James's Hospital Research Articles

P0238 Reliability of magnetic resonance imaging indices for evaluating perianal fistulizing disease activity in paediatric Crohn’s disease

Abstract Background Progress in clinical trials and drug development for paediatric perianal fistulising CD (pfCD) depends upon accurate, reproducible imaging to assess disease activity and measure treatment response. We evaluated the inter- and intra-rater reliability of existing radiologic disease activity indices and anatomical classification systems previously identified as appropriate for assessment of paediatric pfCD.1 Methods A retrospective cohort (N=50) of magnetic resonance imaging (MRI) exams representing a full spectrum of paediatric (<18 years) perianal CD severity was selected by a single expert paediatric radiologist. Methods described by Zou2 were used to estimate sample size. Three separate radiologists (2 paediatric) blinded to clinical information assessed pfCD in the exams using the Magnetic Resonance Novel Index for Fistula Imaging in CD (MAGNIFI-CD), Van Assche Index (VAI), modified VAI (mVAI), Paediatric MRI based Perianal Crohn’s disease (PEMPAC) index, a visual analogue scale (VAS) of overall pfCD disease severity (range, 0-100 mm), as well as with the Park’s and St. James University Hospital anatomic classification systems. Two radiologists read all exams twice, separated by 2 weeks, and 1 radiologist read the exams once (250 total reads). Intra- and inter-rater reliability was evaluated using intraclass correlation coefficients (ICC; equivalent to weighted kappa). Precision of the ICC estimates was quantified with 95% confidence intervals (CIs) obtained using clustered bootstrapping. Degree of reliability was interpreted with benchmarks proposed by Landis and Koch3. Results Median age of children was 13.5 years (IQR 11.4-16.1), and 64% were male. Median Paediatric Crohn’s Disease Activity Index score at time of imaging was 17.5 (IQR 10-32.5). Substantial (ICC>0.61) inter-rater reliability was observed for MAGNIFI-CD, VAI, and mVAI (Table 1), with the highest ICCs observed for the mVAI (0.72 [0.6 to 0.79]). Moderate (ICC>0.41) inter-rater reliability was observed for PEMPAC, St. James University Hospital classification, and the VAS, and fair (ICC>0.21) inter-rater reliability was observed for Parks classification. Intra-rater reliability was nearly perfect (ICC>0.81) for the VAS, and substantial for all indices except for Parks classification, which had moderate (0.60 [0.44 to 0.75]) intra-rater reliability (Table 1). Conclusion Most existing multi-item MRI-based disease activity indices and the St. James Hospital classification were reliable for the assessment of paediatric pfCD in our cohort. Our preliminary results suggest that the disease activity indices may have utility to measure treatment effect in paediatric pfCD clinical trials.

Person Centred Dementia Care Made Simple

Abstract Background This poster will outline the development and rollout of our person-centred dementia care microlearning course for all patient-facing staff based in St. James's Hospital, Dublin. Person centred care (PCC) appears to make a difference to how well people with dementia manage on hospital wards (Scerri et al., 2020). The term PCC is used widely in healthcare services and the approach is embedded in St James's nursing professional practice model, standards and policies. Yet there are health professionals who find PCC difficult to define and demonstrate in their practice (Byrne et al., 2020). Through our interactions with staff on wards and in nursing homes, Dementia Services Information Development Centre (DSiDC) are aware that healthcare staff can face difficulties in establishing a rapport with a patient with dementia. Therefore, we identified the need to design a course to facilitate the continued development of meaningful interactions between hospital staff and people with dementia. The course was divided into four stand-alone modules using clear, uncomplicated language and demonstrates how staff can use key elements of Kitwood’s PCC approach in their daily practice. Methods It is becoming harder for hospital staff to be released for onsite training courses due to increasing demands on their time. With this in mind, the DSiDC team sought innovative time-efficient ways to provide dementia education to hospital staff and so a microlearning educational tool was adopted. Microlearning delivers short pieces of content in easily understandable ways utilising a variety of methods such as: video, short written text and quizzes. Accessed by a QR code, staff can complete the course on their mobile phone at a time and place that suits the individual. A pilot study of the course was conducted among staff in a nursing home and on a key ward in St. James’s Hospital. Results 73% of participants found the content ‘extremely helpful’. At start of course, 21% of participants rated their knowledge about person centred dementia care as ‘excellent’. On completion of course, this increased to 50%. One outcome of the pilot study identified an improvement of course design to three modules as opposed to four. Conclusion As a result of the positive outcomes from pilot study, it was decided as part of the wider dissemination of the course across St. James’s Hospital, to conduct a focus group to further measure the impact of the training.

Design, build and implementation of a solution for prescribing, monitoring and administration of insulin within an inpatient electronic patient record - the st james's hospital experience

Design, build and implementation of a solution for prescribing, monitoring and administration of insulin within an inpatient electronic patient record - the st james's hospital experience

Assessing the relevance and requirement of a prostate cancer passport: A patient-centric survey.

e23037 Background: Prostate cancer, a prevalent malignancy among men worldwide, necessitates a multifaceted approach to its management, encompassing diagnosis, treatment, and post-treatment care. The absence of a consolidated and accessible cancer information for prostate cancer patients prompts an exploration into the necessity and potential benefits of a dedicated Prostate Cancer Passport. This survey-based study seeks to elucidate patient perspectives and assess the demand for it. Methods: We Surveyed prostate cancer patients attending St James Hospital Medical Oncology OPD and day ward. The survey methodology integrated a diverse cohort of individuals representing varying demographics, encompassing age groups and socio-economic backgrounds. Questions were structured to gauge patient awareness, preferences, and perceived benefits associated with the adoption of a Prostate Passport. The survey was structured to gauge patient awareness levels regarding existing health record systems, preferences for a specialized Prostate Cancer Passport, and anticipated benefits associated with its implementation Furthermore, the survey seeked to elucidate potential concerns, barriers to adoption, and the anticipated impact on patient-provider interactions. Results: The survey’s findings indicate a significant interest among participants in the implementation of a specialized platform like the Prostate Passport, with more than 65% expressing enthusiasm about its potential benefits in improving healthcare management. However, 20% of patients reported not receiving any prostate cancer information booklet from their healthcare providers. Additionally, 30% of participants expressed dissatisfaction with communication with their medical team, while over 40% lacked confidence in their understanding of prostate cancer. Nevertheless, concerns related to data security were raised by more than 25% of patients, and approximately 23% identified potential barriers to adoption, such as interoperability with existing healthcare systems and ease of access across different healthcare settings. However, concerns regarding data security were highlighted by over 25% of patients and about 23% of patients identified interoperability with existing healthcare systems, and ease of access across various healthcare settings as potential barriers to adoption. Conclusions: The survey outcomes offer valuable perspectives on the distinct requirements and preferences of individuals facing prostate cancer. This data is crucial for customizing the Prostate Cancer Passport's design and functionalities to effectively cater to those needs. Our initial iteration of the prostate cancer passport has been completed and will be distributed to our oncology patients in the upcoming week. We plan to conduct a feedback survey in six months to gather insights and further enhance the passport based on user experiences.

Assessing our adherence to bone protection standards in metastatic prostate cancer in the largest Irish center: Are we falling behind?

e24145 Background: Prostate cancer ranks as the second most prevalent cancer in men globally, often necessitating androgen deprivation therapy (ADT) as part of its treatment. While ADT effectively diminishes testosterone levels, a vital factor in prostate cancer cell growth, it concurrently poses a notable side effect – an accelerated rate of bone loss. This heightened bone loss elevates the vulnerability of patients to osteoporosis and fractures. The optimization of bone health entails early identification of patients susceptible to osteoporosis and fractures, the implementation of preventative measures, and prompt interventions to mitigate bone loss and fracture risks. Methods: The audit aims to evaluate adherence to established guidelines, such as those outlined by the National Comprehensive Cancer Network (NCCN) and the European Society for Medical Oncology (ESMO), concerning the assessment and optimization of bone health. A retrospective analysis was performed on the medical records of prostate cancer patients who underwent Androgen Deprivation Therapy (ADT) at St James Hospital in Dublin. Pertinent data, encompassing patient demographics, duration of ADT, documentation of bone health assessment, frequency of Bone Mineral Density (BMD) testing, utilization of preventive measures (e.g., calcium and vitamin D supplementation), and any interventions for bone health optimization (such as bisphosphonate therapy and denosumab), was systematically collected. Results: A comprehensive review encompassed 57 patients, revealing that 38 patients (67%) exhibited metastatic disease. Among them, 42 patients (74%) presented with castrate-sensitive disease. Out of the total, 24 patients (42%) underwent a baseline Dual-Energy X-ray Absorptiometry (DEXA) scan. Results indicated that 12 patients were diagnosed with Osteoporosis, 9 with Osteopenia, and 3 with normal Bone Mineral Density (BMD). At baseline, 26 patients (46%) initiated Calcium and Vitamin D supplements, while 12 patients (21%) commenced Bisphosphonates/Denosumab based on DEXA scan outcomes. Notably, 8 (14%) patients experienced a skeletal/bone-related event which is much higher than reported in literature. Conclusions: The findings are intended to guide healthcare professionals in optimizing bone health strategies, with the ultimate goal of improving patients' quality of life and minimizing the risk of fractures and related complications. Identified areas for improvement in clinical practices will be addressed by implementing recommended recommendations, aiming to enhance patient care, decrease the risk of osteoporosis and fractures, and overall improve patient outcomes. A re-audit is planned to monitor ongoing adherence to guidelines and assess the impact of interventions implemented as a result of this study.

Experience of pediatric to adult transition in immunology services: patient experience questionnaire and micro-costing analysis.

The effective transition from pediatric to adult care for individuals with chronic medical conditions should address the medical, psychosocial and educational needs of the cohort. The views and experiences of service users and their families are an integral component of service development. This study sought to evaluate the current provision of transition services from pediatric immunology services to adult immunology services for patients with a diagnosis of an inborn error of immunity at St. James's Hospital, Dublin. We gathered patient perspectives on the experience of the transition process using a structured survey. In addition, we adopted a micro-costing technique to estimate the cost of implementing the current standard of care for these patients. Results of a micro-costing analysis suggest that the most significant component of cost in assessing these patients is on laboratory investigation, an area where there is likely significant duplication between pediatric and adult care. Perspectives from patients suggested that the transition period went well for the majority of the cohort and that they felt ready to move to adult services, but the transition was not without complications in areas such as self-advocacy and medication management. The transition process may benefit from enhanced communication and collaboration between pediatric and adult services.

Pride in Death.

Background: While all ageing populations share common concerns as they plan for old age and end of life, many older LGBTQIA+ people face significant additional challenges.
 Prevailing social, religious, and cultural attitudes which existed in Ireland until relatively recently, mean older LGBTQIA+ people grew up in society where asserting their identity often led to exclusion and isolation.
 Planning for end of life can make things easier but these factors create barriers for older LGBTQIA+ people who may fear that asserting their identity will lead to discrimination or neglect.
 Research has demonstrated while many LGBTQIA+ people feel uncomfortable disclosing their sexual orientation to health care professionals and allied health staff they are also considerably more likely to rely on formal support services as they get older due to diminished support networks in comparison to their heterosexual peers.
 During Palliative Care Week we encourage people to have tender conversations about death, dying and bereavement and what it means to them. Therefore we sought to engage and have honest conversations with the LGBTQIA+ community at a death cafe type event, to understand key issues for the community, relating to death and dying, and what really matters to them in planning ahead for their end of life care. 
 Who Was Engaged: AIIHPC leads Palliative Care Week and engaged with LGBT Ireland as the gateway organisation to the LGBTQIA+ community. We also engaged with the Irish Hospice Foundation (IHF) who has expertise in advance care planning. Liz Gleeson from Shapes of Grief also joined as a facilitator given her expertise on bereavement. Bettina Korn also joined as a facilitator given her expertise as an end of life coordinator at St James Hospital and an LGBT Champion.
 Impact:
 - LGBTQIA+ Bereavement Support Groups are needed in the Republic of Ireland. LGBTQIA+ people suffer from ambiguous and complicated grief, making the creation of specific bereavement support groups essential.
 - Intergenerational Networks. Younger and older LGBTQIA+ people want connect with one another. Currently spaces outside pubs are lacking.
 - Creation of LGBTQIA+ Reference Group to co-design health information resources. LGBTQIA+ people do not see themselves reflected in the health information provided and are thus, less likely to inform themselves.
 - Need for creativity around funeral planning outside of religious rituals that mutes the voice of the individual.
 Learning for an International Audience: By having honest conversations with people from different communities, we gain insight into their real needs. Sounds simple enough but open and honest conversations can only take place in a safe space where trust exists. Trust can be gained by working with gateway organisations of underrepresented communities, to ensure services are co-designed with the true individual at heart.
 Next Steps:
 - LGBTQIA+ death cafe being held on 8th of December in Dublin.
 - Training of IHF Bereavement Support Line by LGBT Ireland
 - Engaging with other gateway organisations of underrepresented communities such as homeless, refugees, people with disabilities, amongst others to provide a safe space for conversations to take place.

Development and Initial Implementation of a Clinical Monitoring Strategy in a Non-regulated Trial: a research note from the ReStOre II Trial.

Data and Safety Monitoring is integral to quality assurance of clinical trials. Although monitoring is a core legal component of regulated clinical trials, non-regulated trials are not mandated to incorporate monitoring. Consequently, the monitoring process has been underutilised and underreported in this setting. This research report outlines the development and plans for implementing a bespoke Clinical Monitoring Strategy within the ' Rehabilitation Strategies Following Oesophagogastric and Hepatopancreaticobiliary Cancer (ReStOre II) Trial', a non-regulated trial comparing a 12-week multidisciplinary programme of rehabilitation to standard care in a cohort of 120 cancer survivors. This research note provides a detailed overview of the ReStOre II Clinical Monitoring Strategy and describes the development of the strategy pre and post awarding of the grant. The strategy consists of the establishment and implementation of a comprehensive trial governance structure, inclusive of a Trial Management Group, Trial Steering Committee Meeting, and Independent Data Monitoring Committee. In addition, external trial monitoring by the Clinical Research Facility at St James's Hospital. Three monitoring visits will be conducted during the trial; i) site initiation visit, ii) interim monitoring visit, and iii) close our visit. The Clinical Monitoring Strategy has been finalised and is currently being implemented within the ReStOre II Trial. Two site initiation visits and one interim monitoring visit have been completed to date. This research note provides a template for implementation of a Clinical Monitoring Strategy in a non-regulated clinical trial. ReStOre II Trial: https://clinicaltrials.gov/ct2/show/NCT03958019.

An evaluation of the effectiveness of an advanced practice physiotherapist in the emergency department setting in Ireland.

One of the means of easing increased pressure on emergency care worldwide has been the development of advanced musculoskeletal physiotherapy practice in the emergency department setting. This model of care is in its infancy in Ireland. To evaluate the effectiveness of an advanced practice physiotherapist working as a primary contact clinician in the emergency department at St. James's Hospital, Dublin. A three-month retrospective chart review was undertaken for patients assigned the advanced practice physiotherapist as their primary clinician during their emergency department attendance. Three widely accepted measures of quality in emergency medicine were used to evaluate effectiveness, namely, time from attendance to discharge, time from triage to assessment, and unplanned reattendance within seven days. A total of 129 patients were included in this study. Time from attendance to discharge was significantly less in the APP group (mean 208.5min, standard deviation 122.4min) than in the ED group (mean 377.1min, standard deviation 314.7min) (mean difference - 168.61 (95% C.I - 191.24- - 145.98)) (p < 0.001). Time from triage to assessment was significantly less in the APP group (mean 72.1min, standard deviation 51.9min) than in the ED group (mean 94.1min, standard deviation 96.5min) (mean difference - 22.08 (95% C.I - 31.28- - 12.89)) (p < 0.001). The unplanned reattendance rate was 3.9%. No adverse events were identified. The findings of this study indicate that an advanced practice physiotherapist can provide a timely, effective, and safe service for patients attending the emergency department with musculoskeletal complaints in Ireland.

Advice following a possible first seizure: what do patients attending for EEG remember?

PurposeThis study was designed to assess the knowledge of patients following their first seizure or blackout of unknown cause. We aimed to compare the advice our cohort of patients recalled against that suggested in the current literature. Background5 % of the population will experience a non- febrile seizure in their lifetime. Education and advice for the patient and their family is an important aspect of their care. MethodAfter reviewing the recommended guidelines from both the Scottish Intercollegiate Guidelines Network and the National Institute for Health and Care Excellence, a questionnaire was developed. From 1st May 2020 to 1st September 2022, a questionnaire was given to eligible patients attending St. James's Hospital for an electroencephalogram (EEG) following their first possible seizure or blackout of unknown cause. The patients were provided with a list of topics and were asked to select which, if any, were discussed with them. ResultsA total of 50 eligible adults participated in the study. Driving was the topic most frequently recalled as having been discussed at 66 % followed by family education. ConclusionIt has been recognised that patients remember as little as a fifth of information initially discussed, failing to recall 40–80 % of content within medical encounters and our study supports this. This highlights the need to review the information delivered and how this is achieved as well as using new methods to help increase the retention of this vital information.

Psychosocial impact of being diagnosed with mild cognitive impairment: patient and carer perspective.

Mild cognitive impairment (MCI) may represent an intermediate, prodromal phase of dementia. Although persons with MCI (PwMCI) are able to function independently, they often experience reduced ability to carry out their usual activities. This can result in social, emotional and functional challenges. To explore the understanding and psychosocial impact of receiving a diagnosis of MCI on patients and carers. A cross-sectional cohort study was conducted at St James's Hospital Memory Clinic involving patients who attended the clinic for assessment from 1 January 2020 to 30 April 2021 and received a diagnosis of MCI. We completed questionnaires with patients and a nominated family member or friend of each patient (FwMCI). Forty-seven PwMCI participated in the study, and 36 nominated family members and/or friends completed the FwMCI questionnaire. In our cohort of PwMCI, most of the participants were not aware of their diagnosis; only 21% used the term MCI, and only 25% attributed their problems to a pathological cause. The majority of participants had no recollection of any discussion around the likelihood of progression. One-third of participants expressed relief that they did not have dementia. Most PwMCI reported positive psychological well-being and did not endorse symptoms of depression or anxiety. There was slight discordance of illness perception among the PwMCI-FwMCI dyads. Forty-seven per cent of FwMCI reported at least a mild degree of carer burden on the Zarit Burden Scale. Patients' awareness of being diagnosed with MCI is relatively limited. Public education campaigns raising awareness about MCI can help influence the 'illness representation' for MCI and enable people to seek timely advice and support.

Time-to-Event Analysis of Factors Influencing Delay in Discharge from a Subacute Complex Discharge Unit during the First Year of the Pandemic (2020) in an Irish Tertiary Centre Hospital.

Our study aimed to analyse delaying factors amongst patients with a length of stay (LOS) > 15 days during the COVID-19 pandemic using time-to-event analysis. A total of 390 patients were admitted between March 2020-February 2021 to the subacute complex discharge unit in St James's Hospital: 326 (83.6%) were >65 years of age and 233 (59.7%) were female. The median (IQR) age was 79 (70-86) years with a median (IQR) of 19.4 (10-41) days. A total of 237 (60.7%) events were uncensored, with LOS > 15 days, of which 138 (58.2%) were female and 124 (52.32%) had >4 comorbidities; 153 (39.2%) were censored into LOS ≤ 15 days, and death occurred in 19 (4.8%). Kaplan-Meier's plot compared factors causing a delay in discharge to the single factors: age, gender, and multimorbidity. A multivariate Cox regression analysis adjusted to age, gender, and multimorbidity predicted factors affecting LOS. Further research is required to explore multimorbidity as a risk factor for mortality in patients with prolonged LOS within a complex discharge unit and target gender-specific frailty measures to achieve high-quality patient management.

Blood Pressure Signal Entropy as a Novel Marker of Physical Frailty: Results from the FRAILMatics Clinical Cohort.

In this study we investigated the association between information entropy in short length blood pressure signals and physical frailty status, in a group of patients aged 50+ recruited from the Falls and Syncope Unit at the Mercer's Institute for Successful Ageing in St James's Hospital, Dublin, Ireland. This work is an external clinical validation of findings previously derived in a population-based cohort from The Irish Longitudinal Study on Ageing (TILDA). The hypothesis under investigation was that dysregulation (as quantified by entropy) in continuous non-invasive blood pressure signals could provide a clinically useful marker of physical frailty status. We found that in the 100 patients investigated, higher entropy in continuously measured resting state diastolic blood pressure was associated with worse physical frailty score, as measured by the Frailty Instrument for primary care of the Survey of Health, Ageing and Retirement in Europe (SHARE-FI). Since physical frailty is defined as a pre-disability state and hence it can be difficult for clinicians to identify at an early stage, the quantification of entropy in short length cardiovascular signals could provide a clinically useful marker of the physiological dysregulations that underlie physical frailty, potentially aiding in identifying individuals at higher risk of adverse health outcomes.

Investigation of the Suitability of a Commercial Radiation Sensor for Pretherapy Dosimetry of Radioiodine Treatment Patients.

Radioiodine (I-131) therapy is routinely used to treat conditions of the thyroid. Dosimetry planning in advance of I-131 therapy has been shown to improve patient treatment outcomes. However, this pretherapy dosimetry step requires multiple outpatient appointments and is not feasible for patients living at greater distances. Here, the feasibility of a commercially available smartphone-operated radiation sensor (Smart Geiger Pro, Technonia) for at-home patient pretherapy dosimetry has been investigated. The influence of both treatment-specific parameters (radioisotope activity, gamma photon energy, patient size) and external factors (sensor placement and motion) on the ability of the radiation sensor to accurately quantify radiation dose rates has been studied. The performance limits of the radiation sensor have been identified. A preliminary trial of the sensor on four I-131 patients prior to their therapy, conducted at the Nuclear Medicine/Endocrinology departments of St James's Hospital Dublin, is also presented. A comparable performance between the low-cost radiation sensor and that of a hospital-grade thyroid uptake probe is reported. This work demonstrates the potential of low-cost commercially available radiation sensors as a solution for at-home pretherapy dosimetry for long distance patients, or indeed for hospitals who wish to implement dosimetry at reduced cost. Recommended conditions for optimum sensor performance use are presented.

Blood Transfusion Ameliorates Free Haem-Induced Loss of Thrombomodulin and Endothelial Cell Activation in Paediatric Sickle Cell Anaemia

Background Sickle cell anaemia (SCA) is responsible for major paediatric morbidity including risk of stroke in up to 10% of children. Mainstays of therapy include hydroxyurea (HU) and chronic blood transfusion (BT), with BT considered gold standard for stroke prevention. However, the biological mechanisms through which BT is significantly superior to HU in reducing stroke risk have not been clearly defined. SCA complications are due in part to free haem-mediated damage, leading to endothelial cell (EC) activation, tissue factor (TF) expression and release of von Willebrand Factor (VWF), which binds platelets and sickle RBCs. Recently loss of EC Thrombomodulin (TM) has been shown to promote EC activation and VWF release and soluble TM (sTM) plays a role in disseminated intravascular coagulation and COVID-19. Hypothesis We hypothesize free haem alters EC TM expression, promoting EC activation and VWF release. Intravascular hemolysis and free haem may be reduced in children on chronic BT, maintaining EC quiescence and enhancing protection against stroke compared with HU. Methods Following ethical approval and informed consent 180 SCA children (median age 12 years, 49% male) were recruited. Steady-state plasma samples were collected prior to BT (n=84) or at outpatient clinic for HU (n=96). Paired crisis samples were obtained in a subset of HU (n=30). EC activation markers and Weibel Palade Body (WPB) proteins were evaluated by ELISA, VWF multimers were assessed by 1.8% agarose gels and ADAMTS13 activity assessed by FRET. Free haem levels were quantified and thrombin generation (TG) assays performed. Factor VIII:C was measured in the National Coagulation Centre at St James's Hospital. Human umbilical vein endothelial cells (HUVEC) were exposed to cell-free haem (Hemin), hydroxyurea, cytokines (IL-1β and TNFα) or patient plasma; VWF was measured in the supernatant and HUVEC TM expression assessed by flow cytometry. Results VWF:Ag and VWF propeptide (VWFpp) levels were significantly increased in HU compared to BT children, suggesting acute EC activation (median plasma VWF:Ag levels 1.29 v 1.03 IU/ml; p=0.0005, median VWFpp levels 1.63 v 1.17 IU/ml p=0.006). Furthermore, plasma levels of other WPB stored proteins were also significantly elevated in the HU cohort, including Angiopoietin-2 (Ang-2) (4214 v 2337 pg/ml, p<0.0001 and Osteoprotegerin (OPG) (1825 v 1507 pg/ml p=0.006). Abnormalities of high-, intermediate- and low molecular weight VWF multimers were noted in HU but not BT. While putative inhibitors of ADAMTS13 were increased in HU, including Platelet Factor 4 and Interleukin-6, ADAMTS13 activity did not differ between groups. FVIII:C levels were significantly elevated in 65% of the total cohort and were higher in HU than BT (1.62 IU/ml v 1.30 IU/ml p<0.0001). Free haem levels were increased in HU versus BT (12.5 v 9.5μM p=0.0003), as well as levels of LDH and reticulocyte %, pointing to increased intravascular hemolysis. EC markers and FVIII:C correlated strongly with hemolytic indices. In addition to increased EC activation, increased endogenous thrombin potential (ETP), peak thrombin and velocity index were observed in HU versus BT, pointing to enhanced TG, possibly driven by elevated circulating TF. TG parameters correlated with plasma FVIII:C levels. Exposure of HUVEC to free haem in vitro resulted in a significant decrease in surface TM expression and increased VWF secretion (p<0.05). Conversely exposure to hydroxyurea or cytokines did not alter TM expression. Exposure of HUVEC to plasma from crisis but not paired steady-state plasma induced EC activation and VWF release compared with control plasma. Importantly, free haem levels ≥20 μM were observed in 25% HU children in steady state and 40% in crisis, compared with 0% of BT. Furthermore, significantly elevated sTM levels in SCA children with a history of cerebrovascular disease compared to those without (5.7 v 4.6 ng/ml p=0.02), suggesting that sTM may predict end-organ dysfunction. Conclusions We have shown in a large cohort of SCA children that EC activation, TG and intravascular haemolysis are increased in HU-treated children compared with BT. Free haem induces loss of EC TM and VWF release in vitro, leading to EC activation. Elevated sTM levels were a biomarker of end-organ damage. These data suggest that reduction of free haem in children undergoing regular BT is a key mechanism through which EC quiescence is maintained in paediatric SCA.

Vitamin B12 supplementation post-gastrectomy: a service closed-loop audit at St. James's Hospital, Dublin.

BackgroundVitamin B12 (VB12) deficiency is a well-described complication post-gastrectomy. It is caused by the loss of parietal cell mass leading to megaloblastic anaemia. This closed-loop audit assesses patient understanding of and adherence with VB12 supplementation guidelines post-gastrectomy.MethodsA closed-loop audit cycle was performed. After the first cycle, an educational intervention was actioned prior to re-audit. One hundred twenty-five patients who underwent gastrectomy between 2010 and 2020 were available for study (86 total gastrectomies (TG), 39 subtotal gastrectomies (STG)). Twenty-nine patients who had not been adherent with VB12 supplementation/surveillance were eligible for re-audit.Results91.9% (79/86) of TG patients reported adherence in regular parenteral VB12 supplementation. Adherence was significantly lower for STG for checking (and/or replacing) their VB12, with only 53.8% (21/39) checking their VB12 levels. 67/125 (53.6%) of the patients stated that they knew it was important to supplement B12 post-gastrectomy. 37.8% (43/113) of participants could explain why this was important, and 14.4% (18/125) had any knowledge of the complications of VB12 deficiency. Following re-audit, 5/8 (57.5%) of TG patients who had not been adherent with VB12 supplementation in the first cycle were now adherent with VB12 supplementation following our educational intervention. 7/17 (41.2%) of the STG group had received VB12 or made arrangements to receive supplemental VB12 if it was indicated.ConclusionThis study demonstrates good adherence in those undergoing TG. Patient understanding correlates with adherence, suggesting that patient education and knowledge reinforcement may be key to adherence with VB12 supplementation. A simple educational intervention can improve adherence with VB12 supplementation in patients undergoing gastrectomy.

Motivators and barriers to blood donation among potential donors of African and Caucasian ethnicity.

Minority blood donation, especially from individuals of African ethnicity, is a focus for many countries with diverse populations. As the need for antigen-negative RBC transfusions for patients with Sickle Cell Disease (SCD) continues to grow, inclusion of more African blood donors is essential to ensure this demand is met. This study aims to explore barriers and motivators to blood donation and awareness of SCD among potential donors of diverse ethnic backgrounds in Ireland. Following ethical approval, patients attending the National Sickle Cell Disease and Thalassemia service at St James's Hospital were invited to share an online anonymous survey within their local communities to achieve snowball-sampling. 387 respondents completed the survey, including 311 non-donors (median age 25 years, 67% female). Ethnic backgrounds included: African or African-Irish (59%), White or Caucasian (25%), Asian (8%), Hispanic or Latino (3%), Middle Eastern (3%), Multiracial or Biracial (2%). The most commonly identified barrier overall was lack of information on blood donation. African respondents were significantly more likely to report lack of information and malaria-related barriers than Caucasians. Motivators also varied across ethnic groups, with African respondents more likely to donate to help someone within their own community or for religious motivators. Awareness of SCD was higher among African respondents. While some barriers to blood donation are shared across all ethnic groups including lack of information, notable differences exist between Caucasian and African respondents. Specific actions to recruit and retain African blood donors should focus on these key areas.

Biomarker development in amyotrophic lateral sclerosis: Challenges and viable strategies.

European Journal of NeurologyVolume 29, Issue 7 p. 1867-1868 COMMENTARY Biomarker development in amyotrophic lateral sclerosis: Challenges and viable strategies Peter Bede, Corresponding Author Peter Bede bedep@tcd.ie orcid.org/0000-0003-0492-4772 Computational Neuroimaging Group, Trinity College Dublin, Dublin, Ireland Department of Neurology, St James's Hospital, Dublin, Ireland Correspondence Peter Bede, Computational Neuroimaging Group, Trinity College Dublin, Dublin, Ireland. Email: bedep@tcd.ie Contribution: Conceptualization (equal), Writing - original draft (equal)Search for more papers by this authorJasmin Lope, Jasmin Lope Computational Neuroimaging Group, Trinity College Dublin, Dublin, Ireland Contribution: Conceptualization (equal), Writing - original draft (equal)Search for more papers by this author Peter Bede, Corresponding Author Peter Bede bedep@tcd.ie orcid.org/0000-0003-0492-4772 Computational Neuroimaging Group, Trinity College Dublin, Dublin, Ireland Department of Neurology, St James's Hospital, Dublin, Ireland Correspondence Peter Bede, Computational Neuroimaging Group, Trinity College Dublin, Dublin, Ireland. Email: bedep@tcd.ie Contribution: Conceptualization (equal), Writing - original draft (equal)Search for more papers by this authorJasmin Lope, Jasmin Lope Computational Neuroimaging Group, Trinity College Dublin, Dublin, Ireland Contribution: Conceptualization (equal), Writing - original draft (equal)Search for more papers by this author First published: 25 April 2022 https://doi.org/10.1111/ene.15372 See paper by Y. M. Falzone et al. on page 1930 Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume29, Issue7July 2022Pages 1867-1868 RelatedInformation

Study protocol for the St James's Hospital, Tallaght University Hospital, Trinity College Dublin Allied Researchers' (STTAR) Bioresource for COVID-19

Background: The current coronavirus disease 2019 (COVID-19) pandemic began in Ireland with the first confirmed positive case in March 2020. In the early stages of the pandemic clinicians and researchers in two affiliated Dublin hospitals identified the need for a COVID-19 biobanking initiative to support and enhance research into the disease. Through large scale analysis of clinical, regional, and genetic characteristics of COVID-19 patients, biobanks have helped identify, and so protect, at risk patient groups The STTAR Bioresource has been created to collect and store data and linked biological samples from patients with SARS-CoV-2 infection and healthy and disease controls. Aim: The primary objective of this study is to build a biobank, to understand the clinical characteristics and natural history of COVID-19 infection with the long-term goal of research into improved disease understanding, diagnostic tests and treatments. Methods: This is a prospective dual-site cohort study across two tertiary acute university teaching hospitals. Patients are recruited from inpatient wards or outpatient clinics. Patients with confirmed COVID-19 infection as well as healthy and specific disease control groups are recruited. Biological samples are collected and a case report form detailing demographic and medical background is entered into the bespoke secure online Dendrite database. Impact: The results of this study will be used to inform national and international strategy on health service provision and disease management related to COVID-19. In common with other biobanks, study end points evolve over time as new research questions emerge. They currently include patient survival, occurrence of severe complications of the disease or its therapy, occurrence of persistent symptoms following recovery from the acute illness and vaccine responses.

Study protocol for theSt James's Hospital, Tallaght University Hospital, Trinity College Dublin Allied Researchers' (STTAR) Bioresource for COVID-19.

Background: The current coronavirus disease 2019 (COVID-19) pandemic began in Ireland with the first confirmed positive case in March 2020. In the early stages of the pandemic clinicians and researchers in two affiliated Dublin hospitals identified the need for a COVID-19 biobanking initiative to support and enhance research into the disease. Through large scale analysis of clinical, regional, and genetic characteristics of COVID-19 patients, biobanks have helped identify, and so protect, at risk patient groups The STTAR Bioresource has been created to collect and store data and linked biological samples from patients with SARS-CoV-2 infection and healthy and disease controls. Aim: The primary objective of this study is to build a biobank, to understand the clinical characteristics and natural history of COVID-19 infection with the long-term goal of research into improved disease understanding, diagnostic tests and treatments. Methods: This is a prospective dual-site cohort study across two tertiary acute university teaching hospitals. Patients are recruited from inpatient wards or outpatient clinics. Patients with confirmed COVID-19 infection as well as healthy and specific disease control groups are recruited. Biological samples are collected and a case report form detailing demographic and medical background is entered into the bespoke secure online Dendrite database. Impact: The results of this study will be used to inform national and international strategy on health service provision and disease management related to COVID-19. In common with other biobanks, study end points evolve over time as new research questions emerge. They currently include patient survival, occurrence of severe complications of the disease or its therapy, occurrence of persistent symptoms following recovery from the acute illness and vaccine responses.