title: Sleep disordered breathing in patients with Prader-Willi syndrome: A multicenter study.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  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).
  
  
  METHODS
  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.
authors: Respiratory Unit, Bambino Ges, ù, Children's Hospital, Rome, Italy.
authors: Martino
authors: M
authors: Pavone
authors: Respiratory Unit, Bambino Ges, ù, Children's Hospital, Rome, Italy.|Pediatric Unit, IRCCS-Arcispedale Santa Maria Nuova, Reggio Emilia, Italy.
authors: Valeria
authors: V
authors: Caldarelli
authors: Pediatric Noninvasive Ventilation and Sleep Unit, AP-HP, H, ô, pital Necker, Paris, France.|ASV Santé, Gennevilliers, France.
authors: Sonia
authors: S
authors: Khirani
authors: Clinica Pediatrica, Istituto G. Gaslini, Genoa, Italy.
authors: Marina
authors: M
authors: Colella
authors: Pediatric Noninvasive Ventilation and Sleep Unit, AP-HP, H, ô, pital Necker, Paris, France.|ADEP Assistance, Suresnes, France.
authors: Adriana
authors: A
authors: Ramirez
authors: Pediatric Pulmonary Department, AP-HP, Hôpital Armand Trousseau, Paris, France.
authors: Guillaume
authors: G
authors: Aubertin
authors: Endocrinology Unit, Bambino Gesù Children's Hospital, Rome, Italy.
authors: Antonino
authors: A
authors: Crinò
authors: Endocrinology Unit, AP-HP, Hôpital Armand Trousseau, Paris, France.
authors: Frédéric
authors: F
authors: Brioude
authors: Endocrinology Unit, Hôpitaux pédiatriques de Nice CHU-Lenval, Nice, France.
authors: Frédérique
authors: F
authors: Gastaud
authors: Lung Function and Sleep Unit, Physiology Department, AP-HP, Hôpital Armand Trousseau, Paris, France.
authors: Nicole
authors: N
authors: Beydon
authors: Lung Function and Sleep Unit, Physiology Department, AP-HP, Hôpital Armand Trousseau, Paris, France.|Pierre et Marie Curie University - Paris 6, Paris, France.
authors: Michèle
authors: M
authors: Boulé
authors: Pediatric Pulmonary-Allergy Department, Hôpitaux pédiatriques de Nice CHU-Lenval, Nice, France.|Université de Nice Sophia Antipolis, Nice, France.
authors: Lisa
authors: L
authors: Giovannini-Chami
authors: Respiratory Unit, Bambino Ges, ù, Children's Hospital, Rome, Italy.
authors: Renato
authors: R
authors: Cutrera
authors: Pediatric Noninvasive Ventilation and Sleep Unit, AP-HP, H, ô, pital Necker, Paris, France.|Paris Descartes University, Paris, France.|INSERM U 955, Equipe 13, Créteil, France.
authors: Brigitte
authors: B
authors: Fauroux
mesh_terms: D000293:Adolescent; D000367:Age Factors; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010092:Oximetry; D010100:Oxygen / Q000097:blood; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D012891:Sleep Apnea Syndromes / Q000175:diagnosis*; D055815:Young Adult
chemical_list: D010100:Oxygen
keywords: Prader-Willi syndrome; growth hormone treatment; noninvasive ventilation; polygraphy; sleep disordered breathing; upper airway surgery