title: Obstructive sleep apnea and sickle cell anemia.
abstract: OBJECTIVE
  To ascertain the prevalence of and risk factors for obstructive sleep apnea syndrome (OSAS) in children with sickle cell anemia (SCA).
  
  
  METHODS
  Cross-sectional baseline data were analyzed from the Sleep and Asthma Cohort Study, a multicenter prospective study designed to evaluate the contribution of sleep and breathing abnormalities to SCA-related morbidity in children ages 4 to 18 years, unselected for OSAS symptoms or asthma. Multivariable logistic regression assessed the relationships between OSAS status on the basis of overnight in-laboratory polysomnography and putative risk factors obtained from questionnaires and direct measurements.
  
  
  RESULTS
  Participants included 243 children with a median age of 10 years; 50% were boys, 99% were of African heritage, and 95% were homozygous for β(S) hemoglobin. OSAS, defined by obstructive apnea hypopnea indices, was present in 100 (41%) or 25 (10%) children at cutpoints of ≥1 or ≥5, respectively. In univariate analyses, OSAS was associated with higher levels of habitual snoring, lower waking pulse oxygen saturation (Spo2), reduced lung function, less caretaker education, and non-preterm birth. Lower sleep-related Spo2 metrics were also associated with higher obstructive apnea hypopnea indices. In multivariable analyses, habitual snoring and lower waking Spo2 remained risk factors for OSAS in children with SCA.
  
  
  CONCLUSIONS
  The prevalence of OSAS in children with SCA is higher than in the general pediatric population. Habitual snoring and lower waking Spo2 values, data easily obtained in routine care, were the strongest OSAS risk factors. Because OSAS is a treatable condition with adverse health outcomes, greater efforts are needed to screen, diagnose, and treat OSAS in this high-risk, vulnerable population.
authors.affiliation: Department of Pediatrics and Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio; carol.rosen@case.edu.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Rosen
authors.affiliation: Vanderbilt University School of Medicine and Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee;
authors.forename: Michael R
authors.initials: MR
authors.lastname: Debaun
authors.affiliation: Division of Allergy, Immunology, and Pulmonary Medicine, Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri;
authors.forename: Robert C
authors.initials: RC
authors.lastname: Strunk
authors.affiliation: Department of Medicine, Brigham and Women's Hospital and Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts;
authors.forename: Susan
authors.initials: S
authors.lastname: Redline
authors.affiliation: Heart and Vascular Institute, Cleveland Clinic Foundation, Cleveland, Ohio;
authors.forename: Sinziana
authors.initials: S
authors.lastname: Seicean
authors.affiliation: Department of Pediatrics and Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio;
authors.forename: Daniel I
authors.initials: DI
authors.lastname: Craven
authors.affiliation: Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital, London, United Kingdom;
authors.forename: Johanna C D
authors.initials: JC
authors.lastname: Gavlak
authors.affiliation: North Middlesex Hospital National Health Service Trust, London, United Kingdom;
authors.forename: Olu
authors.initials: O
authors.lastname: Wilkey
authors.affiliation: Evelina Children's Hospital, Guy's and St Thomas Hospital, London, United Kingdom;
authors.forename: Baba
authors.initials: B
authors.lastname: Inusa
authors.affiliation: Department of Paediatrics, Imperial College and Imperial College Healthcare National Health Service Trust, London, United Kingdom;
authors.forename: Irene
authors.initials: I
authors.lastname: Roberts
authors.affiliation: Vanderbilt University School of Medicine and Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee;
authors.forename: R Lucas
authors.initials: RL
authors.lastname: Goodpaster
authors.affiliation: Vanderbilt University School of Medicine and Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, Tennessee;
authors.forename: Beth
authors.initials: B
authors.lastname: Malow
authors.affiliation: Independent statistician, Chicago, Illinois; and.
authors.forename: Mark
authors.initials: M
authors.lastname: Rodeghier
authors.affiliation: University College London Institute of Child Health, London, United Kingdom.
authors.forename: Fenella J
authors.initials: FJ
authors.lastname: Kirkham
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000453:epidemiology* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D008297:Male; D015999:Multivariate Analysis; D010092:Oximetry; D015995:Prevalence; D012307:Risk Factors; D020181:Sleep Apnea, Obstructive / Q000453:epidemiology* / Q000503:physiopathology; D055815:Young Adult
keywords: blood disorders; cohort study; epidemiology; obstructive sleep apnea; polysomnography; sickle cell anemia; sleep disorders; sleep medicine