Search

Background For 10 consecutive years, the ESPN/ERA-EDTA Registry has included data on children with stage 5 chronic kidney disease (CKD 5) receiving kidney replacement therapy (KRT) in Europe. We examined trends in incidence and prevalence of KRT and patient survival. Methods We included all children aged Results Incidence of children commencing KRT Conclusions We found a stable incidence and increasing prevalence of European children on KRT 2007–2016. Five-year patient survival was good and was unchanged over time. These data can inform patients and healthcare providers and aid health policy makers on future resource planning of pediatric KRT in Europe.

Background Gentamicin is one of the most commonly reported medications associated with drug errors amongst paediatric inpatients.1 Such errors include inappropriate dosing, insufficient monitoring and lack of awareness of its role in acute kidney injury (AKI). As with other aminoglycosides, gentamicin side-effects are dose-related, highlighting the significance of dose calculation and therapeutic monitoring. Collaborative services is one of the key recommendations from the RCPCH standards for paediatric care.2 Within Northern Ireland there is a well-established regional neonatal gentamicin prescription chart, however, it has previously lacked a paediatric equivalent. Lack of standardisation of practice across units is a common source of frustration for paediatric trainees and trainers. It also contributes to variations in care across Northern Ireland. Objectives To improve gentamicin prescription, drug monitoring and avoidance of nephrotoxicity, through the design, implementation and evaluation of a standardised gentamicin prescription chart for children (one month to sixteen years old). Particular emphasis was placed on dose adjustment and close monitoring in renal impairment, and ideal-body-weight (IBW) for height prescribing in grossly oedematous or overweight children. Additionally, a stronger focus was placed on antibiotic stewardship. For instance, following two gentamicin doses the prescriber is prompted to review diagnosis, culture results and on-going antimicrobial indication. A further prompt for Paediatric Infectious Diseases discussion is highlighted in treatment courses exceeding five doses. Methods A rapid-fire PDSA QI approach was employed, with contribution from Paediatric Infectious Diseases, Paediatric Nephrology and Pharmacy departments. The team pursued this project during the COVID-19 pandemic, despite some initial colleague hesitancies. Throughout implementation, the chart design, content and accessibility were regularly scrutinised. An initial three month pilot was conducted in RBHSC PICU & surgical ward (August- November 2020), prior to hospital-wide adoption. Pre- and post- implementation multidisciplinary education sessions helped to embed its clinical use and facilitate user feedback. Following pilot, the chart was amended to include specific instructions to review both anaesthetic and emergency department records, to ensure gentamicin doses weren't missed. An audit of toxic gentamicin levels (≥1), renal function monitoring and associated AKI was conducted, comparing serum gentamicin levels sent in the 6 months prior to and following chart introduction. Quantitative and qualitative staff feedback was also obtained. Results Audit data showed improvement in renal function monitoring (84.6% to 100%) and associated reduction in AKI (33.4% to 22.2%), following chart introduction. However, similar levels of gentamicin toxicity were encountered before and after chart implementation (9.8% and 10.7% of all gentamicin results respectively). Staff feedback was overwhelmingly positive, with 100% of prescribers agreeing the chart enhanced their knowledge of therapeutic drug monitoring, and prescription and monitoring in renal impairment. Furthermore, regional implementation was supported by all survey respondents. New relationships with laboratory colleagues has facilitated the development of an auto-analysis function to process creatinine results when serum gentamicin levels are requested;this will help to limit clinician variability and may prompt enhanced AKI recognition. Conclusions A collaborative, multi-professional approach to a standardised gentamicin prescription chart will help to harmonise paediatric clinical care throughout Northern Ireland and may contribute to improved antimicrobial prescribing, monitoring and stewardship.

* The median height z-score for paediatric patients on dialysis was −2.1 and for those with a functioning transplant −1.3. Children transplanted before the age of 12 years improved their height z-score over the subsequent five years, whereas those older than 12 maintained their height z-score, with all transplanted patients having a similar median height z-score after five years of starting renalreplacement therapy (RRT).* The median weight z-score for children on dialysis was −1.4 whereas children with a functioning transplant had a near normal weight for age and sex witha median z-score of −0.3.* Of those with data, 75% of the prevalent paediatric RRT population had one or more ‘traditional’ risk factors for cardiovascular disease, with 1 in 10 having all three risk factors present.* For the 10 centres reporting quarterly laboratory data, the average creatinine in transplant patients was 79 μmol/L; dialysis patients had normal average anaemia and acidosis markers and evidence of secondary hyperparathyroidism with an average PTH of 17.3 pmol/L.* For transplant patients, 80% achieved the systolic blood pressure (SBP) standard and 93% achieved the haemoglobin standard.* For haemodialysis patients, 57% achieved the SBP standard, 62% achieved the haemoglobin standard, 82% achieved the calcium standard, 51% achieved the phosphate standard and 39% achieved the parathyroid hormone (PTH) standard.* For peritoneal dialysis patients, 70% achieved the SBP standard, 77% achieved the haemoglobin standard, 72% achieved the calcium standard, 54% achieved the phosphate standard and 33% achieved the PTH standard.

* A total of 917 children and young people under 18 years with established renal failure (ERF) were receiving treatment at paediatric nephrology centres in 2014.* At the census date (31st December 2014), 79.3% of prevalent paediatric patients aged * In patients aged * The most common primary renal diagnosis was renal dysplasia ± reflux, present in 32.6% of prevalent paediatric patients aged * About a third of patients had one or more reported comorbidity at onset of renal replacement therapy (RRT).* The improvement in rates of pre-emptive transplantation for those referred early has been maintained over the last 10 years at 37.5%, compared to 27.4% in 2000–2004.* At transfer to adult services, 90.3% of patients had a functioning kidney transplant.* Survival during childhood amongst children commencing RRT was the lowest in those aged less than two years compared to those aged 12 to less than 16 years with a hazard ratio of 4.1 (confidence interval 2.2–8.0), and in those receiving dialysis compared to having a functioning transplant with a hazard ratio of 6.3 (confidence interval 3.9–10.2)

Antiglomerular basement membrane (anti-GBM) antibody disease is uncommon in the pediatric population. There are no cases in the literature describing the development of anti-GBM disease following XGP or nephrectomy. We report the case of a 7-year-old boy with no past history of urological illness, treated with antimicrobials and nephrectomy for diffuse, unilateral xanthogranulomatous pyelonephritis (XGP). Renal function and ultrasound scan of the contralateral kidney postoperatively were normal. Three months later, the child represented in acute renal failure with rapidly progressive glomerulonephritis requiring hemodialysis. Renal biopsy showed severe crescentic glomerulonephritis with 95% of glomeruli demonstrating circumferential cellular crescents. Strong linear IgG staining of the glomerular basement membranes was present, in keeping with anti-GBM disease. Circulating anti-GBM antibodies were positive. Treatment with plasma exchange, methylprednisolone, and cyclophosphamide led to normalization of anti-GBM antibody titers. Frequency of hemodialysis was reduced as renal function improved, and he is currently independent of dialysis with estimated glomerular filtration rate 20.7 mls/min/1.73 m2. Case studies in the adult literature have reported the development of a rapidly progressive anti-GBM antibody-induced glomerulonephritis following renal surgery where patients expressed HLA DR2/HLA DR15 major histocompatibility (MHC) antigens. Of note, our patient also expresses the HLA DR15 MHC antigen.

The management of neonatal autosomal recessive polycystic kidney disease (ARPKD) complicated by severe pulmonary insufficiency presents complex clinical challenges. Where massive nephromegaly exists, early bilateral nephrectomy, supportive peritoneal dialysis and early aggressive nutrition can minimise infant mortality. Consensus, however, is lacking on the role and optimal timing of nephrectomy, with decision-making driven by the patient's clinical condition and the expertise of the centre. We report on our experience of an infant with ARPKD requiring neonatal renal replacement therapy and survival at 14 months following early bilateral nephrectomy.

Hypertension in the paediatric renal transplant paediatric population can have significant adverse effects on transplanted kidney function in addition to general and cardiovascular health. The British Association of Paediatric Nephrologists (BAPN) guidelines recommend maintaining blood pressure below the 90 th percentile in this population. An audit of all paediatric renal transplant recipients in the Royal Belfast Hospital for Sick Children assessed current practice of hypertension management against BAPN guidelines. Blood pressure recordings from the most recent clinic attendance were noted and the percentile calculated using the National Heart Lung and Blood Institute paediatric blood pressure charts. We assessed the blood pressure percentile in our transplant population against the audit standard, the BAPN guidelines. The audit identified 15% of the transplant recipients to have blood pressures above their target range. Other areas for improvement included availability of appropriate sized blood pressure cuffs at outpatient clinics, clinician ability to recognise hypertension and resources to confirm if isolated blood pressure measurements reflected true hypertension in individuals. The results of the audit were presented at a multidisciplinary meeting with all renal health professionals engaged in the implementation of changes. Recommendations included ensuring availability of all sizes of blood pressure cuffs, prominent placement of blood pressure percentile charts in all clinic rooms, the creation of a renal transplant clinic stamp to encourage target BP recording and recognition of hypertension and the development of a new ambulatory blood pressure monitoring service. All of the age-appropriate renal transplant patients were invited to have ambulatory blood pressure monitoring (ABPM) and hypertension information leaflets were developed with practical advice reiterated through the dietician and clinician at clinic. After one year the audit cycle was completed. All children identified as being hypertensive were now meeting their target blood pressure. In addition, all age-appropriate assenting patients had ABPM performed, which identified previously undiagnosed hypertensive patients ensuring appropriate treatment. A clinic has now been established for yearly ABPM of all post-transplant patients. By identifying simple measures to improve the measurement of blood pressure and aid the recognition of hypertension in children, we have reduced the degree of hypertension in this transplant population, thereby helping to optimise cardiovascular health and graft survival.