Impact of acute chest syndrome on lung function of children with sickle cell disease |
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Authors: | Sylvester Karl P Patey Richard A Milligan Peter Rafferty Gerrard F Broughton Simon Rees David Thein Swee Lay Greenough Anne |
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Institution: | Departments of Child Health, Health and Life Sciences, and Haematological Medicine, King's College London School of Medicine at Guy's, King's College, and St Thomas' Hospitals, London, United Kingdom. |
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Abstract: | 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 (FEV(1)); forced vital capacity (FVC); FEV(1)/FVC ratio; peak expiratory flow (PEF); forced expiratory flow at 25% (FEF(25)), 50% (FEF(50)), and 75% (FEF(75)) 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 FEF(25) (P = .04) and FEF(75) (P = .03) pre-bronchodilator use and a lower mean FEV(1)/FVC ratio (P = .03) and FEF(75) (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. |
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Keywords: | 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 FRCpleth Functional residual capacity FVC Forced vital capacity PEF Peak expiratory flow Raw Airway resistance RVpleth Residual volume SCD Sickle cell disease SCLD Sickle chronic lung disease TLCpleth Total lung capacity VCpleth Vital capacity |
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