title: Longitudinal assessment of lung function in children with sickle cell disease.
abstract: OBJECTIVE
  To prospectively assess longitudinal lung function in children with sickle cell disease (SCD).
  
  
  OBJECTIVE
  Lung function in SCD children deteriorates with increasing age and the decline is more marked in younger children who have recently suffered ACS episodes.
  
  
  METHODS
  Two prospective longitudinal studies.
  
  
  METHODS
  Two cohorts of SCD children and age and ethnic matched controls were recruited. Cohort One (47 SCD and 26 controls) had a median age of 8.8 years and follow up of 2 years and Cohort Two (45 SCD and 26 controls) a median age of 10.2 years and follow up of 10 years.
  
  
  METHODS
  Forced expiratory volume in one second (FEV1 ), vital capacity (VC), forced expiratory flow between 25% and 75% of VC (FEF 25-75 ), total lung capacity (TLC) and residual volume (RV) were measured on two occasions.
  
  
  RESULTS
  In both groups of SCD children, lung function declined significantly, but in neither control group. ACS episodes were more frequent during the follow up period in Cohort One than Cohort Two (P < 0.0001). The rate of decline was greater in Cohort One than Cohort Two for FEV1 (P = 0.008), VC (P = 0.001), FEF25-75 (P = 0.030), TLC (P = 0.004), and RV (P = 0.043). In Cohort Two restrictive abnormalities were more common at follow up (P = 0.006).
  
  
  CONCLUSIONS
  Lung function deteriorated with increasing age in SCD children and the rate of decline was greater in younger children in whom ACS episodes were more common. Pediatr Pulmonol. 2016;51:717-723. © 2015 Wiley Periodicals, Inc.
authors.affiliation: Division of Asthma, Allergy, and Lung Biology MRC Center for Allergic Mechanisms in Asthma, King's College London, London, United Kingdom.|National Institute for Health Research (NIHR), Biomedical Research Centre based at Guy's and St Thomas' NHS Foundation Trust and King's College London, London, United Kingdom.
authors.forename: Alan
authors.initials: A
authors.lastname: Lunt
authors.affiliation: Division of Asthma, Allergy, and Lung Biology MRC Center for Allergic Mechanisms in Asthma, King's College London, London, United Kingdom.
authors.forename: Emily
authors.initials: E
authors.lastname: McGhee
authors.affiliation: Division of Asthma, Allergy, and Lung Biology MRC Center for Allergic Mechanisms in Asthma, King's College London, London, United Kingdom.
authors.forename: Karl
authors.initials: K
authors.lastname: Sylvester
authors.affiliation: Division of Asthma, Allergy, and Lung Biology MRC Center for Allergic Mechanisms in Asthma, King's College London, London, United Kingdom.|National Institute for Health Research (NIHR), Biomedical Research Centre based at Guy's and St Thomas' NHS Foundation Trust and King's College London, London, United Kingdom.
authors.forename: Gerrard
authors.initials: G
authors.lastname: Rafferty
authors.affiliation: Department of Paediatric Haematology, King's College Hospital NHS Foundation Trust, London, United Kingdom.
authors.forename: Moira
authors.initials: M
authors.lastname: Dick
authors.affiliation: Department of Paediatric Haematology, King's College Hospital NHS Foundation Trust, London, United Kingdom.
authors.forename: David
authors.initials: D
authors.lastname: Rees
authors.affiliation: Department of Paediatric Haematology, King's College Hospital NHS Foundation Trust, London, United Kingdom.
authors.forename: Susan
authors.initials: S
authors.lastname: Height
authors.affiliation: Division of Gene and Cell Based Therapy, King's College London School of Medicine at Guy's, King's College and St Thomas' Hospitals, London, United Kingdom.
authors.forename: Swee Lay
authors.initials: SL
authors.lastname: Thein
authors.affiliation: Division of Asthma, Allergy, and Lung Biology MRC Center for Allergic Mechanisms in Asthma, King's College London, London, United Kingdom.|National Institute for Health Research (NIHR), Biomedical Research Centre based at Guy's and St Thomas' NHS Foundation Trust and King's College London, London, United Kingdom.
authors.forename: Anne
authors.initials: A
authors.lastname: Greenough
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000453:epidemiology / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008450:Maximal Midexpiratory Flow Rate; D011446:Prospective Studies; D014109:Total Lung Capacity
keywords: acute chest syndrome; asthma; restrictive lung disease