title: Upper airway lymphoid tissue size in children with sickle cell disease.
abstract: BACKGROUND
  The prevalence of obstructive sleep apnea syndrome (OSAS) is higher in children with sickle cell disease (SCD) as compared with the general pediatric population. It has been speculated that overgrowth of the adenoid and tonsils is an important contributor.
  
  
  METHODS
  The current study used MRI to evaluate such an association. We studied 36 subjects with SCD (aged 6.9 ± 4.3 years) and 36 control subjects (aged 6.6 ± 3.4 years).
  
  
  RESULTS
  Compared with control subjects, children with SCD had a significantly smaller upper airway (2.8 ± 1.2 cm(3) vs 3.7 ± 1.6 cm(3), P < .01), and significantly larger adenoid (8.4 ± 4.1 cm(3) vs 6.0 ± 2.2 cm(3), P < .01), tonsils (7.0 ± 4.3 cm(3) vs 5.1 ± 1.9 cm(3), P < .01), retropharyngeal nodes (3.0 ± 1.9 cm(3) vs 2.2 ± 0.9 cm(3), P < .05), and deep cervical nodes (15.7 ± 5.7 cm(3) vs 12.7 ± 4.0 cm(3), P < .05). Polysomnography showed that 19.4% (seven of 36) of children with SCD had OSAS compared with 0% (zero of 20) of control subjects (P < .05) and that in children with SCD the apnea-hypopnea index correlated positively with upper airway lymphoid tissues size (r = 0.57, P < 001). In addition, children with SCD had lower arterial oxygen saturation nadir (84.3% ± 12.3% vs 91.2% ± 4.2%, P < .05), increased peak end-tidal CO(2) (53.4 ± 8.5 mm Hg vs 42.3 ± 5.3 mm Hg, P < .001), and increased arousals (13.7 ± 4.7 events/h vs 10.8 ± 3.8 events/h, P < .05).
  
  
  CONCLUSIONS
  Children with SCD have reduced upper airway size due to overgrowth of the surrounding lymphoid tissues, which may explain their predisposition to OSAS.
authors.affiliation: Division of Respiratory and Sleep Medicine, The Children's Hospital at Montefiore and Albert Einstein College of Medicine, Bronx, NY.
authors.forename: Temima
authors.initials: T
authors.lastname: Strauss
authors.affiliation: Division of Respiratory and Sleep Medicine, The Children's Hospital at Montefiore and Albert Einstein College of Medicine, Bronx, NY.
authors.forename: Sanghun
authors.initials: S
authors.lastname: Sin
authors.affiliation: Sleep Center, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Carole L
authors.initials: CL
authors.lastname: Marcus
authors.affiliation: Sleep Center, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Thornton B A
authors.initials: TBA
authors.lastname: Mason
authors.affiliation: Division of Pulmonary Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: McDonough
authors.affiliation: Division of Pulmonary Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Julian L
authors.initials: JL
authors.lastname: Allen
authors.affiliation: Division of Pulmonary Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Jason B
authors.initials: JB
authors.lastname: Caboot
authors.affiliation: Division of Hematology, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Cheryl Y
authors.initials: CY
authors.lastname: Bowdre
authors.affiliation: Division of Pediatrics, The Children's Hospital of Philadelphia, and the Center for Sleep, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Abbas F
authors.initials: AF
authors.lastname: Jawad
authors.affiliation: Division of Hematology, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Kim
authors.initials: K
authors.lastname: Smith-Whitley
authors.affiliation: Division of Hematology, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Kwaku
authors.initials: K
authors.lastname: Ohene-Frempong
authors.affiliation: Circadian Neurobiology, University of Pennsylvania School of Medicine, Philadelphia, PA.
authors.forename: Allan I
authors.initials: AI
authors.lastname: Pack
authors.affiliation: Division of Respiratory and Sleep Medicine, The Children's Hospital at Montefiore and Albert Einstein College of Medicine, Bronx, NY. Electronic address: rarens@montefiore.org.
authors.forename: Raanan
authors.initials: R
authors.lastname: Arens
mesh_terms: D000234:Adenoids / Q000473:pathology*; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications / Q000473:pathology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008221:Lymphoid Tissue / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D014066:Palatine Tonsil / Q000473:pathology*; D017286:Polysomnography; D015995:Prevalence; D020181:Sleep Apnea, Obstructive / Q000175:diagnosis / Q000453:epidemiology