Pulmonary function profile of children with sickle cell disease

Background: Lung disease is a major cause of morbidity and mortality in sickle cell disease (SCD). In view of severe anemia, decreased blood oxygen affinity, pulmonary arterial vaso-occlusion, microinfarction and microfibrosis being associated with sickle cell disease; an analysis of pulmonary function in them will be of great interest.Methods: Seventy 6-12 years old children with SCD (SS pattern) were studied as cases along with age, sex and socioeconomic status matched 70 controls (AA pattern) and comparisons drawn between the two groups.Results: All the static and dynamic pulmonary functions such as FVC, FEV1, MMEFR25-75%, PEFR and MVV were found reduced in sickle cell disease, most of them significantly.Conclusions: Any restrictive, obstructive or combined pattern may be produced in SCD depending upon frequency & severity of the acute chest syndrome and vaso-occlusive crises in past