TY  - JOUR
AU  - Marsteller, Nathan L.
AU  - Nussbaum, Eliezer
AU  - Morphew, Tricia
AU  - Randhawa, Inderpal S.
PY  - 2019
TI  - Cystic fibrosis patients at risk for disease progression marked by decline in FEV1% predicted: development of the cystic fibrosis risk of disease progression score
JF  - Journal of Thoracic Disease; Vol 11, No 12 (December 31, 2019): Journal of Thoracic Disease
Y2  - 2019
KW  - 
N2  - Background: Cystic fibrosis (CF) is one of the most common recessively inherited disorders diagnosed in early childhood in the United States. Determining the phenotype of CF patients likely to experience a significant drop in FEV1% predicted will help target efforts for mitigating this deleterious disorder.   Methods: This retrospective cohort study evaluated potential risk variables that account for the decline in FEV1% predicted in 81 CF patients treated at Miller Children’s and Women’s Hospital, CA. Cystic fibrosis risk of disease progression (CF RD-Pro) score was evaluated as a tool to identify high-risk patients for accelerated disease progression (event = drop in FEV1% predicted ≥10 percentage points) based on risk variables identified as significant.  Results: ROC analysis determined classification of high versus low-moderate risk of FEV1% decline during year two based on RD-Pro score. Scores ≥2 applied as threshold for high-risk revealed relatively good validity estimates: sensitivity =82.8%, specificity =66.7%, PVP =77.4%, PVN =73.7%, and correct classification =76%. Patients with CF RD-Pro scores suggestive of high (≥2 points) vs. low-moderate (
UR  - https://jtd.amegroups.org/article/view/34374