title: Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease.
abstract: Lung function abnormalities occur in children with sickle cell disease (SCD) and may be associated with elevated pulmonary blood volume. To investigate that association, we determined whether blood transfusion in SCD children acutely increased pulmonary capillary blood volume (PCBV) and increased respiratory system resistance (Rrs5). Measurements of Rrs5 and spirometry were made before and after blood transfusion in 18 children, median age 14.2 (6.6-18.5) years. Diffusing capacity for carbon monoxide and nitric oxide were assessed to calculate the PCBV. Post transfusion, the median Rrs5 had increased from 127.4 to 141.3% predicted (p<0.0001) and pulmonary capillary blood volume from 39.7 to 64.1 ml/m2 (p<0.0001); forced expiratory volume in one second (p=0.0056) and vital capacity (p=0.0008) decreased. The increase in Rrs5 correlated with the increase in PCBV (r=0.50, p=0.0493). Increased pulmonary capillary blood volume may at least partially explain the lung function abnormalities in SCD children.
authors.affiliation: Division of Asthma, Allergy and Lung Biology MCR Centre for Allergic Mechanisms in Asthma, King's College London, UK; National Institute for Health Research (NIHR) Biomedical Research Centre based at Guy's and St. Thomas' NHS Foundation Trust and King's College London, UK. Electronic address: alan.lunt@nhs.net.
authors.forename: Alan
authors.initials: A
authors.lastname: Lunt
authors.affiliation: Division of Asthma, Allergy and Lung Biology MCR Centre for Allergic Mechanisms in Asthma, King's College London, UK. Electronic address: emily.mcghee@kcl.ac.uk.
authors.forename: Emily
authors.initials: E
authors.lastname: McGhee
authors.affiliation: Division of Asthma, Allergy and Lung Biology MCR Centre for Allergic Mechanisms in Asthma, King's College London, UK. Electronic address: polly.robinson@gmail.com.
authors.forename: Polly
authors.initials: P
authors.lastname: Robinson
authors.affiliation: Department of Paediatric Haematology, King's College Hospital NHS Foundation Trust, London, UK. Electronic address: david.rees2@nhs.net.
authors.forename: David
authors.initials: D
authors.lastname: Rees
authors.affiliation: Department of Paediatric Haematology, King's College Hospital NHS Foundation Trust, London, UK. Electronic address: sue.height@nhs.net.
authors.forename: Susan
authors.initials: S
authors.lastname: Height
authors.affiliation: Division of Asthma, Allergy and Lung Biology MCR Centre for Allergic Mechanisms in Asthma, King's College London, UK; National Institute for Health Research (NIHR) Biomedical Research Centre based at Guy's and St. Thomas' NHS Foundation Trust and King's College London, UK. Electronic address: anne.greenough@kcl.ac.uk.
authors.forename: Anne
authors.initials: A
authors.lastname: Greenough
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000628:therapy; D001803:Blood Transfusion; D001810:Blood Volume / Q000502:physiology*; D001811:Blood Volume Determination; D002196:Capillaries / Q000503:physiopathology*; D002200:Capillary Resistance / Q000502:physiology; D002248:Carbon Monoxide / Q000097:blood; D002648:Child; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000098:blood supply* / Q000503:physiopathology*; D008297:Male; D009569:Nitric Oxide / Q000097:blood; D013147:Spirometry; D016896:Treatment Outcome
chemical_list: D009569:Nitric Oxide; D002248:Carbon Monoxide
keywords: Lung function abnormalities; Pulmonary capillary blood volume; Sickle cell disease; Transfusion