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Introduction: Autosomal dominant tubulointerstitial kidney disease (ADTKD) is a rare genetic cause of renal impairment resulting from mutations in the MUC1, UMOD, HNF1B, REN, and SEC61A1 genes. Neither the national or global prevalence of these diseases has been determined. We aimed to establish a database of patients with ADTKD in Ireland and report the clinical and genetic characteristics of these families. Methods: We identified patients via the Irish Kidney Gene Project and referral to the national renal genetics clinic in Beaumont Hospital who met the clinical criteria for ADTKD (chronic kidney disease, bland urinary sediment, and autosomal dominant inheritance). Eligible patients were then invited to undergo genetic testing by a variety of methods including panel-based testing, whole exome sequencing and, in five families who met the criteria for diagnosis of ADTKD but were negative for causal genetic mutations, we analyzed urinary cell smears for the presence of MUC1fs protein. Results: We studied 54 individuals from 16 families. We identified mutations in the MUC1 gene in three families, UMOD in five families, HNF1beta in two families, and the presence of abnormal MUC1 protein in urine smears in three families (one of which was previously known to carry the genetic mutation). We were unable to identify a mutation in 4 families (3 of whom also tested negative for urinary MUC1fs). Conclusions: There are 4443 people with ESRD in Ireland, 24 of whom are members of the cohort described herein. We observe that ADTKD represents at least 0.54% of Irish ESRD patients. [ABSTRACT FROM AUTHOR]

Background: Home haemodialysis (HHD) has the potential to impact positively on patient outcomes and health resource management. There has been rejuvenated international interest in HHD in recent years. Aim: We aimed to review the activity and outcomes of the Irish HHD Programme since inception (2009-16). Design: Retrospective review. Methods: Patient data were collected using the national electronic Renal Patient database (eMEDRenal version 3.2.1) and individual centre records. All data were recorded in a coded fashion on a Microsoft Excel Spread-sheet and analysed with Stata SE software. Results: One hundred and one patients completed training and commenced HHD; a further fourty-five patients were assessed for HHD suitability but did not ultimately dialyse at home. Twenty patients switched to nocturnal HHD when this resource became available. The switch from conventional in-centre dialysis to HHD led to an increase in the mean weekly hours on haemodialysis (HD) and a reduction in medication burden for the majority of patients. The overall rate of arteriovenous fistula (AVF) as primary vascular access was 62%. Most HHD complications were related to access function or access-related infection. Over the 7-years, 29 HHD patients were transplanted and 9 patients died. No deaths resulted directly from a HHD complication or technical issue. Conclusions: Patient and technique survival rates compared favourably to published international reports. However, we identified several aspects that require attention. A small number of patients were receiving inadequate dialysis and require targeted education. Ongoing efforts to increase AVF and self-needling rates in HD units must continue. Psychosocial support is critical during the transition between dialysis modalities. [ABSTRACT FROM AUTHOR]

Pressure related complications such as abdominal wall hernias occur with relative frequency in patients on peritoneal dialysis. Less frequently, a transudative pleural effusion containing dialysate can develop. This phenomenon appears to be due to increased intra-abdominal pressure in the setting of congenital or acquired diaphragmatic defects. We report three cases of pleuroperitoneal leak that occurred within a nine-month period at our institution. We review the literature on this topic, and discuss management options. The pleural effusion resolved in one patient following drainage of the peritoneum and a switch to haemodialysis. One patient required emergency thoracocentesis. The third patient developed a complex effusion requiring surgical intervention. The three cases highlight the variability of this condition in terms of timing, symptoms and management. The diagnosis of a pleuroperitoneal leak is an important one as it is managed very differently to most transudative pleural effusions seen in this patient population. Surgical repair may be necessary in those patients who wish to resume peritoneal dialysis, or in those patients with complex effusions. Pleuroperitoneal leak should be considered in the differential diagnosis of a pleural effusion, particularly a right-sided effusion, in a patient on peritoneal dialysis. [ABSTRACT FROM AUTHOR]

Objectives Drug development is increasingly focused on treatments for oncology and rare diseases. The estimated annual/treatment cost per patient and incremental cost effectiveness ratio (ICER) were extracted from the pharmacoeconomic reviews. [Extracted from the article]

Objective: To analyze the impact of centralization on key metrics, outcomes, and patterns of care at the Irish National Center., Background: Overall survival rates for esophageal cancer in the West have doubled in the last 25 years. An international trend towards centralization may be relevant; however, this model remains controversial, with Ireland centralizing esophageal cancer surgery in 2011., Methods: All patients (n=1245) with adenocarcinoma of the esophagus or junction treated with curative intent involving surgery, including endoscopic surgery, were included (n=461 from 2000 to 2011, and 784 from 2012 to 2022). All data entry was prospectively recorded. Overall survival was measured (1) for the entire cohort, (2) for patients with locally advanced disease (cT 2-3 N 0-3 ), and (3) for patients undergoing neoadjuvant therapy. All complications were recorded as per Esophageal Complication Consensus Group definitions, and the Clavien-Dindo severity classification. Data were analyzed using GraphPad Prism (v.6.0) for Windows and SPSS (v.23.0) software (SPSS) R Studio (R version 4.2.2). Survival times were calculated using a log-rank test and Cox regression analysis, and Kaplan-Meier curves were generated., Results: Endotherapy for cT1a/intramucosal cancer adenocarcinoma increased from 40 (9% total) to 245 (31% total) procedures between the pre-centralization and post-centralization (post-C) periods. A significantly ( P < 0.001) higher proportion of patients with cT 2-3 N 0-3 disease in the post-C period underwent neoadjuvant therapy (66% vs 53%). Operative mortality was lower ( P =0.02) post-C, at 2% versus 4.5%, and ≥IIIa Clavien-Dindo major complications decreased from 33% to 25% ( P < 0.01). Recurrence rates were lower post-C (38% vs 53%, P < 0.01). Median overall survival was 73.83 versus 47.23 months in the 2012 to 2022 and 2000 to 2011 cohorts, respectively ( P < 0.001). For those who received neoadjuvant therapy, the median survival was 28.5 months pre-centralization and 42.5 months post-C ( P < 0.001)., Conclusions: These data highlight improvements in both operative outcomes and survival from the time of centralization, and a major expansion of endoscopic surgery. Although not providing proof, the study suggests a positive impact of formal centralization with governance on key quality metrics and an evolution in patterns of care., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Aim: Advanced therapy medicinal products (ATMPs) are medicines for human use that are based on genes, tissues or cells. They offer groundbreaking new opportunities for the treatment of disease and injury. However, ATMP adoption requires adjustments to current clinical practices and frameworks. This study investigates the readiness of the Irish healthcare system to adopt licensed ATMPs. Materials & methods: Scoping review, guided by the preferred reporting items for systematic reviews and meta-analyses - scoping review extension. A systematic search of English articles from 2013 to 2023 (published and grey literature) will be conducted. Results: Findings will be presented via narrative summary, graphical and tabular formats. Discussion: Review findings will be discussed in the context of recommendations that will inform national policy and strategy on the adoption of ATMPs in Ireland.

Background: The EU-wide, cross-sectional observational study of lipid-lowering therapy (LLT) use in secondary and primary care (DA VINCI) assessed the proportion of patients achieving low-density lipoprotein cholesterol (LDL-C) goals recommended by the European Society of Cardiology (ESC)/European Atherosclerosis Society (EAS) guidelines and provided an insight into regional use of LLT in Europe, including Ireland., Aims: This analysis focuses on data from patients in Ireland who participated in the DA VINCI study., Methods: The DA VINCI study enrolled patients receiving LLT at primary and secondary care sites across 18 European countries between June 2017 and November 2018. The study assessed the achievement of risk-based 2016 and 2019 ESC/EAS LDL-C goals. This subgroup analysis aimed to evaluate LDL-C goal attainment in an Irish cohort of primary and secondary care patients., Results: In total, 198 patients from Ireland were enrolled from three primary care and three secondary care centres. Most patients were White and male, and were receiving moderate- or high-intensity statin therapy (most frequently atorvastatin or rosuvastatin). Few patients (< 10%) were receiving combination therapy of statin and ezetimibe. Approximately 60% of patients achieved their 2016 ESC/EAC LDL-C goals while less than half the patients achieved their 2019 ESC/EAS goals. Approximately half of secondary prevention patients achieved their 2016 ESC/EAS goals and only 20% of secondary prevention patients achieved their 2019 ESC/EAS goals., Conclusions: These results highlight the disparity between dyslipidaemia management in clinical practice in Ireland and guideline recommendations., Trial Registration: ENCePP; EU PAS 22,075; date registered 06 February 2018., (© 2022. The Author(s).)

Aims: Recent increases in the number of patients with atrial fibrillation (AF) prescribed oral anticoagulants (OAC) are evident in Ireland and internationally, largely due to the availability of direct oral anticoagulants (DOACs). This study aimed to determine the rate of stroke in the context of increasing anticoagulation utilisation, with a focus on AF-related ischaemic stroke (IS)., Methods: Dispensing data for OACs were identified for the period 2010-2018 as well as hospital discharges for IS (2005-2018). Irish National Stroke Register data were used to elucidate the characteristics of patients with acute ischaemic stroke., Results: The number of patients prescribed OACs increased by 94% from 2010-2018 with a significant change from 2013 (β = 2.57, P = .038), associated with a large increase in the number of patients on DOACs. There was 3.3-fold increase in expenditure on OACs nationally from 2013 to 2018, of which 94% was DOAC related. Using the 2013 timepoint, ischaemic stroke rates until 2018 did not show a significant deviation from the previous trend (β = 0.00, P = .898). The percentage of AF-related ischaemic stroke was stable from 2013 to 2017 with a 4.5% decrease in 2018. The percentage of ischaemic stroke patients with previously diagnosed AF decreased from 2013 to 2018; however, there was an increase in the percentage of ischaemic strokes while on OAC in this cohort., Conclusion: Large increases in OAC utilisation have not resulted in changes in ischaemic stroke rates at a national level. The percentage of ischaemic strokes with a previous diagnosis of AF has decreased indicating a possible benefit from greater OAC utilisation. However, the percentage presenting with an ischaemic stroke while on OAC treatment is increasing. The increase in patients presenting with stroke while treated with OAC may largely reflect the national increase in patients prescribed DOACs but the findings raise concerns about treatment failures. The real-world effectiveness of DOACs requires further examination., (© 2021 The Authors. British Journal of Clinical Pharmacology published by John Wiley & Sons Ltd on behalf of British Pharmacological Society.)

This study aims to estimate the theoretical excess expenditure that would be incurred by the Irish state-payer, should drugs be reimbursed at their original asking ("list") price rather than at a price at which the drug is considered cost-effective. In Ireland, all new drugs are evaluated by the National Centre for Pharmacoeconomics. For this study, drugs that were submitted by pharmaceutical companies from 2012 to 2017 and considered not cost-effective at list price were reviewed. A total of 43 such drugs met our inclusion criteria, and their pharmacoeconomic evaluations were further assessed. The price at which the drug could be considered cost-effective (cost-effective price) at the upper cost-effectiveness threshold used in Ireland (€ 45 000/quality adjusted life-year) was estimated for 18 drugs with an available cost-effectiveness model. Then, for each drug, the list price and cost-effective price (both per unit) were both individually applied to 1 year of national real-world drug utilization data. This allowed the estimation of the expected expenditures under the assumptions of list price paid and cost-effective price paid. The resulting theoretical excess expenditure, the expenditure at list price minus the expenditure at the cost-effective price, was estimated to be €108.2 million. This estimate is theoretical because of the confidentiality of actual drug prices. The estimation is calculated using the list price and likely overestimates the actual excess expenditure, which would reduce to zero if cost-effective prices are agreed. Nevertheless, this estimate illustrates the importance of a process to assess the value of new drugs so that potential excess drug expenditure is identified., (Copyright © 2021 ISPOR–The Professional Society for Health Economics and Outcomes Research. Published by Elsevier Inc. All rights reserved.)

Polycystic kidney diseases (PKDs) comprise the most common Mendelian forms of renal disease. It is characterised by the development of fluid-filled renal cysts, causing progressive loss of kidney function, culminating in the need for renal replacement therapy or kidney transplant. Ireland represents a valuable region for the genetic study of PKD, as family sizes are traditionally large and the population relatively homogenous. Studying a cohort of 169 patients, we describe the genetic landscape of PKD in Ireland for the first time, compare the clinical features of patients with and without a molecular diagnosis and correlate disease severity with autosomal dominant pathogenic variant type. Using a combination of molecular genetic tools, including targeted next-generation sequencing, we report diagnostic rates of 71-83% in Irish PKD patients, depending on which variant classification guidelines are used (ACMG or Mayo clinic respectively). We have catalogued a spectrum of Irish autosomal dominant PKD pathogenic variants including 36 novel variants. We illustrate how apparently unrelated individuals carrying the same autosomal dominant pathogenic variant are highly likely to have inherited that variant from a common ancestor. We highlight issues surrounding the implementation of the ACMG guidelines for variant pathogenicity interpretation in PKD, which have important implications for clinical genetics.

Aims: Entresto (sacubitril/valsartan) is used to treat symptomatic chronic heart failure with reduced ejection fraction. Given its high potential budget impact, the Health Services Executive introduced a reimbursement application system (RAS) to ensure its appropriate use. The aim of this study was to evaluate the utilisation of Entresto in Ireland and compare patient characteristics to those of the pivotal PARADIGM-HF trial., Methods: We used dispensed claims data from the Primary Care Reimbursement Services, clinical data obtained from the RAS, and data from published studies of Entresto utilisation. Differences in the baseline characteristics in the study populations vs the Entresto arm of the PARADIGM-HF trial were analysed. We also investigated cardiovascular medication use in the 6 months pre- and post-Entresto initiation., Results: In 2018, there were 1043 individuals receiving Entresto, corresponding to an expenditure of €1.2 million. Patients prescribed Entresto in Ireland were older, had lower left ventricular ejection fraction and were more symptomatic than those in the PARADIGM-HF trial. Irish patient characteristics were reflective of Entresto-treated populations in other real-world studies. More than 63% of patients were commenced on the lowest Entresto dose. Entresto initiation was associated with a reduction in the use of other medications for heart failure., Conclusion: The utilisation of Entresto has been steadily increasing in Ireland since its reimbursement approval. The expenditure in the first year was substantially lower than predicted, and the RAS is an example of how health technology management can facilitate appropriate and cost-effective use of medicines., (© 2020 The British Pharmacological Society.)

Background: The lidocaine 5% medicated plaster, Versatis ® , has one therapeutic indication listed on the Summary of Product Characteristics-symptomatic relief of post-herpetic neuralgia (PHN) in adults. Increased expenditure on Versatis ® suggests that there is considerable off-label use. To support the appropriate use of Versatis ® , the Health Service Executive's Primary Care Reimbursement Service (PCRS) introduced a reimbursement application system for Versatis ® from 1 September 2017., Objective: The aim of this study was to investigate the effect of introducing a reimbursement application system on Versatis ® prescribing under the General Medical Services (GMS) scheme., Methods: This study was carried out using prescription dispensing data from the PCRS pharmacy claims database. We carried out segmented linear regression to assess changes in the Versatis ® prescribing rate per 1000 GMS eligible population, before and after the introduction of the online reimbursement application system., Results: The results of the segmented regression analysis show that there was a statistically significant level (- 4.91, p < 0.001) and trend change (- 0.69, p < 0.001) in the rate of Versatis ® prescribing post-introduction of the reimbursement application system. In the year prior to the introduction of the system, 2016, the annual GMS expenditure on Versatis ® lidocaine 5% patches was over €27 million, whereas the GMS expenditure in 2018 was reduced to just over €2 million., Conclusion: In our study, a substantial decrease in the dispensing of Versatis ® was seen after the implementation of a reimbursement application system. Prescribing of Versatis ® should be restricted to patients with a diagnosis of PHN not only to reduce costs, but to ensure evidence-based use of this medication.

Background: Infection prevention and timely and effective treatment are among the major aims of care in kidney transplant recipients. Pre-transplant vaccination and pre-transplant viral screening have been extensively studied and are now considered standard practice. Early post-operative infection surveillance is mandatory in other vulnerable cohorts, but has not been extensively studied in this population. We hypothesized that surveillance of the most common bacterial infection types in the post-transplant setting would be beneficial and identify key areas for improvement., Methods: All adult kidney transplant recipients whose surgeries were performed in the Irish national kidney transplant unit over a 1-year period had prospective early post-transplant (first 30 days) infection surveillance in 2014 for surgical site infection, urinary tract infection, and secondary bloodstream infections (Group T0). Several key changes were implemented following scrutiny of infection patterns and clinical practice. Subsequently, infection surveillance was undertaken for 2016 and 2017 (Group T1) to assess the impact of these changes., Results: Between 2014 and 2017, the number of kidney transplants increased by 32%. The following aspects of clinical practice were the focus of change following analysis of Group T0 data: timing of surgical antimicrobial prophylaxis (SAP) administration, choice of SAP antimicrobial agent, and routine microbiological testing in the peri-operative period. Following implementation of these changes, the timing of SAP administration was greatly improved (45%-100% of cases appropriately timed). The infection rate decreased from 8.9% to 7.4% in 2016, with a further decrease to 4% in 2017 (OR 0.42 (95% CI: 0.16-1.10); P = .08). Compliance with pre-operative microbiological screening improved in Group T1., Conclusions: Simple clinical practice changes, implemented upon analysis of common bacterial infection surveillance data in the first 30 days after kidney transplantation resulted in more effective SAP administration and improved compliance with routine microbiological testing in the peri-operative period. These interventions have potentially contributed to reduced early post-operative infection rates, despite increased transplant activity in the unit. Infection surveillance is an important and under-utilized way of reducing infections in this vulnerable patient cohort., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Oral anticoagulation (OAC) for stroke prevention in patients with atrial fibrillation is underutilised. One of the impediments to warfarin therapy is the frequent monitoring required, usually at a specialised warfarin clinic. The advent of direct oral anticoagulants (DOACs) facilitates OAC therapy without an onerous monitoring regimen. This benefit may result in the more significant adoption of DOACs in areas without a warfarin clinic. This study analysed national administrative data for reimbursed pharmacy claims to assess OAC prescribing from 2010 to 2017 and compared the use of DOACs in areas with warfarin clinics compared to those without. Over the study period, the number of patients on OAC increased by 84%, due to a rapid increase in DOAC prescribing. The findings demonstrate that DOACs have resulted in an increase in the overall uptake of OAC therapy in Ireland. However, the increased utilisation was not evidently related to populations underserved by warfarin clinics., (© 2019 The British Pharmacological Society.)

Aim: The aim of this was to identify and synthesize the evidence underpinning the health policymaking process to inform the development of a health-related policy analysis framework., Design: A mixed methods review using "Best Fit" Framework synthesis., Data Sources: PUBMED and CINAHL+ databases for English language papers published between March 2013 - March 2017., Review Methods: Titles were screened, data abstracted and analysed by two authors at each stage. Findings from included studies were coded against six a priori categories which had been constructed through a preliminary literature review, consultation and consensus., Results: Sixty-eight papers were included. There exists empirical support for six key domains which require to be addressed in the policymaking and analysis process: (1) Context; (2) Process; (3) Content; (4) Stakeholder Consultation; (5) Implementation; and (6) Evaluation. Failure to contextualize and integrate these six domains in problem identification, policy analysis, strategy and policy development, policy enactment and policy implementation is problematic., Conclusion: There is a need to test and refine the constructs linked to the policymaking cycle taking cognizance of the context where these are developed, implemented and evaluated., Impact: This review makes a novel contribution to the synthesis of evidence to inform the policymaking and analysis process. Findings illuminate the complexity of policymaking, the competing pressures involved and the importance of the local, national and international context. These findings have international relevance and provide empirical support for key criteria to guide those involved in context specific policymaking and/or the analysis of existing policy., (© 2019 John Wiley & Sons Ltd.)

Approximately 500 monogenic causes of chronic kidney disease (CKD) have been identified, mainly in pediatric populations. The frequency of monogenic causes among adults with CKD has been less extensively studied. To determine the likelihood of detecting monogenic causes of CKD in adults presenting to nephrology services in Ireland, we conducted whole exome sequencing (WES) in a multi-centre cohort of 114 families including 138 affected individuals with CKD. Affected adults were recruited from 78 families with a positive family history, 16 families with extra-renal features, and 20 families with neither a family history nor extra-renal features. We detected a pathogenic mutation in a known CKD gene in 42 of 114 families (37%). A monogenic cause was identified in 36% of affected families with a positive family history of CKD, 69% of those with extra-renal features, and only 15% of those without a family history or extra-renal features. There was no difference in the rate of genetic diagnosis in individuals with childhood versus adult onset CKD. Among the 42 families in whom a monogenic cause was identified, WES confirmed the clinical diagnosis in 17 (40%), corrected the clinical diagnosis in 9 (22%), and established a diagnosis for the first time in 16 families referred with CKD of unknown etiology (38%). In this multi-centre study of adults with CKD, a molecular genetic diagnosis was established in over one-third of families. In the evolving era of precision medicine, WES may be an important tool to identify the cause of CKD in adults., (Copyright © 2019 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Purpose: The aims of this study were to examine a national database to assess codeine poisonings before and after the new guidance for pharmacists while also evaluating rates of codeine prescriptions following the introduction of restrictions on supply., Methods: Anonymised enquiry data of reported poisoning cases were reviewed for a period from 2005 to 2016 inclusive. The rate of pharmacy claims for codeine containing products was also examined using the national pharmacy claims database. Segmented regression analysis was used to detect changes in poisonings and claims before and after the new guidance., Results: There were 1851 codeine-related poisonings reported over the study period. An annual decline was evident with a significant 33% reduction from 2010 to 2011 (β2 coefficient for level change, 42.1; 95% CI, -68.1 to -16.0; P = 0.006). Following 2011, the declining rate of codeine poisonings plateaued. Analysis of the national pharmacy claims data revealed no change in the reimbursement rate for co-codamol products restricted by the guidance in 2010 (Incidence rate ratio 1.04, 95% CI, 0.997-1.08; P = 0.07). There was no corresponding increase in the reimbursement of alternative opioid medications., Conclusions: New guidance on codeine supply coincided with an initial reduction in reported codeine poisoning cases. This reduction was in keeping with the previous trend. However, this was without an increase in the prevailing rate of prescription claims for these products or potential substitutes. Policymakers may consider further restriction of codeine products to improve public health outcomes., (© 2019 John Wiley & Sons, Ltd.)

The configuration of one's social environment influences the course and experience of depression. Research suggests that experiencing depression is associated with stigmatisation and the concomitant experiencing of discrimination across many facets of social life. This is identified as a particularly important factor in rural communities. Contemporary work is absent in relation to understanding the stigma towards depression in Ireland, and its manifestation in rural Ireland specifically. Evidence is presented which suggests that depression is a significant source of stigmatisation in this setting, and that entrenched views centring upon disability and fear are prevalent.

Background: IgA nephropathy is the most common primary glomerulonephritis worldwide and a significant cause of end-stage renal disease (ESRD). While most cases of IgA nephropathy are considered sporadic, familial cases have been reported., Methods: We performed a national audit of 1,809 patients attending renal clinics and dialysis units to identify a family history among patients with kidney disease. We reviewed all renal biopsies performed at our institution spanning a 30-year period. Paediatric cases were not included., Results: We identified 14 families involving 41 affected individuals with biopsy-proven IgA nephropathy and at least one other member with either biopsy-proven IgA nephropathy or ESRD. Detailed family histories were obtained, medical records reviewed and family pedigrees constructed. Retrospective application of the MESTC criteria to all familial IgA biopsies was performed. Seven families had 2 or more members with biopsy-proven IgA nephropathy, equating to 23 (1.8%) of 1,283 biopsies with IgA nephropathy over the last 30 years. A complex inheritance pattern was observed, with autosomal dominant and autosomal recessive families identified with varying penetrance. There was a male preponderance (68%), and a complex heterogeneity in the clinical and histopathological features of familial IgA patients (age range 16-60 years; creatinine range 60-350 μmol/L). We observed a high rate (66%) of progression to ESRD, with a mean time to progression of 5.13 years (SD 1.8 years; range 2-8 years). Among those patients who had undergone transplantation, recurrence of disease was reported in 5 (50%) cases., Conclusion: These data suggests familial aggregation of IgA nephropathy, confirm the clinical and histopathological heterogeneity and raise the possibility of monogenic inheritance., (© 2018 S. Karger AG, Basel.)

Introduction: Primary focal segmental glomerular sclerosis (p-FSGS) is commonly complicated by recurrence (r-FSGS) post-transplantation. Our objective was to describe Irish outcomes for transplantation after end-stage renal disease (ESRD) due to p-FSGS, specifically rates of, and treatments for, r-FSGS., Patients and Methods: Irish patients with biopsy-proven FSGS were identified from the Irish National Kidney Transplant database (1982-2015). Medical record review was performed to identify predictors of r-FSGS and treatments for r-FSGS. Transplant outcomes were compared to outcomes in all renal transplants performed during the same time period using registry data. Demographic and clinical predictors of r-FSGS were identified. Statistical analysis was performed using Stata (version 13, College Station, TX, USA)., Results: Thirty-eight transplant recipients had biopsy-proven p-FSGS, 16 received a second transplant. A total of 3846 transplants formed the comparator group. r-FSGS complicated 60.5% (23/38) of first transplants. Eighty-six percent (10/12) of patients with previous r-FSGS developed recurrent disease after further transplantation. Patients with p-FSGS receiving a first renal transplant had higher rate of graft failure than those with another cause of ESRD (HR 1.9, 95% CI 1.152-3.139). Sixteen patients received immunotherapy for r-FSGS; 12 (86%) had at least partial response, but two (14%) developed significant complications., Discussion: We demonstrate high rates of r-FSGS and describe modest success from with treatments for r-FSGS., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Aims: To review, discuss and compare nursing and midwifery regulatory and professional bodies' scope of practice and associated decision-making frameworks., Background: Scope of practice in professional nursing and midwifery is an evolving process which needs to be responsive to clinical, service, societal, demographic and fiscal changes. Codes and frameworks offer a system of rules and principles by which the nursing and midwifery professions are expected to regulate members and demonstrate responsibility to society., Design: Discussion paper., Data Sources: Twelve scope of practice and associated decision-making frameworks (January 2000-March 2014)., Implications for Nursing: Two main approaches to the regulation of the scope of practice and associated decision-making frameworks exist internationally. The first approach is policy and regulation driven and behaviour oriented. The second approach is based on notions of autonomous decision-making, professionalism and accountability. The two approaches are not mutually exclusive, but have similar elements with a different emphasis. Both approaches lack explicit recognition of the aesthetic aspects of care and patient choice, which is a fundamental principle of evidence-based practice., Conclusion: Nursing organizations, regulatory authorities and nurses should recognize that scope of practice and the associated responsibility for decision-making provides a very public statement about the status of nursing in a given jurisdiction., (© 2015 John Wiley & Sons Ltd.)

Aim: This paper reports on the qualitative findings from a national review of a nursing and midwifery scope of practice framework., Background: Scope of professional practice frameworks offers a system of rules and principles to regulate its members and demonstrate its responsibility to society. Key issues in reviewing the scope of practice include notions of specialist and advanced practice, accountability, autonomy, competence, supervision, continuing professional development and delegation. Evaluation of scope of practice frameworks has particular application value to nurses, midwives, regulatory bodies and healthcare employers across the globe., Design: A mixed methods approach was used. This included a national survey of nurses and midwives and focus groups and interviews with key stakeholders. The qualitative data are reported in this paper., Methods: Focus groups and interviews were conducted among a convenience purposive sample of key stakeholders, including nurses and midwives working in the widest range of services and settings in 2014. The participants contributed to thirteen focus groups and thirteen interviews., Findings: Six global themes, as follows: Evolution of the nursing and midwifery professions and practice; Scope of practice: understanding and use; Expanding scope of practice; Professional competence; Practice setting and context; Reflections on the current framework., Conclusion: Practitioners understand the scope of professional practice and while some see it as empowering others see it as potentially restrictive. Nurses and midwives are generally willing to expand their scope of practice and see it as resulting in improved patient care, improvement in overall quality of standards and increased job satisfaction., (© 2015 John Wiley & Sons Ltd.)

Purpose: The purpose of this paper is to describe a national, comprehensive quality indicator set to support delivering high-quality clinical care in Irish general practice., Design/methodology/approach: Potential general practice quality indicators were identified through a literature review. A modified two-stage Delphi process was used to rationalise international indicators into an indicator set, involving both experts from key stakeholder groups (general practitioners (GPs), practice nurses, practice managers, patient and health policy representatives) and predominantly randomly selected GPs. An illustrative evaluation approach was used to road test the indicator set and supporting materials., Findings: In total, 80 panellists completed the two Delphi rounds and staff in 13 volunteer practices participated in the road test. The original 171 indicators was reduced to 147 during the Delphi process and further reduced to 68 indicators during the road test. The indicators were set out in 14 sub-domains across three areas (practice infrastructure, practice processes and procedures, and practice staff). Practice staff planned 77 quality improvement activities after their assessment against the indicators and 31 (40 per cent) were completed with 44 (57 per cent) ongoing and two (3 per cent) not advanced after a six-month road test. A General Practice Indicators of Quality indicator set and support materials were produced at the conclusion., Practical Implications: It is important and relatively easy to customise existing quality indicators to a particular setting. The development process can be used to raise awareness, build capacity and drive quality improvement activity in general practices., Originality/value: The authors describe in detail a method to develop general practice quality indicators for a regional or national population from existing validated indicators using consensus, action research and an illuminative evaluation.

Background: Bibliometric analysis of scientific performance within a country or speciality, facilitate the recognition of factors that may further enhance research activity and performance. Our aim was to illicit the current state of Irelands orthopaedic research output in terms of quantity and quality., Methods: We performed a retrospective bibliometric analysis of all Irish orthopaedic publications over the past 5 years, in the top 20 peer-reviewed orthopaedic journals. Utilising the MEDLINE database, each journal was evaluated for articles that were published over the study period. Reviews, editorials, reports and letters were excluded. Each article abstract was analysed for research content, and country of origin. A nation's mean IF was defined by multiplying each journal's IF by the number of articles. Publications per million (PmP) was calculated by dividing the total number of publications by the population of each country., Results: We analysed a total of 25,595 article abstracts. Ireland contributed 109 articles in total (0.42% of all articles), however ranking according to population per million was 10th worldwide. Ireland ranked 18th worldwide in relation to mean impact factor, which was 2.91 over the study period. Ireland published in 16 of the top 20 journals, 9 of these were of European origin, and 1 of the top 5 was of American origin. In total, 61 Irish articles were assignable to clinical orthopaedic units. Clinical based studies (randomised controlled trials, observational, and epidemiology/bibliometric articles) and research based studies (In vivo, In vitro, and biomechanical) numbered 76 (69.7%) and 33 (30.2%) articles, respectively., Conclusion: This study provides a novel overview of current Irish orthopaedic related research, and how our standards translate to the worldwide orthopaedic community. In order to maintain our publication productivity, academic research should continue to be encouraged at post graduate level., (Copyright © 2013 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.)

Introduction: Primary glomerular diseases such as primary focal segmental glomerular sclerosis (FSGS), IgA Nephropathy and membrano-proliferative glomerulonephritis (MPGN) may recur in renal transplants, and can potentially lead to graft failure. The rate of recurrence in second and subsequent renal transplants, following failure of the first graft due to recurrence, is unclear., Methods: A retrospective review of the Irish transplant database from 1982 to 2009 was performed. Patients were included for analysis if their first graft failed due to biopsy-confirmed recurrent glomerular disease (primary FSGS, IgA nephropathy or MPGN) and they underwent subsequent re-transplantation., Results: 3,330 deceased and living renal transplants were performed during the time period in question. 33 patients had a deceased donor renal transplant following recurrence of primary FSGS, IgA nephropathy or MPGN causing first graft failure. Clinically significant disease recurrence was seen in 44% of re-transplants at 10 years. Median second graft survival in this group was 9.1 years. The median graft survival was 10.5 years for all other re-transplants performed in Ireland during the same time period., Conclusion: Clinically significant disease recurrence does not necessarily affect re-transplants following loss of the first graft to disease recurrence. Selected patients who experience first graft failure due to recurrent glomerular disease should not be precluded from receiving a second transplant.

Objective: To report the pregnancy outcomes in Irish female renal transplant recipients on modern maintenance immunosuppression., Methods: The Republic of Ireland transplant database was accessed to identify the patient cohort in question. All female renal transplant recipients whose transplantation was in Ireland before or during their reproductive years were included. A questionnaire was sent to the identified women. A chart review was performed for those women who reported a pregnancy following renal transplantation., Results: Two hundred and ten women met the inclusion criteria. There was a response rate of 70% (n = 148). Eighteen women reported 29 pregnancies. The live birth rate was 76%. The mean gestation of the live births was 36.2 weeks with a mean birth weight of 3.0 kg. There were six cases of pre-eclampsia. Twin pregnancies and those entering pregnancy with a creatinine greater than 135 µmol/l had particularly complicated clinical courses. Four women had not conceived post transplant despite actively trying for over 1 year. Two women utilised assisted fertility methods (in vitro fertilisation), one of whom became pregnant., Conclusions: A significant proportion of women who attempt to conceive following renal transplantation are successful, without the use of assisted fertility. Pregnancy in this setting warrants meticulous multidisciplinary care.

The introduction of new models of care in the Irish maternity services has been recommended by both advocacy groups and strategic reports. Yet there is a dearth of information about what models of care pregnant women want. We surveyed women in early pregnancy who were attending a large Dublin maternity hospital. Demographic and clinical details were recorded from the hospital chart. Of the 501 women, 351 (70%) (352 (70.3%) of women wanted shared antenatal care between their family doctor and either a hospital doctor or midwife. 228 (45.5%) preferred to have their baby delivered in a doctor-led unit, while 215 (42.9%) preferred a midwifery-led unit. Of those 215 (42.9%), 118 (55%) met criteria for suitability. There was minimal demand (1.6%) for home births. Choice was influenced by whether the woman was attending for private care or not. Safety is the most important factor for women when choosing the type of maternity care they want. Pregnant women want a wide range of choices when it comes to models of maternity care. Their choice is strongly influenced by safety considerations, and will be determined in part by risk assessment.

Introduction: H1N1 influenza A, was first described in April 2009. A significant cohort of patients from this outbreak developed acute respiratory distress syndrome or pneumonia. H1N1 has since been transmitted across the world. Little has been described on the renal complications of this illness., Methods: A retrospective review of all patients admitted to our institution with H1N1 infection was carried out from July to November 2009. Renal biochemistry, need for renal replacement therapy and hospital outcome was recorded., Results: Thirty-four patients with H1N1 were admitted. Average length of admission was 10 days (3-84). Eleven patients (32%) developed acute kidney injury (AKI) as defined by the RIFLE criteria (creatinine range 120-610). Four patients required renal replacement therapy, for a range of 10-52 days. Seven patients developed AKI that responded to volume resuscitation. The commonest cause of AKI was sepsis with acute tubular necrosis., Conclusion: This study highlights the significance and frequency of renal complications associated with this illness.

Nephroaenic systemic fibrosis (NSF) is a potentiallv fatal dermatiological condition found exclusively in patients with advanced renal I failure. There is minimal literature regarding the epidemiology and outcomes of patients with NSF in Ireland. A retrospective chart review was performed for all patients with NSF in Ireland. Ireland's experience with the disease was examined in light of international reports. There have been three cases of NSF in Ireland; an area which serves 1915 dialysis patients--giving a point prevalence among Irish end-stage kidney disease patients of 0.002. There was a large variation in disease severity between the three patients. All three patients had significant exposure to gadolinium chelate. Caution with gadolinium administration must be exercised in patients with advanced renal failure.

Reasons for Performing Study: Rhodococcoccus equi is a significant cause of bronchopneumonia in foals worldwide. Infection of the lungs is believed to result from inhalation of virulent R. equi in dust from contaminated environments. A measure of infectious risk in an environment is the level of airborne contamination., Objectives: To assess and compare the level of airborne virulent R. equi in paddocks and stables., Methods: Air samples were collected sequentially over the 2003 foaling season from the paddocks and stables on 3 Irish horse breeding farms affected by R. equi pneumonia. Colony blotting and DNA hybridisation techniques allowed quantitation of virulent R. equi., Results: The odds of detecting airborne virulent R. equi in stables were 173 times greater than in paddocks. The median airborne concentration of virulent R. equi was significantly higher (P < 0.001) in stables than in paddocks on all farms. These observations suggested that stables were high-risk areas for infection., Conclusions and Potential Relevance: Our results indicate that contaminated stables are a significant risk factor in the epidemiology of R. equi pneumonia on horse-breeding farms in a temperate climate, such as in Ireland. Management strategies that improve the air hygiene of stables, through better ventilation, use of less fragile bedding material and the use of fogging agents to reduce the airborne concentration of virulent R. equi, may reduce the incidence and severity of R. equi pneumonia on farms.

Pomphorhynchus laevis is believed on ecological evidence to exist as three strains in the British Isles. However, the strains have never been shown to be capable of being distinguished using morphological characters. A morphological comparison was made between a sample of P. laevis from Salmo trutta in L. Feeagh in the west of Ireland and a sample from Leuciscus cephalus in R. Culm in the south of England. The length and width of the trunk, neck, bulb, proboscis and hooks were measured. The number of hooks per row, the number of rows and the positions of the stoutest and longest hooks were also recorded. A Principal Components Analysis based on the morphological measurements confirmed the separation of the two populations and showed that two characters successfully identified the populations: the position of the stoutest hook and the ratio of numbers of anterior to posterior hooks.

The population biology of the fish acanthocephalan Acanthocephalus clavula was described from 161 wild brown trout, Salmo trutta sampled over a two-year period in Clogher Lake in the west of Ireland. Overall prevalence of the parasite was 86% and the mean abundance was 53 worms per fish. Despite the presence of large numbers of worms in the trout very few females (2%) attained full reproductive maturity. This suggests that trout is an accidental host. A sample of yellow eels, Anguilla anguilla was examined at a different time from the same lake. The prevalence of A. clavula was 97% and the average abundance was 8 worms per fish. In contrast to the situation in trout, the proportion of female worms attaining full reproductive maturity was 61% fulfilling the expected characteristic of a preferred definitive host. The possible explanations for the very high abundance of A. clavula in trout are discussed and include the influence of fluctuations in host populations, host diet and the absence of a potential competitor.

A hypothesis has been erected stating that in the British Isles the acanthocephalan, Pomphorhynchus laevis can be separated into 3 strains, an English, Irish and marine strain. Ecological and morphological evidence exists in support of this hypothesis. An investigation at the molecular level was conducted in order to test the validity of the existing evidence. A mitochondrial gene, subunit I of cytochrome c oxidase was partially sequenced from 3 Irish populations of P. laevis, 1 Scottish population and 3 English populations. P. laevis sequences from brown trout from Ireland, England and Scotland were very similar, showing a mean sequence divergence of 0.7%. Sequences from two populations of P. laevis from English chub and bullhead were also similar to each other (0.35% divergence). These two groups of sequences, the brown trout group and the chub/bullhead group were 2.2% different. These data confirm the existence of at least 2 strains in Ireland and Britain, although there is evidence to suggest that these strains are defined by their host species rather than by their geographical distributions.

The aim of this paper was to test the hypothesis that when the 2 species of Acanthocephalan Pomphorhynchus laevis and Acanthocephalus clavula are found concurrently within the intestine of brown trout under field conditions, they have the potential to interact negatively. Evidence has shown that Acanthocephala are more likely to exhibit negative interactions with their own and other species, under both field and experimental conditions. Furthermore, the likelihood of these interactions is increased in Ireland because of the absence of certain definitive hosts and the fact that concurrent infections by two or more species of Acanthocephala are more commonly observed in fish. Data collected from wild and stocked brown trout and from 2 lakes provided an opportunity to compare the 2 potentially interacting helminth species in their fundamental and realized niche and several pieces of convincing evidence are provided here to support the hypothesis. A significant negative association between the numbers of each species found in individual fish was reported and this was consistent for both wild and stocked trout. Furthermore, an analysis of the proportions of low, moderate and high intensity infections in single and concurrent infections revealed a significant reduction in increasing intensities in concurrent infections compared to single infections. Finally, strikingly different patterns of niche inhabitation were observed, particularly for P. laevis in the presence of A. clavula in wild trout. Results from the niche width analysis also support the observations on average position in single and concurrent infections. The niche width of P. laevis when it co-occurred with A. clavula decreased markedly in high intensity infections compared to low intensity infections.

A data set on intestinal helminth parasites was collected in the course of an 18 year investigation into the biology of eels in Meelick Bay, Lough Derg, River Shannon. This was used to test two hypotheses relating to the composition and structure of intestinal helminth communities, namely that eels in large rivers do not harbour richer and more diverse communities than those in small rivers but that community composition and structure are more stable over time than in small rivers. The helminth community was species poor, with only six species comprising the component community and a maximum infracommunity richness of three species. The community was overwhelmingly dominated by the acanthocephalan Acanthocephalus lucii, reflecting the importance of its intermediate host Asellus aquaticus in the eels' diet. The remaining helminth species contributed to species richness but made very little contribution to community diversity. Population levels of Acanthocephalus lucii fell and remained low between 1992 and 2000, probably reflecting increased movement of eels from other parts of the lough into Meelick Bay. Diversity values were low, but similar to those reported from other rivers in Britain and Europe. The results provided support for both hypotheses and indicated that in respect of richness, diversity and dominance, the helminth communities of eels in the River Shannon were typical of, and comparable to, those of other large rivers throughout Europe.

Staphylococcus epidermidis is a common cause of prosthetic device-related infection in the intensive care unit (ICU). The environmentally regulated ica operon encodes a polysaccharide adhesin which is a key virulence determinant in the development of S. epidermidis biofilms. To evaluate the capacity of ICU S. epidermidis isolates to form biofilm, we measured biofilm production by 18 isolates associated with device-related infection and 20 contaminating isolates that were not associated with clinically diagnosed infection. Biofilm assays were performed in brain-heart infusion (BHI) medium and in BHI supplemented with salt, ethanol or subinhibitory tetracycline, all of which have the potential to promote biofilm formation. Polymerase chain reaction (PCR) was used to screen for the presence of the ica genes. A significant proportion of S. epidermidis strains associated with device-related infections (89%) were found to contain the ica locus compared with 50% of contaminating isolates (P = 0.01). However only four of 26 (15.3%) of all ica-positive isolates were biofilm-positive when grown in BHI medium, indicating that no significant association existed between the presence of the ica locus and biofilm-forming capacity, under standard growth conditions. In contrast the number of ica-positive isolates that were biofilm-positive under stress-inducing growth conditions or in the presence of subinhibitory tetracycline increased significantly to 73% (P = 0.02). These findings suggest that the presence of the ica locus alone is not sufficient for biofilm formation and that regulation of biofilm formation under altered growth conditions, which may exist in the in vivo environment, also plays a possible role in the pathogenesis of biomaterial-related S. epidermidis infections., (Copyright 2002 The Hospital Infection Society)

The aim of the present study was to compare the helminth infra and component communities of wild and stocked brown trout in Lough Feeagh, in the west of Ireland, and also to examine the establishment and development of helminth communities in stocked brown trout. Fish were sampled in May, August and November 1997 and 1998 and an additional sample of wild brown trout was examined in April 1997. In total 217 wild trout and 122 stocked trout were examined. The acanthocephalans Acanthocephalus clavula and Pomphorhynchus laevis were the first parasite species to infect stocked trout in May 1997. In May 1998 both acanthocephalan species along with the trout specialists Eubothrium crassum and Crepidostomumfarionis were the first to infect trout. Mean species richness values for stocked trout increased from May to November, in 1997 and 1998. For wild trout, mean species richness values increased from April to November, in 1997 and decreased from May to November in 1998. The parasite communities of wild trout were richer than those of stocked trout in May of both years. In August the parasite communities recorded for wild and stocked trout were similar in terms of the number of species present but differed in terms of structure, and in November the parasite communities of stocked trout were richer than those of wild trout.

The St Vincent's Cardiac Rehabilitation Programme is based on an out-patient non-institutionalized service with long-term follow-up and home-based exercise programmes. Return to work rates of more than 90% in the 1960s and 1970s were reported but these rates have not been sustained in the past seven years. These secular changes in return to work rates are attributed to recent changes in the social and economic climate in Ireland. Medical, social, demographic, and psychological factors which may influence successful return to work were studied. The previously employed, young patients and those in the higher social groups, patients with less severe attacks and those free from post-infarction angina, reported the best return to work rates. Reasons for failure to return to work could be attributed equally to organic and functional causes. Factors affecting secondary preventive measures, including smoking cessation, desirable dietary and weight changes, and aerobic exercise programmes, were also studied. These changes were best achieved by young patients, white-collar workers, the better educated and urban dwellers. It is concluded that informal out-patient rehabilitation programmes aimed at early return to a normal life, active secondary prevention measures and home-based exercise programmes, are effective and safe in the great majority of patients recovering from myocardial infarction and coronary bypass surgery.

A population of eels Anguilla anguilla from Lough Derg, R. Shannon, Ireland, harbouring infections of both Acanthocephalus lucii and A. anguillae was studied over three years. Both parasite species had the same intermediate host and eels appeared to be the only definitive host for A. anguillae. Throughout the whole period, A. lucii was the dominant parasite, was over-dispersed throughout the eel population and most frequently occurred as a single species infection. A. anguillae was far less common, its dispersion was close to random at most times and it almost invariably occurred as a mixed species infection. The proportions of the two species remained fairly constant over the period. Despite some indication of site selection in the intestine, the distribution of both species overlapped considerably and there was no evidence of competitive displacement of one species by the other or of resource partitioning in space. The life-histories of both species were similar: they infected eels, bred and were lost from fish at the same time of year and there was no indication of resource partitioning in time. Congeneric species of acanthocephalans can thus co-exist in apparently stable equilibrium in fish as predicted and without any evidence of interactions, but it is still considered that exploitation competition between the species may be occurring in eels.

Three splenectomized persons in Yugoslavia, California, and Ireland have been reported to be infected by three different Babesia species; two cases were fatal. In a study of the site where the fatal infection was contracted in Ireland, blood samples from 36 persons who had recently been bitten by ticks were inoculated into two splenectomized calves; no response to Babesia divergens was detected. Field-collected Ixodes ricinus ticks inoculated into another splenectomized calf resulted in fever and recovery of the agent of tick-borne fever (Cytoecetes phagocytophilia). This attempt to determine the presence of latent infection in human beings with intact spleens should be repeated on a larger scale in areas with a demonstrably high incidence of Babesia in ticks and animals. Few places in the world are free of piroplasms; their presence may present a hazard to splenectomized persons or to those whose splenic function is deficient.