Analysis of a large cohort of cystic fibrosis patients with severe liver disease indicates lung function decline does not significantly differ from that of the general cystic fibrosis population

• DOI: 10.1371/journal.pone.0205257
• Publication Date: 2018-10-11T13:43:26Z
• Authors: Deepika Polineni; Annalisa V. Picco...; William B. Hannah; Sarah N. Dalrymple; Rhonda G. Pace; Peter R. Durie; Simon C. Ling; Michael R. Knowles; Jaclyn R. Stonebr...
• Keywords: Liver disease; Vital capacity

Previous reports of lung function in cystic fibrosis (CF) patients with liver disease have shown worse, similar, or even better forced expiratory volume 1 second (FEV1), compared to CF without disease. Varying definitions likely contribute these inconsistent relationships reported between and We retrospectively evaluated spirometric data 179 subjects (62% male; 58% Phe508del homozygous) severe (CFLD; defined by presence portal hypertension due cirrhosis). FEV1 values were referenced both a normal population (FEV1% predicted) (CF-specific percentile). utilized linear mixed model repeated measures assess changes (before after diagnosis CFLD), relative the populations. At CFLD, mean was 81% predicted, at 53rd percentile CFLD. There significant difference post-CFLD slope pre-CFLD (post–pre) using FEV1% predicted (-1.94, p-value < 0.0001). However, there insignificant evidence this CF-specific measure (-0.99, = 0.1268). Although declined following CFLD diagnosis, not decline percentiles. Thus, observed study cohort indicates associated worsened when large reference population.