Sleep disordered breathing in patients with Prader–Willi syndrome: A multicenter study. Issue 12 (7th April 2015)

• Record Type: Journal Article
• Authors: Pavone, Martino; Caldarelli, Valeria; Khirani, Sonia; Colella, Marina; Ramirez, Adriana; Aubertin, Guillaume; Crinò, Antonino; Brioude, Frédéric; Gastaud, Frédérique; Beydon, Nicole; Boulé, Michèle; Giovannini‐Chami, Lisa; Cutrera, Renato; Fauroux, Brigitte
• Is Part Of: Pediatric pulmonology. Volume 50:Issue 12(2015)
• Page Start: 1354
• Page End: 1359
• Publication Date: 2015-04-07
• Subjects: Prader–Willi syndrome -- sleep disordered breathing -- polygraphy -- growth hormone treatment -- noninvasive ventilation -- upper airway surgery; Pediatric respiratory diseases -- Periodicals; Pediatrics -- Periodicals; 618.922
• DOI: 10.1002/ppul.23177
• Languages: English
• ISSNs: 8755-6863

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/m 2, 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 SpO2 <90% 0.1 [0.0–1.0]%, and oxygen desaturation index 2 [1–4]/hr. An apnea–hypopnea index (AHI) ≥1.5 and ≥5 events/hr was observed in 53% of children and 41% of adults, respectively. No correlations were observed between MOAHI and anthropometrics data (age, BMI, BMI z‐score), while MOAHI significantly correlated with SpO2 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. © 2015 Wiley Periodicals, Inc.