Sleep disordered breathing in patients with Prader-Willi syndrome: A multicenter study

Martino Pavone, Valeria Caldarelli, Sonia Khirani, Marina Colella, Adriana Ramirez, Guillaume Aubertin, Antonino Crinò, Frédéric Brioude, Frédérique Gastaud, Nicole Beydon, Michèle Boulé, Lisa Giovannini-Chami, Renato Cutrera, Brigitte Fauroux

Research output: Contribution to journalArticle

Abstract

Summary Objectives Sleep disordered breathing (SDB) is common in patients with Prader-Willi syndrome (PWS) and systematic screening is recommended, especially before growth hormone treatment. The aim of the study was to describe the baseline SDB and therapeutic interventions in a large cohort of patients. Study design Retrospective study. Subject selection Eighty-eight patients with PWS, median [interquartile range] age of 5.1 [1.0-14.5] years old (range 0.3-44.3), who were followed in three centers (France, Italy). Methodology Anthropometrics, polygraphy (PG), and gas exchange data were analyzed. Results Median body mass index (BMI) was 20 [16-34] kg/m2, BMI z-score for patients aged 2-20 years old was 2.1 [1.2-2.8] SD, mixed-obstructive apnea-hypopnea index (MOAHI) 1.8 [0.6-5.0] events/hr, and central apnea index (CAI) 0.1 [0.0-0.6] events/hr. Minimum pulse oximetry (SpO2) was 88 [84-91]%, percentage of time with SpO22 indexes. Age and BMI only weakly correlated with SpO2 indexes. Growth hormone could be initiated in 48 patients. Regarding post-PG therapy, 9 patients had upper airway surgery, and noninvasive CPAP/bilevel ventilation was started in 16 patients. Conclusions Patients with PWS exhibit a high prevalence of SDB. The lack of association between obesity and SDB leads to hypothesize that hypotonia and/or facial dysmorphic features may play a major role in the occurrence of SDB. Pediatr Pulmonol. 2015; 50:1354-1359.

Original languageEnglish
Pages (from-to)1354-1359
Number of pages6
JournalPediatric Pulmonology
Volume50
Issue number12
DOIs
Publication statusPublished - Dec 1 2015

Keywords

  • growth hormone treatment
  • noninvasive ventilation
  • polygraphy
  • Prader-Willi syndrome
  • sleep disordered breathing
  • upper airway surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

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    Pavone, M., Caldarelli, V., Khirani, S., Colella, M., Ramirez, A., Aubertin, G., Crinò, A., Brioude, F., Gastaud, F., Beydon, N., Boulé, M., Giovannini-Chami, L., Cutrera, R., & Fauroux, B. (2015). Sleep disordered breathing in patients with Prader-Willi syndrome: A multicenter study. Pediatric Pulmonology, 50(12), 1354-1359. https://doi.org/10.1002/ppul.23177