https://ogma.newcastle.edu.au/vital/access/ /manager/Index en-au 5 https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:45112 Wed 26 Oct 2022 13:25:03 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:11837 Wed 11 Apr 2018 16:30:22 AEST ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:19489 Wed 11 Apr 2018 16:22:13 AEST ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:28065 Tue 24 Aug 2021 14:27:26 AEST ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:46902  8. Overall, there was no difference in survival or disease progression, regardless of antacid treatment, GORD diagnosis or GORD symptoms. Conclusions: Neither the use of antacid therapy nor the presence of GORD symptoms affects longer term outcomes in IPF patients. This contributes to the increasing evidence that antacid therapy may not be beneficial in IPF patients and that GORD directed therapy should be considered on an individual basis to treat the symptoms of reflux.]]> Tue 06 Dec 2022 16:07:24 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:24852 Thu 17 Feb 2022 09:28:28 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:30829 Thu 09 Dec 2021 11:04:14 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:37810 Mon 31 Jan 2022 15:14:29 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:41841 Tnfsf10^−/− BALB/c mice were administered bleomycin to induce fibrosis and some groups were treated with the FTY720 analogue AAL(s) to activate PP2A. Mouse fibroblasts were treated with recombinant TRAIL and fibrotic responses were assessed. Results: TRAIL in serum and MID1 protein levels in biopsies from IPF patients were increased compared to controls. MID1 levels were inversely associated while PP2A activity levels correlated with DLco. Tnfsf10^−/− and mice treated with the PP2A activator AAL(s) were largely protected against bleomycin-induced reductions in lung function and fibrotic changes. Addition of recombinant TRAIL to mouse fibroblasts in-vitro increased collagen production which was reversed by PP2A activation with AAL(s). Conclusion: TRAIL signalling through MID1 deactivates PP2A and promotes fibrosis with corresponding lung function decline. This may provide novel therapeutic targets for IPF.]]> Mon 15 Aug 2022 10:15:38 AEST ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:46958 40% predicted. We set out to observe the most sensitive clinical measure that would change with treatment in terms of exercise capacity or lung function in adults with severe lung disease as defined by an FEV1 < 40% predicted when clinically stable. Methods: 10 adults homozygous for the Phe508del received LUM/IVA. We assessed; six minute walk test (6MWT), spirometry, gas transfer (DLCO), plethysmography, and nitrogen multiple breath washout (MBW) at baseline, 4, 12, 24 and 52 weeks. Comparison was made with 10 matched historical controls that had been observed over 12 months. Results: There was a significant improvement in 6MWT by 4 weeks of treatment; with a mean increase of 78 m (SD 62.3) and this increased to 118.1 m (SD 80.9) (ANOVA p = 0.006) by 52 weeks. Significant improvements were also seen in the resting heart rate and the oxygen saturation (SaO2) after 6 min walking. A significant improvement was not seen in FEV1 though until 24 weeks, though this was maintained at 52 weeks (ANOVA, p = 0.0004). There were no significant differences seen in the MBW or DLCO. After 12 months treatment with LUM/IVA, in comparison to historical controls; the 6MWT increased by 118 m (SD 80.9), but fell in the controls - 61.3 m (SD 31.1). FEV1; LUM/IVA led to an increase of 0.398 L/min, compared to a fall in the controls - 0.18 (SD 0.2). Conclusion: In adults homozygous for Phe508del with severe disease, treatment with LUM/IVA results in a clinically significant improvement in 6MWT that was evident at 4 weeks and maintained at 52 weeks. Improvement in exercise tolerance is an important outcome to consider in those with more severe airways disease. Trial registration: This was an observational trial conducted on individuals who became eligible to receive LUM/IVA. All investigations were carried out as part of routine clinical care. The trial was registered in retrospect on the 13/5/2019 on the Australian New Zealand Clinical Trials registry; ACTRN12619000708156.]]> Mon 12 Dec 2022 10:01:33 AEDT ]]> https://ogma.newcastle.edu.au/vital/access/ /manager/Repository/uon:37978 Fri 16 Jul 2021 18:14:37 AEST ]]>