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Differences in lung function between children with sickle cell anaemia from West Africa and Europe
  1. Michele Arigliani1,
  2. Luigi Castriotta2,
  3. Ramatu Zubair3,
  4. Livingstone Gayus Dogara4,
  5. Chiara Zuiani1,
  6. Emma Raywood5,
  7. Katy Vecchiato6,
  8. Enrico Petoello7,
  9. Ashel Dache Sunday3,
  10. Sharon Ndoro8,
  11. Mario Canciano Canciani1,
  12. Atul Gupta9,
  13. Paola Cogo1,
  14. Baba Inusa8
  1. 1Department of Medicine, Division of Pediatrics, University Hospital of Udine, Udine, Italy
  2. 2Institute of Hygiene and Clinical Epidemiology, University Hospital of Udine, Udine, Italy
  3. 3Department of Pediatrics, Barau Dikko Teaching Hospital, Kaduna State University, Kaduna, Nigeria
  4. 4Department of Hematology, Barau Dikko Teaching Hospital, Kaduna State University, Kaduna, Nigeria
  5. 5Respiratory, Critical Care and Anaesthesia Section, III Programme, Great Ormond Street Institute of Child Health, University College London, London, UK
  6. 6University of Trieste, Trieste, Italy
  7. 7Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, Italy
  8. 8Department of Paediatric Haematology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Trust, London, UK
  9. 9Department of Pediatric Respiratory Medicine, King’s College Hospital NHS Foundation Trust, London, UK
  1. Correspondence to Dr Michele Arigliani, Division of Pediatrics, University Hospital of Udine, Piazzale S. Maria Misericordia 1, 33100 Udine, Italy; michelearigliani{at}


Introduction Lung function abnormalities are common in sickle cell anaemia (SCA) but data from sub-Saharan Africa are limited. We hypothesised that children with SCA from West Africa had worse lung function than their counterparts from Europe.

Methods This prospective cross-sectional study evaluated spirometry and anthropometry in black African individuals with SCA (haemoglobin phenotype SS) aged 6–18 years from Nigeria and the UK, when clinically stable. Age-matched controls were also included in Nigeria to validate the Global Lung Initiative spirometry reference values.

Results Nigerian SCA patients (n=154) had significant reductions in both FEV1 and FVC of ~1 z-score compared with local controls (n=364) and ~0.5 z-scores compared with the UK patients (n=101). Wasting (body mass index z-score<−2) had a prevalence of 27% in Nigerian patients and 7% in the UK ones (p<0.001). Among children with SCA, being resident in Nigeria (OR 2.4, 95% CI 1.1 to 4.9), wasting (OR 2.3, 95% CI 1.1 to 5.0) and each additional year of age (OR 1.2, 95% CI 1.1 to 1.4) were independently associated with increased risk of restrictive spirometry (FVC z-score<−1.64+FEV1/FVC≥−1.64).

Conclusions This study showed that chronic respiratory impairment is more severe in children with SCA from West Africa than Europe. Our findings suggest the utility of implementing respiratory assessment in African children with SCA to early identify those with chronic lung injury, eligible for closer follow-up and more aggressive therapies.

  • paediatric lung disaese
  • clinical epidemiology
  • respiratory measurement

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  • Contributors MA conceived the study, performed data collection, interpreted data and wrote the manuscript; BI and PC conceived the study and contributed to the manuscript; MA, RZ, LGD, CZ, KV, EP, ADS and SN performed data collection and contributed to the manuscript; LC performed statistical analysis, interpreted data and contributed to the manuscript; ER, AG and MCC interpreted data and contributed to the manuscript. All the authors approved the final draft of the manuscript.

  • Funding ‘Sickle Cell Cohort Research Foundation’ ( and ‘A.L.P.I. associazione allergie pneumopatie infantile’ ( offered financial support to refund travel expenses to Nigeria for two investigators (MA and CZ) and to buy a mobile spirometer that was used for data collection and was donated to the local hospital in Nigeria at the end of the study.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Ethics approval This cross-sectional multicentre study was conducted under approval by the local ethics committees in Nigeria (HREC 16-0017) and in the UK (REC 12/SW/0319).

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available on reasonable request.

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