Familial Variability of Disease Severity in Adult Patients With ADPKD.

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 ( = 292) from an Irish population. All probands underwent genetic sequencing.

Weight Gain and Nutritional Improvement After Magnetically Controlled Growing Rod Surgery in Early-Onset Scoliosis: A Single Centre Analysis of 68 Patients.

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.

High frequency and mortality of head and neck malignancy in organ transplant recipients in Ireland - A national cohort study.

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.

Children's Hip Predictive (CHiP) Score: A Triage Tool for Hip Dislocation in Children Referred With Suspected Hip Dysplasia.

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.

Is Bigger Better? Living Donor Kidney Volume as Measured by the Donor CT Angiogram in Predicting Donor and Recipient eGFR after Living Donor Kidney Transplantation.

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.

Hip fracture surgery within 36 hours reduces both 30-day and 1-year mortality rates.

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.

Calcium and phosphate levels after kidney transplantation and long-term patient and allograft survival.

Background: Non-traditional cardiovascular risk factors, including calcium and phosphate derangement, may play a role in mortality in renal transplant. The data regarding this effect are conflicting. Our aim was to assess the impact of calcium and phosphate derangements in the first 90 days post-transplant on allograft and recipient outcomes.

A multi-site review of second hip fractures across 6 Dublin teaching hospitals.

Introduction: 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.

The Irish experience of kidney transplantation among recipients with prior non-renal solid organ transplants: A retrospective study on short- and long-term outcomes.

Background: 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.

Methods: We performed an analysis on kidney transplant recipients who underwent kidney transplantation after a non-renal solid organ transplant.

A retrospective application of the pulmonary embolism rule out criteria (PERC) of the American College of Physicians would reduce the number of CTPAS by 6% without a false negative in an Irish hospital.

Background: Internationally, the number of computerised tomographic pulmonary angiographies (CTPAs) being performed to rule out pulmonary embolism (PE) has caused some concern.

Aim: This study was performed to assess if the application of Pulmonary Embolism Rule-out Criteria (PERC) in an Irish Emergency Department (ED) would have helped to safely reduce the number of D-dimer assays and computed tomographic pulmonary angiographies (CTPAs) ordered.

Methods: The PERC was retrospectively calculated in all patients who underwent CTPA for possible PE.

The diagnostic utility of late night salivary cortisol (LNSF) and cortisone (LNSE) in Cushing's syndrome.

Background: Measurement of late night salivary cortisol (LNSF) is useful in the identification of cyclical Cushing's syndrome (CS); the usefulness of its metabolite cortisone (late night salivary cortisone, LNSE) is less well described.

Aim: The aim of this study was to determine the utility of measuring LNSE in patients with confirmed CS compared with other diagnostic tests and to analyse serial LNSF measurements for evidence of variable hormonogenesis.

Methods: This was a retrospective observational study including patients with confirmed CS in whom LNSF and LNSE were measured.

Cancer survival in kidney transplant recipients in Ireland.

Background: Transplantation is a well-known risk factor for malignancy. However, outcomes of cancer in transplant recipients compared with non-transplant recipients are less well studied. We aim to study the survival in kidney transplant recipients who develop cancer and compare this with cancer outcomes in the general population.

The impact of switching to mTOR inhibitor-based immunosuppression on long-term non-melanoma skin cancer incidence and renal function in kidney and liver transplant recipients.

Solid organ transplantation is associated with increased risk of non-melanoma skin cancer. Studies with short follow up times have suggested a reduced occurrence of these cancers in recipients treated with mammalian target of rapamycin inhibitors as maintenance immunosuppression. We aimed to describe the occurrence of skin cancers in renal and liver transplant recipients switched from calcineurin inhibitor to sirolimus-based regimes.

Predictors of long-term renal allograft survival after second kidney transplantation.

Introduction: Few studies investigate significant perioperative predictors for long-term renal allograft survival after second kidney transplant (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.

Risk factors and long-term consequences of new-onset diabetes after renal transplantation.

Background: New-onset diabetes after transplant (NODAT) confers risk of diabetes-related complications as well as a threat to graft function and overall patient survival. The reported incidence of NODAT varies from 14 to 37% in renal transplant recipients worldwide; however, NODAT is yet to be studied in the Irish renal transplant population.

Aims: Primary aims of this project were to estimate the incidence, to determine associated risk factors and to assess the long-term consequences of NODAT on graft survival and patient survival in the Irish renal transplant population.

Assessing the discrimination of the Kidney Donor Risk Index/Kidney Donor Profile Index scores for allograft failure and estimated glomerular filtration rate in Ireland's National Kidney Transplant Programme.

Background: The Kidney Donor Risk Index (KDRI)/Kidney Donor Profile Index (KDPI) is relied upon for donor organ allocation in the USA, based on its association with graft failure in time-to-event models. However, the KDRI/KDPI has not been extensively evaluated in terms of predictive metrics for graft failure and allograft estimated glomerular filtration rate (eGFR) outside of the USA.

Methods: We performed a retrospective analysis of outcomes in the Irish National Kidney Transplant Service Registry for the years 2006-13.

Post-transplant malignancy in solid organ transplant recipients in Ireland, The Irish Transplant Cancer Group.

Objective: Solid organ transplant recipients are at increased risk of cancer compared to the general population. To date, this risk in Ireland has not been investigated. We conducted a national registry study of cancer incidence following solid organ transplantation.

Warm Ischemia Time at Vascular Anastomosis is an Independent Predictor for Delayed Graft Function in Kidney Transplant Recipients.

Objectives: Delayed graft function after kidney transplant can affect patient and graft survival, resulting in prolonged hospital stay and need for dialysis. Ischemia times during organ procurement and reanastomosis at transplant are key factors in delayed graft function.

Materials And Methods: We analyzed all living- and deceased-donor renal transplants in Ireland over a 33-month period, with effect of warm ischemia time during anastomosis on delayed graft function being the primary outcome.

Progressive improvement in short-, medium- and long-term graft survival in kidney transplantation patients in Ireland - a retrospective study.

It is often quoted that while short-term graft survival in kidney transplantation has improved in recent years, it has not translated into a commensurate improvement in long-term graft survival. We considered whether this was true of the entire experience of the national kidney transplant program in Ireland. A retrospective analysis of the National Kidney Transplant Service (NKTS) database was undertaken to investigate patient and graft survival for all adult first deceased donor kidney transplant recipients in Ireland, 1971-2015.

Variation in Nonmelanoma Skin Cancer Incidence by Treatment Modality Among Patients Receiving Multiple Kidney Transplants.

Importance: Existing data suggest that nonmelanoma skin cancer (NMSC) is more common in renal transplant recipients than in maintenance dialysis patients. However, whether the risk of NMSC varies as the treatment modality for end-stage kidney disease (ESKD) changes between dialysis and transplantation is not well described.

Objective: To determine whether the incidence of NMSC is attenuated during periods of graft loss with a return to dialysis in those who receive multiple kidney transplants.

A comparative analysis of survival of patients on dialysis and after kidney transplantation.

Background: Kidney transplant survival benefits are not observed for around 8 months after transplantation because of a higher complications rate in early post-transplant periods. This study compares survival of patients awaiting transplantation with survival of transplant recipients and non-listed dialysis patients in Ireland.

Methods: In this retrospective analysis, the relative-risk (RR) of death was assessed with time-dependent, non-proportional hazards analysis, with adjustment for age, cause of end-stage kidney disease (ESKD), time from first treatment for ESKD to placement on the waiting list and year of initial placement on the list.

The impact of donor and recipient weight incompatibility on renal transplant outcomes.

Background: Donor/recipient size mismatching and correlation to allograft outcome remains poorly defined. This study assessed the impact of donor body weight (DBW) to recipient body weight (RBW) ratio on allograft function and survival.

Methods: A total of 898 deceased donor renal transplant recipients were included in the study.