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https://www.selleckchem.com/products/GDC-0449.html 3% [24/45] vs. 55.3% [26/47]; P = 1.000) but significantly higher than that in group 3 (53.3% [24/45] vs. 28.2% [42/149], P = 0.002). Seven-year OS rates in group 2 were similar to those in group 1 (55.6% vs. 60.8%, P = 0.988) but significantly lower than those in group 3 (55.6% vs. 83.3%, P = 0.001). Presence of standard CSPH surrogate (hazard ratio = 1.89 [1.08-3.30], P = 0.025) and CT-enhanced CSPH surrogate (hazard ratio = 2.60 [1.56-4.39], P less then 0.001) were significant predicting factor for OS. However, CT-enhanced CSPH surrogate had significantly higher Harrell's C-index than standard surrogate (0.619 vs. 0.553, P = 0.034). Conclusion The presence of CT-enhanced CSPH surrogate including varices on CT was the significant predictive of poor OS, providing better predictive power than standard surrogate.Background Exercise testing is routinely used to measure exercise capacity in children with cystic fibrosis (CF). Various tests are available, however the psychometric properties of these measures have not been systematically reviewed for this population. Method A systematic search of electronic databases (PubMed, Web of Science, Medline, CINHAL, Cochrane, and PEDro) was performed to identify papers that (a) reported original psychometric data, (b) examined a measure of exercise capacity, (c) examined children with CF aged eight to 18 years; and (d) were published in English after 1950. The level of psychometric evidence was evaluated using the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist. Results Searches identified 1025 papers. Forty-six papers were included, covering 15 tests incremental cardiopulmonary exercise test using a cycle ergometer (CPET(cycle)) or treadmill (CPET(treadmill)), 6 minute walk test (6MWT), modified shuttle test (MST), 3-minute step test (3MST), 2 minute walk test (2MWT), Bratteby walk test, intermittent sprint test, speed ramp test,

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