Introduction Gross total resection (GTR) improves progression-free and overall survival in CNS WHO grade 2 intramedullary spinal ependymomas. En bloc resection (circumferential dissection and removal in one piece) may maximise GTR while preserving cord integrity, compared with piecemeal resection (internal debulking and fragmentary removal). Aim To compare en bloc and piecemeal resection strategies with respect to GTR rates, neurological outcomes and disease relapse. Methods A retrospective cohort study of 39 patients treated over a 13-year period at a single tertiary centre. Primary outcomes were extent of resection and neurological deterioration (modified McCormick Scale). Disease relapse (post-GTR recurrence or post-STR progression) was a secondary outcome. Results On multivariate analysis, en bloc resection independently predicted GTR (OR 6.54, 95% CI 1.14-37.5, p = 0.035), while the presence of a syrinx was associated with reduced odds of GTR (OR 0.08, p = 0.047). Neurological deterioration (>= 1 mMCS grade) did not differ significantly between techniques (36.4% vs 58.8%, p=NS). Disease relapse occurred in five patients and was strongly associated with residual tumour (OR 44.4, p = 0.003). Conclusion Residual disease was the dominant predictor of relapse. En bloc resection was associated with higher GTR rates without evidence of increased neurological morbidity, identifying surgical strategy as a potentially modifiable determinant of oncological outcome. These findings are hypothesis-generating and support prospective, multicentre studies to define evidence-based resection strategies.

BACKGROUND:It has been over 60 years since the first kidney transplant in Ireland was carried out in 1964. We examined the Irish cohort of individuals with allografts functioning for more than 40 years. We sought to describe the features of these patients and identify clinical factors associated with ultra-long-term allograft survival. METHODS:This retrospective analysis of the Irish National Kidney Transplant Registry included kidney transplants performed in Ireland between January 1st, 1970, and March 31st, 1983. Follow-up analysis was until March 31st, 2023. RESULTS:There were 428 transplants in 394 patients. Longitudinal data were available for 390 (98.9%) patients. Thirty-three (33/428, 7.7%) kidney transplant grafts survived for 40 years or more, with 25 grafts functioning at date of analysis. Multivariable analysis identified transplant type-living donor versus deceased donor (odds ratio [OR] 3.51, confidence interval [CI] 1.17-11.1, P = .027) as significantly associated with long-term graft survival. The median serum creatinine of patients with surviving transplants was 107 µmol/L (range 66-322 µmol/L). Non-melanoma skin cancer was common, affecting 22/33 (67%) patients. CONCLUSIONS:For ultra-long survivors, graft function is excellent if patients are maintained on low-level immunosuppression. Living donor transplantation, but not donor or recipient age or sex, was associated with improved graft survival.

BACKGROUND:Bloodstream infections cause significant complications for haemodialysis (HD) patients and increase healthcare costs. Infection prevention and control bundles are continually evolving to reduce bloodstream infections. This study aims to describe the change in the microbiology of HD bloodstream infections at our centre. We also describe how changes in infection prevention and control bundles are associated with changes in the microbiology of bloodstream infections in HD patients. METHODS:We conducted a retrospective longitudinal cohort study and analysed blood cultures and clinical data from 2009 to 2023 in a large Irish hospital. Data were collected from the electronic patient records. Positive blood cultures were categorised by microorganism. Episodes of bloodstream infection per 100 patient-years were calculated at yearly intervals and compared over time. An interrupted time series analysis incorporating incidence rate ratios (IRR) was performed to assess the impact of three infection prevention and control interventions on bloodstream infections in our cohort. RESULTS:A total of 1248 HD patients were included in the study, among whom 522 episodes of bloodstream infections occurred.The proportion of bloodstream infections caused by staphylococci (Staphylococcus aureus and coagulase-negative staphylococcus) decreased over the course of the study (60% in 2009-2011 to 34.8% in 2021-2023) and while the proportion caused by gram-negative rods rose (18.5% in 2009-2011 to 43.9% In 2021-2023).Age >34 years, male gender (IRR 1.43, P < .001), and access via central venous catheter (CVC) were all associated with increased risk of bloodstream infections.The opening of a new dialysis unit had the greatest effect on the reduction of bloodstream infections ((S. aureus IRR = 0.42, P < .001) and (gram-negative rods IRR = 0.49, P .048)). CONCLUSIONS:The epidemiology of bloodstream infections in HD patients is changing. There has been a shift from gram-positive-predominant to gram-negative-predominant bloodstream infections among our HD cohort.The opening of a new dialysis unit had the greatest temporal association with the decrease in both S. aureus and gram-negative rod bloodstream infections in this study.

Previously published literature from our institution found that patients with a fragility hip fracture were estimated to have a 4–10

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic nephropathy and has striking familial variability of disease severity. Methods: To better comprehend familial phenotypic variability, we analyzed clinical and pedigree data on 92 unrelated ADPKD kindreds with >= 2 affected individuals (N = 292) from an Irish population. All probands underwent genetic sequencing. Age at onset of kidney failure (KF), decline in estimated glomerular filtration rate (eGFR), predicting renal outcome in polycystic kidney disease (PROPKD) score, and imaging criteria were used to assess and grade disease severity as mild, intermediate, or severe. One mild and 1 severe case per family defined marked intrafamilial variability of disease severity. Results: Marked intrafamilial variability was observed in at least 13% of the 92 families, with a higher proportion of families carrying PKD1-nontruncating (PKD1-NT) variants. In families with >= 2 members affected by KF, the average intrafamilial age difference was 7 years, and there was no observed difference in intrafamilial variability of age at KF between allelic groups. The prespecified criteria showed marked familial variability in 7.7%, 8.4%, and 24% for age at KF, the PROPKD score, and imaging criteria, respectively. In our multivariate mixed-effects model, the intrafamilial variability in kidney survival was independent of the measured genotypic factors associated with prognosis and survival (P = <0.001). Conclusion: Using objective measures, we quantified marked intrafamilial variability in ADPKD disease phenotype in at least 13% of families. Our findings indicate that intrafamilial phenotypic variability remains incompletely understood and necessitates a more thorough identification of relevant clinical and genotypic factors.

Background: Solid organ transplant recipients are recognized to carry a high burden of malignancy and frequently this cancer develops in the head and neck region. Furthermore, cancer of the head and neck post-transplant carries a significantly increased mortality. In this study, we aim to conduct a national retrospective cohort study to investigate the impact of head and neck cancer in terms of frequency and mortality in a large group of solid organ transplant recipients over a 20 year time span and compare the mortality in transplant patients to non-transplant patients with head and neck cancer.Methods: Patients in the Republic of Ireland who underwent solid organ transplantation between 1994 and 2014 who developed post-transplant head and neck malignancy were identified from the records of two prospective, national databases (National Cancer Registry of Ireland (NCRI) and The Irish Transplant Cancer Group database) working in conjunction with each other. Incidence of head and neck malignancy post-transplant was compared with the general population by means of standardised incidence ratios (SIR). Cumulative incidence of all cause and cancer related mortality from head and neck keratinocytic was undertaken by a competing risks analysis.Results: A total of 3346 solid organ transplant recipients were identified, 2382 (71.2 %) kidney, 562 (16.8 %) liver, 214 (6.4 %) cardiac and 188 (5.6 %) lung. During the period of follow up of 428 patients developed head and neck cancer, representing (12.8 %) of the population. 97 % of these patients developed keratinocytic cancers, specifically, of head and neck. The frequency of post-transplant head and neck cancer was related to the duration of immunosuppression with 14 % of patients developing cancer at 10 years and 20 % having developed at least one cancer by 15 years. 12 (3 %) patients developed non-cutaneous head and neck malignancy. 10 (0.3 %) patients died due to head and neck keratinocytic malignancy post-transplant. Competing risk analysis demonstrated that organ transplantation conferred a strong independent effect of death, compared to non-transplant patients with head and neck keratinocytes. This applied specifically for kidney (HR 4.4, 95 % CI 2.5-7.8) and heart transplants (HR 6.5, 95 % CI 2.1-19.9), and overall, across the four transplant categories (P < 0.001). The SIR of developing keratinocyte cancer varied based on primary tumor site, gender, and type of transplant organ.Conclusion: Transplant patients demonstrate a particularly high rate of head and neck keratinocyte cancer with a very high rate of associated mortality. Physicians should be cognizant of the increased rate of malignancy in this population and monitor for red flag signs/symptoms.

Background: Early-onset scoliosis (EOS) is frequently associated with complex spine and chest wall deformities that may lead to severe cardiopulmonary impairment and malnutrition. The aim of this study is to evaluate the change in the nutritional status of EOS patients after treatment with magnetically controlled growing rod instrumentation (MCGR) in a single center. Methods: We prospectively collected data of patients treated with MCGR for EOS in a single center. Exclusion criteria were <2 years’ follow-up and incomplete weight-for-age Z-scores (WAZ) data. Preoperative and postoperative WAZ, radiographic parameters, including major coronal curve, kyphosis angle, space available for lung ratios, thoracic height, and unplanned returns to the operating room (UPROR), were analyzed. SD and 95% Confidence intervals (CI) are presented with means. Results: Sixty-eight patients (37 males/31 females) were included. The mean age at surgery was 8.2 years (SD 2.8, range 1.8–14.2), and the mean follow-up time was 3.8 years (SD 1.0, range 2.1–6.8). The study population was categorized by the primary diagnosis as follows: 23 neuromuscular, 18 idiopathic, 15 congenital, and 12 syndromic patients. The major coronal curve improved between the preoperative and latest visits by 40% ( P <0.005, SD 27, CI 33–47), while the space available for lung ratios improved by 8% ( P <0.005, SD 13, CI 5–12). Thoracic height increased by 25% ( P <0.005, SD 13, CI 22–28), and kyphosis angle decreased by 25% ( P <0.005, SD 26, CI 9–39). Eighteen patients (27%) required a total of 53 UPRORs. WAZ improved significantly between the preoperative and the latest follow-up ( P =0.005). Regression analysis showed WAZ improvements were most significant in the underweight patients and the Idiopathic or Syndromic EOS patients. UPROR was not associated with deterioration in WAZ. Conclusions: Treatment of EOS patients with MCGR resulted in an improvement in nutritional status, as evidenced by the significant increase in WAZ. Underweight, Idiopathic and Syndromic EOS patients, and those who required UPROR all had significant improvement in their WAZ with MCGR treatment. Level of Evidence: Therapeutic Study—Level II.

BACKGROUND:A fundamental tenent of treating developmental dysplasia of the hip is to identify patients with dislocated hips early so as to avoid the long-term sequelae of late diagnosis. The aim of this study was to develop a readily useable triage tool for patients with suspected hip dislocation, based on the clinical history and examination findings of the referring practitioner.METHODS:All primary care referrals (n=934) over a 3-year period for suspected developmental dysplasia of the hip to a tertiary pediatric center were evaluated. Defined parameters with respect to history and clinical examination were evaluated. Multivariable logistic regression was used to establish predictors of hip dislocation, and from this a predictive model was derived which incorporated significant predictors of dislocation. An illustrative nomogram translated this predictive model into a usable numerical scoring system called the Children's Hip Prediction score, which estimates probability of hip dislocation.RESULTS:There were 97 dislocated hips in 85 patients. The final predictive model included age, sex, family history, breech, gait concerns, decreased abduction, leg length discrepancy, and medical/neurological syndrome. The area under receiver operating curve for the model is 0.761. A Children's Hip Prediction score of≥5 corresponds to a sensitivity of 76.3% and a score of≥15 has a specificity of 97.8%, corresponding to an odds ratio of 27.3 for increased risk of dislocation.CONCLUSION:We found that a novel clinical prediction score, based on readily available history and examination parameters strongly predicted risk of dislocations in hip dysplasia referral. It is hoped that this tool could be utilized to optimize resource allocation and may be of particular benefit in less well-resourced health care systems.LEVEL OF EVIDENCE:Level II.

Background and purpose: Currently the Irish Hip Fracture Standards [IHFS] recommend a Time-to-Surgery [TTS] of within 48 h of admission. The aim of our research is to determine if there was a statistically significant relationship between TTS and 30-day or one-year mortality and to assess whether a 48 h window for surgery is still the most appropriate recommendation. Methods used: This was a single-hospital retrospective review of all of the fragility hip fractures between 1st January 2013 and 31st December 2017. Patient demographics were described using descriptive statistics. Dependent variables of interest were 30-day mortality and one-year mortality. Independent predictor variables analysed included age, ASA grade, fracture type, surgery performed, anaesthesia administered, length of stay and TTS (hours as an interval variable), TTS in less than 36 h (binary variable) and TTS in less than 48 h (binary variable). When the significant predictor variables were identified, in order to control for confounder variables, a multivariate regression analysis was performed to identify which predictors were still significantly associated with the outcome variables even after controlling for all other known confounder variables. Results: In total, 806 patients were identified. TTS within 36 h was predictive of a significantly lower 30-day mortality when compared to those undergoing surgery after 36 h (p = 0.031). In contrast, TTS within 48 h did not demonstrate a significantly lower 30-day mortality when compared to those undergoing surgery after 48 h (p = 0.104). On multivariate regression analysis, TTS <36 h (p = 0.011) and age (p < 0.0001) were all independently predictive of 30-day mortality. On multivariate regression analysis, both age (p < 0.0001) and TTS < 36 h (p = 0.002) were significantly predictive of one-year mortality. Conclusion: Performing hip fracture surgery within 36 h confers a significant reduction in both 30-day and one-year mortality rates when compared to patients undergoing surgery outside of this time frame. A 36-h window also appears to be superior to a 48-h window because performing surgery within 48 h has no significant impact on the reduction of 30-day mortality rates. We recommend that national guidelines reflect these important findings. (C) 2021 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.

Background: Fragility hip fracture patients are vulnerable to high rates of short-term mortality, which may have been exacerbated by the coronavirus disease 2019 (COVID-19) pandemic. This study will assess the effect of COVID-19 on 30-day mortality rates amongst a group of Irish hip fracture patients. Additionally, patient demographics, length of stay, admission haematological parameters, fracture type and surgical procedure will be assessed. Methods: A multicentre, observational, retrospective study of hip fracture patients (n=1,017) admitted to six Dublin teaching hospitals during the COVID-19 pandemic was performed. For comparative purposes, equivalent data was retrospectively collected relating to hip fracture patients admitted to the same teaching hospitals in 2019. Results: There were 481 patients admitted during the 2020 study period, compared with 536 patients in 2019. Approximately sixty-six percent of patients were female and the mean patient age was 77.6 years. Recorded 30-day mortality rates were 5.4% in 2020 and 4.3% in 2019 (P=0.338). There was an insignificant decrease in patients’ mean length of stay (17.85 days in 2020 vs. 18.82 days in 2019; P=0.106). Advancing age (P=0.021), male gender (P=0.019), low admission haemoglobin (P=0.024) and high admission white cell count (P=0.019) were all associated with 30-day mortality. Conclusions: We found no significant difference in 30-day mortality rates amongst our cohort of hip fracture patients during the COVID-19 pandemic. Advancing age, male gender, anaemia at admission and leucocytosis at admission were associated with increased 30-day mortality. The continuation of COVID-19 related safety protocols in the treatment of hip fracture patients is essential in maintaining a safe hip fracture service.

Fragility hip fractures are common and costly. Secondary fracture prevention is a treatment goal following hip fracture; however, the number of those that proceed to fracture their contralateral hip in Ireland is unknown. There are plans to introduce a Fracture Liaison Service Database in Ireland which will aim to prevent secondary fractures. To establish a baseline figure for secondary hip fractures, the injury radiographs of 1284 patients from 6 teaching hospitals over a 1-year period were reviewed. Irish Hip Fracture Datasheets and corresponding injury radiographs were reviewed locally for all hip fractures within each respective teaching hospital for a 1-year period (2019). A total of 8.7% of all fragility hip fractures across the 6 hospitals were secondary hip fractures (range 4.9–11.5%). 46% occurred within years 1 to 3 following index hip fracture. Forty-eight per cent of patients were started on bone protection medications following their second hip fracture. Approximately 1 in 11 hip fractures treated across the 6 teaching hospitals assessed in 2019 was a patient’s second hip fracture. We advocate for the widespread availability of Fracture Liaison Services to patients throughout Ireland to assist secondary fracture prevention.

Background. The role of kidney volume measurement in predicting the donor and recipient kidney function is not clear. Methods. We measured kidney volume bilaterally in living kidney donors using CT angiography and assessed the association with the donor remaining kidney and recipient kidney (donated kidney) function at 1 year after kidney transplantation. Donor volume was categorized into tertiles based on lowest, middle, and highest volume. Results. There were 166 living donor and recipient pairs. The mean donor age was 44.8 years (SD ± 10.8), and donor mean BMI was 25.5 (SD ± 2.9). The recipients of living donor kidneys were 64% male and had a mean age of 43.5 years (SD ± 13.3). Six percent of patients experienced an episode of cellular rejection and were maintained on dialysis for a mean of 18 months (13–32) prior to transplant. Kidney volume was divided into tertiles based on lowest, middle, and highest volume. Kidney volume median (range) in tertiles 1, 2, and 3 was 124 (89–135 ml), 155 (136–164 ml), and 184 (165–240 ml) with donor eGFR ml/min (adjusted for body surface area expressed as ml/min/1.73 m2) at the time of donation in each tertile, 109 (93–129), 110 (92–132), and 101 ml/min (84–117). The median (IQR) eGFR in tertiles 1 to 3 in kidney recipients at 1 year after donation was 54 (44–67), 62 (50–75), and 63 ml/min (58–79), respectively. The median (IQR) eGFR in tertiles 1 to 3 in the remaining kidney of donors at 1 year after donation was 59 (53–66), 65 (57–72), and 65 ml/min (56–73), respectively. Conclusion. Bigger kidney volume was associated with better eGFR at 1 year after transplant in the recipient and marginally in the donor remaining kidney.

This study aims to evaluate allograft and patient outcomes among recipients of kidney transplants after non‐renal solid organ transplants. We also aim to compare our findings with recipients of a repeat kidney transplant.

The survival of incident dialysis patients’ end-stage kidney disease in some European and American has been reported to improve in modern era compared to earlier periods. However, in Ireland, this has not been well documented. To investigate the survival outcomes of incident end-stage kidney failure dialysis patients in a tertiary center over a 24-year period, 1993–2017. A retrospective analysis was carried out utilizing the Beaumont Hospital Renal Database. Consecutive adults with incident dialysis were analyzed. Kaplan-Meier methods and the estimated mean survival times were used to evaluate survival at successive 4-year periods of time. In total, 2106 patients were included, of whom 830 underwent subsequent renal transplantation during follow-up. During the study period, from 1993 up to 2017, the mean patients’ age increased from 56.3 ± 17.4 in 1993–1996 to 60.6 ± 18.3 in 2014–2017. There was an overall decrement in mortality over successive time intervals which were mirrored by the improvements in median survival after commencement of dialysis treatment from 6.14 years during 1993–1996 to 8.01 years during 2009–2012. Patients’ survival has steadily improved, with the 5-year survival has risen over time, by almost 15%. This positive signal persisted and became more pronounced after adjusting Kaplan-Meier curve to age, where the 5-year survival estimates were exceeding 80% in 2014–2017. Survival rates among incident dialysis patients have improved progressively between 1993 and 2017 in Beaumont Hospital in Dublin, Ireland. The factors which led to this improvement are not entirely clear, but likely to be multifactorial.

INTRODUCTION Few studies investigate significant perioperative predictors for long-term renal allograft survival after SKT. We compared long term survival following SKT with primary kidney transplant and determined predictors of renal allograft failure after SKT. METHODS Outcomes of all primary or second kidney transplant recipients at a national kidney transplant center between 1993 and 2017 were reviewed. The primary outcomes measurements were renal allograft survival for both first and second kidney transplants. Secondary outcome measurements were incidence of delayed graft function (DGF), incidence of acute rejection (AR) and predictors for renal allograft survival in SKT recipients. RESULTS In total, there were 392 SKTs and 2,748 primary kidney transplants performed between 1993 and 2017. The 1-, 5- and 10-year death censored graft survival for deceased donor recipients were 95.3%, 88.7% and 78.2% for primary kidney transplant and 94.9%, 87.1% and 74.9% for SKT(p=0.0288). Survival of primary renal allograft <6 years (HR0.6, p=0.017), AR episodes (HR1.6, p=0.031), DGF (HR 2.0, p=0.005) and HLA-DR MM (HR 1.7, p=0.018) were independent predictors of long-term renal allograft failure after SKT. CONCLUSION These findings may provide important information on long-term survival outcomes after SKT and for identifying patients at risk for long-term renal allograft failure after SKT.