyon 1, Lyon, France.
  • Franco P; Service Épilepsie-Sommeil-Explorations, Fonctionnelles Neurologiques Pédiatriques, Hôpital Femme Mère Enfant, Hospices Civils de, Lyon, Bron, France.
  • Coutier L; Unité INSERM U1028 CNRS UMR 5292, Université Lyon 1, Lyon, France.
Pediatr Pulmonol ; 59(4): 938-948, 2024 Apr.
Article en En | MEDLINE | ID: mdl-38179881
ABSTRACT

OBJECTIVES:

Few data on alveolar hypoventilation in Prader-Willi syndrome (PWS) are available and the respiratory follow-up of these patients is not standardized. The objectives of this study were to evaluate the prevalence of alveolar hypoventilation in children with PWS and identify potential risk factors. STUDY

DESIGN:

This retrospective study included children with PWS recorded by polysomnography (PSG) with transcutaneous carbon dioxide pressure (PtcCO2) or end-tidal CO2 (ETCO2) measurements, between 2007 and 2021, in a tertiary hospital center. The primary outcome was the presence of alveolar hypoventilation defined as partial pressure of carbon dioxide (pCO2) ≥ 50 mmHg during ≥2% of total sleep time (TST) or more than five consecutive minutes.

RESULTS:

Among the 57 included children (38 boys, median age 4.8 years, range 0.1-15.6, 60% treated with growth hormone [GH], 37% obese), 19 (33%) had moderate-to-severe obstructive sleep apnea syndrome (defined as obstructive apnea-hypopnea index ≥5/h) and 20 (35%) had hypoventilation. The median (range) pCO2 max was 49 mmHg (38-69). Among the children with hypoventilation, 25% were asymptomatic. Median age and GH treatment were significantly higher in children with hypoventilation compared to those without. There was no significant difference in terms of sex, BMI, obstructive or central apnea-hypopnea index between both groups.

CONCLUSION:

The frequency of alveolar hypoventilation in children and adolescents with PWS is of concern and may increase with age and GH treatment. A regular screening by oximetry-capnography appears to be indicated whatever the sex, BMI, and rate of obstructive or central apneas.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndrome de Prader-Willi / Apnea Obstructiva del Sueño Tipo de estudio: Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Child / Child, preschool / Humans / Infant / Male Idioma: En Revista: Pediatr Pulmonol Asunto de la revista: PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Síndrome de Prader-Willi / Apnea Obstructiva del Sueño Tipo de estudio: Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Child / Child, preschool / Humans / Infant / Male Idioma: En Revista: Pediatr Pulmonol Asunto de la revista: PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Estados Unidos