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 UK Renal Registry currently collects information on UK children with kidney failure requiring long-term kidney replacement therapy (KRT), which supports disease surveillance and auditing of care and outcomes; however, data are limited on children with chronic kidney disease (CKD) not on KRT.MethodsIn March 2020, all UK Paediatric Nephrology centres submitted data on children aged <16 years with severely reduced kidney function as of December 2019, defined as an estimated glomerular filtration rate <30 mL/min/1.73 m2.ResultsIn total, 1031 children had severe CKD, the majority of whom (80.7%) were on KRT. The overall prevalence was 81.2 (95% CI 76.3 to 86.3) per million of the age-related population.ConclusionsThe prevalence of severe CKD among UK children is largely due to a high proportion of children on long-term KRT. Expanding data capture to include children with CKD before reaching failure will provide greater understanding of the CKD burden in childhood.

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tested, and to investigate if any of those features corresponded with a greater likelihood of predicting a genetic diagnosis. Information was obtained by accessing electronic clinical records. Of the 99 children who had genetic testing, 5 children were excluded due to insufficient available information. The characteristics that were assessed in the 94 remaining children included: hearing loss or visual problems either in the proband or in first degree relatives, proteinuria at the time of referral, family history of renal disease, abnormal renal function, hypertension and proteinuria, and a history of previous urinary tract infections. The probability of certain characteristics being associated with an underlying genetic diagnosis was evaluated by calculating likelihood ratios Results Of the 94 children, 65% were male. Median age was 9 years (range 9 months-16 years). Median time from haematuria onset to referral to the tertiary nephrology centre was 7 months (range 1-108 months) and median time from referral to genetic testing was 8 months (range 1-86 months). 28% of the children were found to have an underlying genetic cause of their haematuria or had genetic variants of yet unknown clinical significance (VUS). In the children who had VUS, 38% of mutations were in COL4A3, 31% in COL4A4, 15% in COL4A5, 8% in COL4A6 and 8% in NPHS2. Of the characteristics analysed, co-existing visual problems demonstrated a notable increased likelihood of a genetic diagnosis (likelihood ratio=25). Co-existing family history of renal disease only led to a marginal increased likelihood of a genetic diagnosis (likelihood ratio=1.87). Conclusion This is the largest single centre review to date correlating clinical phenotype in children with persistent haematuria with COL4 genetic testing. From this we can infer that children who present with haematuria should have an early ophthalmological assessment and those who also have visual impairment should be prioritised for genetic testing to assess for variations in the COL4A genes as this increases the pretest probability of a positive genetic result.

AimTo review the demographics and outcomes of kidney transplantation in Northern Ireland over two decades.MethodsAll kidney transplants performed from 1995–2016 were included. Donor and recipient demographics and transplant details are recorded at the time of transplantation. Recipient and donor outcomes are collected prospectively.ResultsThere were 78 transplants performed during the study period; 61% of recipients were male. The median age was 12 years. The median duration of pre-transplant renal replacement therapy was 14 months. Eighteen patients were transplanted preemptively; 50% of transplants performed since 2010 have been pre-emptive.Kidneys donated after brain death were the organ source in 65% of cases. Since 2010, the majority of transplants performed have been from living donors. The median donor age was 29 years and the median number of HLA mismatches was two. The ischaemic time ranged from 143–2785 minutes (median 1143 min).Median follow up time was 9.6 years. Discharge creatinine was available for 42 patients with a median of 54 mmol/L. One and two years after transplant, the median creatinine was 76 mmol/L and 87 mmol/L respectively.The median graft survival was 84.5 months. There were 22 cases of death-censored graft loss in the study period. In five, graft loss was secondary to early technical problems; these all occurred between 1995 and 2005. In the other 17, grafts functioned for 5–18 years prior to failure. In multivariate analysis, recipient age, donor age and era of transplantation were associated with graft survival.Three deaths occurred in the follow up period. Two patients died within the first month and a third died from post-transplant lymphoproliferative disorder 17 years after transplantation.ConclusionOutcomes after paediatric kidney transplantation in Northern Ireland have improved over the past twenty years. There have been no early graft losses or deaths in the past decade. The expansion of the living donor programme means that 50% of children are now transplanted pre-emptively.