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

https://doi.org/10.1016/j.jpeds.2005.12.059Get rights and content

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.

Section snippets

Methods

Afro-Caribbean children with SCD and homozygous for sickle hemoglobin were recruited from 2 specialty clinics into a prospective study examining pulmonary abnormalities in children with SCD. The study was approved by the King’s College Hospital Research Ethics Committee, and parents gave informed written consent for their children’s participation. Consecutive children whose parents gave informed written consent were recruited without regard to their asthma status. For the purposes of this

Results

The 20 children in the ACS group had a median age of 11 years (range, 6 to 16 years) (Table I; Figure). Fifteen of the 20 “pairs” were also matched for sex. The children in the ACS group tended to be shorter than the children in the non-ACS group (P = .22), but there were no significant differences in the numbers of males or asthmatics between the 2 groups (Table I). The results of 23 of the children (8 in the ACS group) were reported in a study comparing lung function in children with SCD and

Discussion

We have demonstrated that the lung function results of the children with SCD who had been hospitalized for ACS episodes differed significantly from those of the children who had not experienced an ACS episode. The mean Raw, TLC, and RV were significantly higher in the ACS group than in the non-ACS group. The 2 groups differed (albeit not significantly so) with regard to height; hence we compared the results expressed as the percentages of those predicted for height. The proportions of children

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      Using a different methodology, in which each patient was classified as “normal”, “obstructive”, or “restrictive” according to pre-specified criteria, Koumbourlis et al. [16] confirmed their findings in infants reporting that over 57% of their patients had normal lung function, 35% had obstructive and only 8% had a restrictive pattern. During the last decade many more studies have been published with information on the lung function of children and adults with SCD [17–34] [Table 1]. While there is now a general recognition that SCD is not associated with a single pattern of lung function and that certain patients may actually have a normal or obstructive pattern, the disease remains mostly associated with a restrictive pattern especially among adult patients.

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    Karl Sylvester and Richard Patey were supported by the Community Fund and the Well Child Trust Medical Research Fund. Karl Sylvester is currently supported by the Amanda Smith Charitable Trust.

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