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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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