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BACKGROUND: COVID-19 pandemic has been challenging for all, particularly for high-risk groups including people with cystic fibrosis (PWCF). AIM: This study aims to examine impact of COVID-19 pandemic on the lives of PWCF in relation to hospital visits, use of telemedicine, employment, and mental well-being. METHODS: A cross-sectional online survey was developed by the Cystic Fibrosis (CF) Ireland research team and uploaded on SmartSurvey UK. The survey was advertised by CF Ireland via their website and social media in October 2020. The University College Dublin research partner team conducted the analysis. Logistic regression was used for the analysis, using IBM SPSS Version 26. RESULTS: One hundred nineteen PWCF responded. 47.5% deferred their hospital visits, with delays ranging from 1 to 6 months. Deferrals impacted rehabilitation therapies, medical care at hospital, and diagnostic tests. For many, online consultation was a new experience (51.7%), and 87.8% were satisfied with this method. Among those who worked during lockdown (47.8%), 87.2% (n = 48) worked at home. PWCF aged < 35 years (9.6%) were more likely to work onsite as compared to those > 35 years (1.9%). When adjusted for gender and employment, PWCF aged < 35 years were more likely to feel "nervous" (OR: 3.28; P = 0.02), "nothing could cheer them up" (OR: 3.24; P = 0.04), and "tired" (OR: 2.76; P = 0.02) as compared to those > 35 years. CONCLUSION: COVID 19 pandemic has greatly impacted PWCF in terms of hospital visits, access to tests, CF care, and psychological well-being. Younger PWCF reported greater impact on psychological health. Online consultation and electronic prescription were welcomed and could have a role post-pandemic.

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Background: The Rare Disease Research Partnership (RAinDRoP) was established in 2018 to bring together a wide variety of diverse voices in the rare disease community in Ireland and form a research partnership. This approach enabled clinicians, patients, carers and researchers to work together to identify top research priorities for rare diseases, which focused on a life-course perspective rather than a disease-specific need.                                                                                                           Methods: A participatory multiple phase approach was used to identify research priorities for rare diseases. The research process involved three main phases: Phase I, Public Consultation Survey on Research in Rare Diseases in Ireland (PCSRRDI); Phase II, Research Prioritisation Workshop (RPW); Phase III, Follow-up Public Consultation and Prioritisation Survey (FWPCPS). Results: In total, 240 individuals completed the phase I PCSRRDI, which comprised of a cross-section of health care professionals, researchers and people living with rare diseases. One thousand and fifteen statements were collected, reflecting issues and shared challenges in rare diseases. A shortlisting step by step was used to identify any statements that had received a total score of above 50% into 10-12 researchable questions or statements per the theme for the phase II workshop. Phase II was focused on three main themes: (1) Route to Diagnosis, (2) Living with Rare Disease, (3) Integrated and Palliative Care. In total, 62 individuals attended the overall workshop; 42 participated in the prioritisation sessions. A cross-section of health care professionals, researchers and people living with rare diseases were engaged at each workshop. Seventy-five individuals completed the final phase III public ranking by priority responses, and they ranked the top 15 research priorities defined by the multi-stakeholders at the phase II consensus meeting. Conclusions: This study identified priorities for rare diseases research aimed at improving the health and wellbeing of people living with rare diseases.

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UNLABELLED: Urotensin II is a novel endogenous vasoconstrictor. There are no data describing cerebrospinal fluid (CSF) concentrations in humans. Therefore, in this study, we aimed to quantify and compare plasma and CSF urotensin II concentrations in patients with essential hypertension and matched controls. Twenty male patients (10 receiving >6 mo of treatment for essential hypertension and 10 normotensive controls scheduled to undergo urological surgery under spinal anesthesia) were recruited into this single-blinded cohort study. Plasma and CSF urotensin II concentrations were measured by radioimmunoassay, along with mean arterial blood pressure (MAP), before admission, on the day of admission, and immediately before anesthesia. CSF and plasma urotensin II concentrations were low. Median (range) values in CSF for all 20 patients were significantly lower than plasma by approximately 15% (19.0 pg/mL [10.6-24.9 pg/mL] compared with 22.3 pg/mL [17.7-28.4 pg/mL]; P = 0.004). There were no significant differences between normotensive and hypertensive patients in either CSF or plasma concentrations. However, there was a significant positive correlation between average MAP and CSF urotensin II concentrations (r(2) = 0.44; P = 0.036) in the hypertensive group. IMPLICATIONS: Urotensin II is the most potent known endogenous human vasoconstrictor. In this pilot study, we report for the first time that cerebrospinal fluid levels are smaller than plasma levels and that there may be some association with increased blood pressure.

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