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  • Title: Sleep disordered breathing in patients with Prader-Willi syndrome: A multicenter study.
    Author: 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.
    Journal: Pediatr Pulmonol; 2015 Dec; 50(12):1354-9. PubMed ID: 25851435.
    Abstract:
    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.
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