Pulmonary function tests in children with sickle cell disease

ABSTRACT

Acute and chronic pulmonary complications in adult patients with sickle cell disease [SCD] have been reported in several studies. The aim of this study was to determine the various patterns of pulmonary functions in children with SCD. Twenty four patients with homozygous Hb SS SCD aged from 8 to 15 years were evaluated for lung functions using maximal expiratory flow volume curves obtained by a forced expiratory maneuver. Each patient was assigned to one of three patterns of lung functions [normal, obstructive or restrictive]. Airway hyper responsiveness was evaluated by means of a trial with a bronchodilator. Normal patterns were detected in 45.8% of the patients, obstructive pattern in 33.3% and restrictive pattern in 21.2%. Total serum iron and serum ferritin were significantly higher in patients with obstructive lung pattern than in those with normal lung function. History of acute chest syndrome was significantly higher in patients with obstructive and restrictive lung patterns than in patients with normal lung functions. Longterm blood transfusion was significantly more common in patients with obstructive lung pattern in comparison to the normal group. A positive response to the bronchodilator was observed in about 42% of the whole group and in 87.5% in patients with obstructive lung pattern. In conclusion, pulmonary dysfunction is common in children with Hb SS SCD, LAO being more common than restrictive pattern, careful observation of total serum iron and the proper use of chelation therapy is needed to reduce the incidence of pulmonary dysfunction