Browsing by Author "Farrell, Philip"

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    Poor recovery from a pulmonary exacerbation does not lead to accelerated FEV1 decline
    (Elsevier, 2017-07-29) Sanders, Don B.; Li, Zhanhai; Zhao, Qianqian; Farrell, Philip; Pediatrics, School of Medicine
    BACKGROUND: Patients with CF treated for pulmonary exacerbations (PEx) may experience faster subsequent declines in FEV1. Additionally, incomplete recovery to baseline FEV1 occurs frequently following PEx treatment. Whether accelerated declines in FEV1 are preceded by poor PEx recovery has not been studied. METHODS: Using 2004 to 2011 CF Foundation Patient Registry data, we randomly selected one PEx among patients ≥6years of age with no organ transplantations, ≥12months of data before and after the PEx, and ≥1 FEV1 recorded within the 6months before and 3months after the PEx. We defined poor PEx recovery as the best FEV1 in the 3months after the PEx <90% of the best FEV1 in the 6months before the PEx. We calculated mean (95% CI) hazard ratios (HR) of having >5% predicted/year FEV1 decline and poor PEx recovery using multi-state Markov models. RESULTS: From 13,954 PEx, FEV1 declines of >5% predicted/year were more likely to precede poor spirometric recovery, HR 1.17 (1.08, 1.26), in Markov models adjusted for age and sex. Non-Responders were less likely to have a subsequent fast FEV1 decline, HR 0.41 (0.37, 0.46), than patients who recovered to >90% of baseline FEV1 following PEx treatment. CONCLUSIONS: Accelerated declines in FEV1 are more likely to precede a PEx with poor recovery than to occur in the following year. Preventing or halting declines in FEV1 may also have the benefit of preventing PEx episodes.
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    Quantitative chest computerized tomography and FEV1 equally identify pulmonary exacerbation risk in children with cystic fibrosis
    (Wiley, 2018-10) Sanders, Don B.; Li, Zhanhai; Parker-McGill, Katelyn; Farrell, Philip; Brody, Alan S.; Pediatrics, School of Medicine
    Background: Chest computerized tomography (CT) scores are associated with the frequency of future pulmonary exacerbations in people with cystic fibrosis (CF). However, cut-off values to identify children with mild lung disease with different risks for frequent future pulmonary exacerbations have not been identified. Methods: Chest CT scans were assessed using the Brody score for participants of the Pulmozyme Early Intervention Trial (PEIT) and Wisconsin Randomized Clinical Trial of CF Newborn Screening (WI RCT). We determined the area under the receiver operating characteristic (ROC) curve for Brody scores and forced expiratory volume in 1 s (FEV1) to compare with the frequency of pulmonary exacerbations up to 10 years later. Results: There were 60 participants in the PEIT with mean (SD) age 10.6 (1.7) years at the time of the CT and 81 participants in the WI RCT with mean age 11.5 (3.0) years. The Brody score cut-off that best identified children at-risk for ≥0.3 annual pulmonary exacerbations was 3.6 in the PEIT and 2.1 in the WI RCT. There were no statistical differences between ROC curves for the Brody CT score and FEV1 % predicted in either study (P ≥ 0.4). Conclusions: CT score cut-off values that identify children with CF with mild lung disease at different risks for frequent pulmonary exacerbations over an extended follow up period are similar in separate cohorts. Brody scores and FEV1 % predicted have similar abilities to identify these children, suggesting that FEV1 % predicted alone may be adequate for predicting future frequency of pulmonary exacerbations.
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    Quantitative chest computerized tomography and FEV1 equally identify pulmonary exacerbation risk in children with cystic fibrosis
    (Wiley, 2018-10) Sanders, Don B.; Li, Zhanhai; Parker-McGill, Katelyn; Farrell, Philip; Brody, Alan S.; Pediatrics, School of Medicine
    Background Chest computerized tomography (CT) scores are associated with the frequency of future pulmonary exacerbations in people with cystic fibrosis (CF). However, cut‐off values to identify children with mild lung disease with different risks for frequent future pulmonary exacerbations have not been identified. Methods Chest CT scans were assessed using the Brody score for participants of the Pulmozyme Early Intervention Trial (PEIT) and Wisconsin Randomized Clinical Trial of CF Newborn Screening (WI RCT). We determined the area under the receiver operating characteristic (ROC) curve for Brody scores and forced expiratory volume in 1 s (FEV1) to compare with the frequency of pulmonary exacerbations up to 10 years later. Results There were 60 participants in the PEIT with mean (SD) age 10.6 (1.7) years at the time of the CT and 81 participants in the WI RCT with mean age 11.5 (3.0) years. The Brody score cut‐off that best identified children at‐risk for ≥0.3 annual pulmonary exacerbations was 3.6 in the PEIT and 2.1 in the WI RCT. There were no statistical differences between ROC curves for the Brody CT score and FEV1 % predicted in either study (P ≥ 0.4). Conclusions CT score cut‐off values that identify children with CF with mild lung disease at different risks for frequent pulmonary exacerbations over an extended follow up period are similar in separate cohorts. Brody scores and FEV1 % predicted have similar abilities to identify these children, suggesting that FEV1 % predicted alone may be adequate for predicting future frequency of pulmonary exacerbations.