Ten-year trends in epidemiology and outcomes of pediatric kidney replacement therapy in Europe: data from the ESPN/ERA-EDTA Registry.

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 <15 years starting KRT 2007-2016 in 22 European countries participating in the ESPN/ERA-EDTA Registry since 2007. General population statistics were derived from Eurostat. Incidence and prevalence were expressed per million age-related population (pmarp) and time trends studied with JoinPoint regression. We analyzed survival trends using Cox regression. RESULTS: Incidence of children commencing KRT <15 years remained stable over the study period, varying between 5.5 and 6.6 pmarp. Incidence by treatment modality was unchanged over time: 2.0 for hemodialysis (HD) and peritoneal dialysis (PD) and 1.0 for transplantation. Prevalence increased in all age categories and overall rose 2% annually from 26.4 pmarp in 2007 to 32.1 pmarp in 2016. Kidney transplantation prevalence increased 5.1% annually 2007-2009, followed by 1.5% increase/year until 2016. Prevalence of PD steadily increased 1.4% per year over the entire period, and HD prevalence started increasing 6.1% per year from 2011 onwards. Five-year unadjusted patient survival on KRT was around 94% and similar for those initiating KRT 2007-2009 or 2010-2012 (adjusted HR: 0.98, 95% CI:0.71-1.35). 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.

UK Renal Registry 18th Annual Report: Chapter 4 Demography of Patients Receiving Renal Replacement Therapy in Paediatric Centres in the UK in 2014.

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 ,18 years had a functioning kidney transplant, 11.2% were receiving haemodialysis (HD) and 9.5% were receiving peritoneal dialysis (PD). In patients aged ,16 years, prevalence of ERF was 60.4 per million age related population (pmarp) and the incidence 9.4 pmarp. The most common primary renal diagnosis was renal dysplasia+reflux, present in 32.6% of prevalent paediatric patients aged ,16 years. 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 2000­2004. 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 2.2­8.0), and in those receiving dialysis compared to having a functioning transplant with a hazard ratio of 6.3 (confidence interval 3.9­10.2).

UK Renal Registry 18th Annual Report: Chapter 10 Clinical, Haematological and Biochemical Parameters in Patients Receiving Renal Replacement Therapy in Paediatric Centres in the UK in 2014: National and Centre-specific Analyses.

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 renal replacement 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 with a 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 mmol/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.

Early bilateral nephrectomy in infantile autosomal recessive polycystic kidney disease.

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.

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report.

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.