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Sleep disordered breathing in patients with Prader–Willi syndrome: A multicenter study
Author(s) -
Pavone Martino,
Caldarelli Valeria,
Khirani Sonia,
Colella Marina,
Ramirez Adriana,
Aubertin Guillaume,
Crinò Antonino,
Brioude Frédéric,
Gastaud Frédérique,
Beydon Nicole,
Boulé Michèle,
GiovanniniChami Lisa,
Cutrera Renato,
Fauroux Brigitte
Publication year - 2015
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.23177
Subject(s) - medicine , interquartile range , body mass index , obstructive sleep apnea , continuous positive airway pressure , polysomnography , apnea–hypopnea index , apnea , pulse oximetry , pediatrics , anesthesia
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 (SpO 2 ) was 88 [84–91]%, percentage of time with SpO 2 <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 SpO 2 indexes. Age and BMI only weakly correlated with SpO 2 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.