Background: Pulmonary dysfunctions due to sickle cell disease (SCD) is one of the leading causes of morbidity and mortality in pediatric age group specifically in central India, Chhattisgarh which lies in Sickling belt. Obstructive and restrictive pulmonary changes develop in children with sickle cell disease, but reports conflict as to the type of changes that predominates.
Aim/Objectives: To evaluate pulmonary function tests in pediatric patients of SCD and to understand the pathophysiology & pattern of pulmonary functions. Material and Methods: An analytical cross-sectional study, a total of 75 cases (34 HbSS and 41 HbAS) of SCD aged between 10-14yrs and 45 normal healthy age, sex and ethnic matched control subjects from general population attending the indoor/outdoor in the department of Pediatrics, Pt.J.N.M. Medical college between Nov.2014-Dec.2016. Subjects with cardiac disease, chronic lung disease/disability, acute chest syndrome, subjects with symptoms of chronic respiratory disease and respiratory infection at least two weeks prior were excluded.
Results: Mean FEV1 was significantly lower in heterozygous male as compared to controls. Both mean FEF25%-75% and MVV were significantly less in heterozygous males and females as compared to controls. Both mean FVC & FEV1 were significantly lower in homozygous male as compared to controls. Both mean FEF25%-75% and MVV were significantly less in homozygous males and females as compared to controls.
Conclusion: Pulmonary function is abnormal in children with HbSS and AS. It is likely that abnormal pulmonary function reflects intrinsic lung disease in these patients and the mechanisms are more complex in this population than originally appreciated.
Keywords
Sickle Cell Disease; Electrophoresis; Restrictive and Obstructive Lung Disease