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Pulmonary function, CT and echocardiographic abnormalities in sickle cell disease
  1. Alan Lunt1,2,
  2. Sujal R Desai3,
  3. Athol U Wells4,
  4. David M Hansell4,
  5. Sitali Mushemi3,
  6. Narbeh Melikian3,
  7. Ajay M Shah2,5,
  8. Swee Lay Thein2,6,
  9. Anne Greenough1,2
  1. 1Division of Asthma, Allergy and Lung Biology, MCR Centre for Allergic Mechanisms in Asthma, King's College London, London, UK
  2. 2National Institute for Health Research (NIHR) Biomedical Research Centre based at Guy's and St Thomas’ NHS Foundation Trust and King's College London, London, UK
  3. 3Department of Radiology and Interstitial Lung Unit, King's College London, London, UK
  4. 4Department of Radiology, Royal Brompton Hospital, London, UK
  5. 5Cardiovascular Division, King's College London British Heart Foundation Centre, London, UK
  6. 6Division of Cancer Studies, King's College London and Dept Haematological Medicine, King's College Hospital, London, UK
  1. Correspondence to Professor Anne Greenough, Neonatal Intensive Care Centre, 4th Floor Golden Jubilee Wing, King's College Hospital, Denmark Hill, London SE5 9RS, UK; anne.greenough{at}


Objectives To test the hypothesis that vascular abnormalities on high-resolution CT (HRCT) would be associated with echocardiographic changes and lung function abnormalities in patients with sickle cell disease (SCD) and the decline in lung function seen in SCD patients.

Methods HRCT, echocardiography and lung function assessments were made in 35 adults, 20 of whom had previously been assessed a median of 6.6 years prior to this study. The pulmonary arterial dimensions on HRCT were quantified as the mean segmental pulmonary artery/bronchus (A/B) ratio and the summated cross-sectional area of all pulmonary vessels <5 mm in diameter (cross-sectional area (CSA)<5 mm%).

Results The segmental A/B ratio was negatively correlated with FEV1, vital capacity (VC), forced expiratory flow between 25% and 75% of VC (FEF25/75) and arterial oxygen saturation (SpO2) and positively with the residual volume: total lung capacity ratio (RV:TLC) and respiratory system resistance (Rrs). CSA<5 mm% was negatively correlated with FEV1, FEF25/75 and SpO2 and positively with RV, RV:TLC and respiratory system resistance (Rrs). There were significant correlations between cardiac output assessed by echocardiography and the segmental A/B ratio and CSA<5 mm%. Lung function (FEV1 p=0.0004, VC p=0.0347, FEF25/75 p=0.0033) and the segmental A/B ratio (p=0.0347) and CSA<5 mm% (p<0.0001) significantly deteriorated over the follow-up period.

Conclusions Abnormalities in pulmonary vascular volumes may explain some of the lung function abnormalities and the decline in lung function seen in adults with SCD.

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