Pulmonary Function Tests in Children with Sickle Cell Anaemia Between Ages 6 and 18 Years - A Cross-Sectional Study

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Nisha Ranjeet Aglave
Girish Nanoti


BACKGROUND: Sickle cell Anaemia is a common hereditary hemoglobinopathy that affects Pulmonary functions as a consequence of microvasculature obstruction by the sickle cells as well as red blood cell hemolysis. Repeated microvascular obstruction leading to pulmonary hypertension, parenchymal fibrosis, and endothelial dysfunction are probably the main mechanisms for chronic pulmonary diseases in SCA.

METHODS: We have assessed pulmonary function in 62 children of sickle cell anemia between 6 to 18 years of age in a steady-state and its correlation with various important factors like past history of ACS, use of hydroxyurea, number of blood transfusions or the levels of HbF. This study was conducted in a tertiary hospital between Oct 2018 to Dec 2020.

RESULTS: Restrictive pattern of pulmomary function was more common in patients with SCA. HbF was found to have a mild to moderate negative correlation with FEV1/FVC which was statistically significant (p<0.05). History of hospital admission was significantly associated with lower FEV1, FVC, and the PEFR, while hydroxyurea use, history of blood transfusion, and history of ACS showed no statistically significant association.

CONCLUSION: SCA may be associated with compromise in the respiratory function in Pediatric patients. Restrictive type changes may be commonly noted in SCA patients which may lead to reduced vital capacity. Hence there is a need for close follow-up and yearly PFT of vulnerable patients.

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Nisha Ranjeet Aglave, & Girish Nanoti. (2023). Pulmonary Function Tests in Children with Sickle Cell Anaemia Between Ages 6 and 18 Years - A Cross-Sectional Study. Journal of Coastal Life Medicine, 11(2), 1382–1388. Retrieved from https://jclmm.com/index.php/journal/article/view/1169


Platt OS, Brambilla DJ, Rosse W, et al: Mortal¬ity in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994; 330: 1639–1644.

Kassim AA, Payne AB, Rodeghier M, et al: Low forced expiratory volume is associated with earlier death in sickle cell anemia. Blood 2015; 126: 1544–1550.

Chaturvedi S, Labib Ghafuri D, Kassim A, et al: Elevated tricuspid regurgitant jet velocity, reduced forced expiratory volume in 1 sec¬ond, and mortality in adults with sickle cell disease. Am J Hematol 2017; 92: 125–130.

4 Desai PC, Ataga KI: The acute chest syn¬drome of sickle cell disease. Expert Opin Pharmacother 2013; 14: 991–999

Achigbu KI, Odetunde OI, Chinawa JM, et al. Pulmonary function indices in children with sickle cell anemia in Enugu, south-east Nigeria. Saudi Med J. 2015;36(8):928–34.

Chinawa JM, Ubesie AC, Chukwu BF, Ikefuna AN, Emodi IJ. Prevalence of hypoxemia among children with sickle cell anemia during steady state and crises: a cross-sectional study. Niger J Clin Pract 2013;16:91-95.

Vieira AK, Alvim CG, Carneiro MCM, Ibiapina CC. Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem? J Bras Pneumol. 2016;42(6):409-415.

Kuti BP, Adegoke SA. Pulmonary function abnormalities in Nigerian children with sickle cell anaemia: Prevalence, pattern and predictive factors. Pediatr Respirol Crit Care Med 2018;2:73-9.

Sylvester KP, Patey RA, Milligan P. Pulmonary function abnormalities in children with sickle cell disease. Thorax. 2004;59:67–70.

Sen N, Kozanoglu I, Karatasli M, Ermis H, Boga C, Eyuboglu FO. Pulmonary function and airway hyperresponsiveness in adults with sickle cell disease. Lung 2009; 187: 195-200.

Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med 2008; 359: 2254-2265.

Faleti OA, Akodu SO, Disu EA, Njokanma OF. Pulmonary functions in children with sickle cell anaemia in steady state in Lagos, Nigeria. Annals of Health Research. 2017;3(1):18-25.

Jaja SI, Opesanwo O, Mojiminiyi FB, Kehinde MO. Lung function, haemoglobin and irreversibly sickled cells in sickle cell patients. West Afr J Med. 2000;19(3):225-9.

Purohit R, Rao SS, Goyal JP, Shah VB, Charan J. Pulmonary Function Tests in Sickle Cell Disease. Indian J Pediatr. 2016;83(8):783-6.

Leong MA, Dampier C, Varlotta L, Allen JL. Airway hyperreactivity in children with sickle cell disease. J Pediatr. 1997;131:278–83.