title: Airway hyperreactivity is frequent in non-asthmatic children with sickle cell disease.
abstract: Asthma is associated with poorer outcomes in sickle cell disease (SCD). Whether AHR can exist in SCD as a distinct entity, separate and independent of asthma, is unknown.
  
  
  
  Our goal was to elucidate the prevalence of AHR, as measured by a methacholine challenge test (MCT), in children with SCD who did not have concomitant asthma or any recent history of acute chest syndrome (ACS). To determine if AHR was associated with asthma-like symptoms, we compared the results of the MCT to a validated asthma questionnaire. We also examined if a correlation between AHR and inflammatory markers exists.
  
  
  
  AHR was identified with a positive MCT defined as a provocation concentration (PC20 ) < 4 mg/ml. The children and/or their parents completed the ISAAC (International Study of Asthma and Allergies in Children) questionnaire. We obtained blood, urine, and exhaled breath condensate samples. We measured cysteinyl leukotriene levels in urine and exhaled breath condensate via enzyme immunoassay.
  
  
  
  Twenty-nine of forty children (72.5%) had a positive MCT. Nine (31.0%) also reported asthma-like symptoms on questionnaire. Inflammatory markers did not correlate with AHR. Among MCT positive subjects, those on hydroxyurea had significantly less severe AHR as quantified by PC20 (P = 0.014).
  
  
  
  In children with SCD, there is a high prevalence of AHR that is not associated with asthma-like symptoms. AHR may be a distinct entity in children with SCD, existing in the absence of concomitant asthma. Hydroxyurea therapy might lessen the severity of AHR in affected individuals. Pediatr Pulmonol. 2016; 51:950-957. © 2015 Wiley Periodicals, Inc.
authors.affiliation: Division of Respiratory Medicine, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Natalie R
authors.initials: NR
authors.lastname: Shilo
authors.affiliation: Division of Respiratory Medicine, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Aceel
authors.initials: A
authors.lastname: Alawadi
authors.affiliation: Division of Respiratory Medicine, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Allard-Coutu
authors.affiliation: Division of Hematology-Oncology, Department of Pediatrics, CHU Sainte-Justine, Montreal, QC, Canada.
authors.forename: Nancy
authors.initials: N
authors.lastname: Robitaille
authors.affiliation: Division of Hematology-Oncology, Department of Pediatrics, CHU Sainte-Justine, Montreal, QC, Canada.
authors.forename: Yves
authors.initials: Y
authors.lastname: Pastore
authors.affiliation: Division of Pulmonology, Department of Pediatrics, CHU Sainte-Justine, Montreal, QC, Canada.
authors.forename: Denis
authors.initials: D
authors.lastname: Bérubé
authors.affiliation: Division of Pulmonology, Department of Pediatrics, CHU Sainte-Justine, Montreal, QC, Canada.
authors.forename: Sheila V
authors.initials: SV
authors.lastname: Jacob
authors.affiliation: Division of Hematology-Oncology, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Sharon
authors.initials: S
authors.lastname: Abish
authors.affiliation: Division of Respiratory Medicine, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.|Research Institute, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Nurlan
authors.initials: N
authors.lastname: Dauletbaev
authors.affiliation: Division of Respiratory Medicine, Department of Pediatrics, The Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.|Research Institute, McGill University Health Centre, Montreal, QC, Canada.
authors.forename: Larry C
authors.initials: LC
authors.lastname: Lands
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000378:metabolism; D016535:Bronchial Hyperreactivity / Q000150:complications* / Q000175:diagnosis* / Q000378:metabolism; D001985:Bronchial Provocation Tests / Q000379:methods; D002648:Child; D003545:Cysteine / Q000378:metabolism; D005260:Female; D006801:Humans; D015289:Leukotrienes / Q000378:metabolism; D008297:Male; D016210:Methacholine Chloride; D011446:Prospective Studies
chemical_list: D015289:Leukotrienes; C112381:cysteinyl-leukotriene; D016210:Methacholine Chloride; D003545:Cysteine
keywords: acute chest syndrome; cysteinyl leukotrienes; exhaled breath condensate; hydroxyurea; methacholine challenge test; provocation concentration at which FEV1 declines by 20% (PC20); vaso-occlusive crisis