Impact of acute chest syndrome on lung function of children with sickle cell disease

Objective

To test the hypothesis that children with sickle cell disease (SCD) who experienced an acute chest syndrome (ACS) hospitalization episode would have worse lung function than children with SCD without ACS episodes.

Study design

Forced expiratory volume in 1 second (FEV₁); forced vital capacity (FVC); FEV₁/FVC ratio; peak expiratory flow (PEF); forced expiratory flow at 25% (FEF₂₅), 50% (FEF₅₀), and 75% (FEF₇₅) of FVC; airway resistance (Raw); and lung volumes were compared in 20 children with ACS and 20 aged-matched children without ACS (median age, 11 years; range, 6 to 16 years). Fourteen age-matched pairs were assessed before and after bronchodilator use.

Results

The mean Raw (P = .03), TLC (P = .01), and RV (P = .003) were significantly higher in the group with ACS than in the group without ACS. There were no significant differences in the changes in lung function test results in response to bronchodilator administration between the 2 groups, but the children with ACS had a lower FEF₂₅ (P = .04) and FEF₇₅ (P = .03) pre–bronchodilator use and a lower mean FEV₁/FVC ratio (P = .03) and FEF₇₅ (P = .03) post–bronchodilator use.

Conclusions

Children with SCD who experienced an ACS hospitalization episode had significant differences in lung function compared with those who did not experience ACS episodes. Our results are compatible with the hypothesis that ACS episodes predispose children to increased airway obstruction.

Abbreviations:  ACS, acute chest syndrome , AHR, Airway hyperresponsiveness , FEF₂₅, Forced expiratory flow at 25% of vital capacity , FEF₅₀, Forced expiratory flow at 50% of vital capacity , FEF₇₅, Forced expiratory flow at 75% of the vital capacity , FEV₁, Forced expiratory volume in 1 second , FRC_pleth, Functional residual capacity , FVC, Forced vital capacity , PEF, Peak expiratory flow , Raw, Airway resistance , RV_pleth, Residual volume , SCD, Sickle cell disease , SCLD, Sickle chronic lung disease , TLC_pleth, Total lung capacity , VC_pleth, Vital capacity