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Purpose Cystic fibrosis (CF), caused by pathogenic variants in the CF transmembrane conductance regulator (CFTR), affects multiple organs including the exocrine pancreas, which is a causal contributor to cystic fibrosis–related diabetes (CFRD). Untreated CFRD causes increased CF-related mortality whereas early detection can improve outcomes. Methods Using genetic and easily accessible clinical measures available at birth, we constructed a CFRD prediction model using the Canadian CF Gene Modifier Study (CGS; n = 1,958) and validated it in the French CF Gene Modifier Study (FGMS; n = 1,003). We investigated genetic variants shown to associate with CF disease severity across multiple organs in genome-wide association studies. Results The strongest predictors included sex, CFTR severity score, and several genetic variants including one annotated to PRSS1, which encodes cationic trypsinogen. The final model defined in the CGS shows excellent agreement when validated on the FGMS, and the risk classifier shows slightly better performance at predicting CFRD risk later in life in both studies. Conclusion We demonstrated clinical utility by comparing CFRD prevalence rates between the top 10% of individuals with the highest risk and the bottom 10% with the lowest risk. A web-based application was developed to provide practitioners with patient-specific CFRD risk to guide CFRD monitoring and treatment.

Cystic fibrosis (CF), caused by pathogenic variants in the CF transmembrane conductance regulator (CFTR), affects multiple organs including the exocrine pancreas, which is a causal contributor to cystic fibrosis-related diabetes (CFRD). Untreated CFRD causes increased CF-related mortality whereas early detection can improve outcomes.Using genetic and easily accessible clinical measures available at birth, we constructed a CFRD prediction model using the Canadian CF Gene Modifier Study (CGS; n = 1,958) and validated it in the French CF Gene Modifier Study (FGMS; n = 1,003). We investigated genetic variants shown to associate with CF disease severity across multiple organs in genome-wide association studies.The strongest predictors included sex, CFTR severity score, and several genetic variants including one annotated to PRSS1, which encodes cationic trypsinogen. The final model defined in the CGS shows excellent agreement when validated on the FGMS, and the risk classifier shows slightly better performance at predicting CFRD risk later in life in both studies.We demonstrated clinical utility by comparing CFRD prevalence rates between the top 10% of individuals with the highest risk and the bottom 10% with the lowest risk. A web-based application was developed to provide practitioners with patient-specific CFRD risk to guide CFRD monitoring and treatment.

Background A significant problem during continuous renal replacement therapy is premature circuit failure, affecting efficacy and molecular clearance. Techniques to improve circuit failure are anticoagulation, access site and modality. A modality change was introduced, moving from continuous veno-venous haemofiltration to continuous veno-venous haemodiafiltration as a result of existing issues with failing circuit times and failure rates. Aim The aim of this service evaluation was to ascertain if the use of continuous veno-venous haemodiafiltration compared to continuous veno-venous haemofiltration had affected failed circuit survival times and rates. Methods A service evaluation was chosen because the focus was to ascertain what effect a practice change had had on a particular service. The service evaluation was registered with the local trust's audit department and gained university ethical approval. It was anticipated that the data generated would be used to inform, question and improve practices. Patients who received renal replacement therapy (RRT) from May 2012 to January 2013 were retrospectively identified. Patients received continuous veno-venous haemofiltration for the duration of their treatment before September 2012 and continuous veno-venous haemodiafiltration after. A total of 78 patients were identified as receiving RRT; 41 of these patients had failed circuits. Results A total of 182 failed circuits were analysed. The median duration of failed circuits during continuous veno-venous haemofiltration was shorter (2·75 h, standard deviation (SD) = 13·82) when compared to continuous veno-venous haemodiafiltration (11 h, SD = 15·26, p 0·007) more likely chance of failure. Conclusion The use of continuous veno-venous haemodiafiltration has had an overall positive effect on the haemofiltration service by reducing the number of failed circuits and increasing circuit survival times, which may have improved the efficacy of the service. Continuous veno-venous haemodiafiltration may be a more appropriate modality of choice in non-septic patients requiring prolonged continuous RRT episodes.

Patients and caregivers living with advanced chronic obstructive pulmonary disease (COPD) have complex care needs and may benefit from palliative care intervention. Little is known about how best to implement and evaluate such initiatives.To determine the feasibility of: 1) implementing a customized home-based palliative care service for patients and caregivers living with advanced COPD and 2) measuring outcomes of providing such services.Single-centre cohort longitudinal observational study.Patients with advanced COPD and their caregivers were followed in their homes for 6 months.Health-related quality of life (HRQoL), caregiver burden, symptom severity, patient/caregiver satisfaction, utilization of acute care services, end-of-life (EOL) outcomes.30 patients and 18 caregivers were enrolled over 33 months. 25 patients (83%) and 14 caregivers (77%) reached our study endpoint. 13 patients (52%) and 5 caregivers (36%) completed outcome measurements at baseline and endpoint. HRQoL, caregiver burden and symptom severity did not change. Palliative care services were welcomed and valued, yet, despite a stated preference to die at home, 16 patients who died within 18 months of study enrollment died in hospital.Providing home-based palliative care services for patients with advanced COPD is feasible but completing repeated questionnaires is impractical. Despite significant palliative supports, managing terminal symptoms exceeded caregivers' capacity to cope and forced hospital admission. Insights into systemic barriers and limitations of current palliative care service models can provide opportunities for local program innovation aimed at improving care for advanced COPD.

Background Advanced resuscitation skills training is an important and enjoyable part of medical training, but requires small group instruction to ensure active participation of all students. Increases in student numbers have made this increasingly difficult to achieve. Methods A single-blind randomised controlled trial of peer-led vs. expert-led resuscitation training was performed using a group of sixth-year medical students as peer instructors. The expert instructors were a senior and a middle grade doctor, and a nurse who is an Advanced Life Support (ALS) Instructor. A power calculation showed that the trial would have a greater than 90% chance of rejecting the null hypothesis (that expert-led groups performed 20% better than peer-led groups) if that were the true situation. Secondary outcome measures were the proportion of High Pass grades in each groups and safety incidents. The peer instructors designed and delivered their own course material. To ensure safety, the peer-led groups used modified defibrillators that could deliver only low-energy shocks. Blinded assessment was conducted using an Objective Structured Clinical Examination (OSCE). The checklist items were based on International Liaison Committee on Resuscitation (ILCOR) guidelines using Ebel standard-setting methods that emphasised patient and staff safety and clinical effectiveness. The results were analysed using Exact methods, chi-squared and t-test. Results A total of 132 students were randomised: 58 into the expert-led group, 74 into the peer-led group. 57/58 (98%) of students from the expert-led group achieved a Pass compared to 72/74 (97%) from the peer-led group: Exact statistics confirmed that it was very unlikely (p = 0.0001) that the expert-led group was 20% better than the peer-led group. There were no safety incidents, and High Pass grades were achieved by 64 (49%) of students: 33/58 (57%) from the expert-led group, 31/74 (42%) from the peer-led group. Exact statistics showed that the difference of 15% meant that it was possible that the expert-led teaching was 20% better at generating students with High Passes. Conclusions The key elements of advanced cardiac resuscitation can be safely and effectively taught to medical students in small groups by peer-instructors who have undergone basic medical education training.