Double filtration plasmapheresis - 10-year pediatric experience as an alternative to plasma exchange.

BACKGROUND: Double filtration plasmapheresis (DFPP) is more selective at removing antibodies compared to plasma exchange (PE), thus reducing the need for replacement blood products. METHODS: We retrospectively analyzed the records of all pediatric patients whom DFPP had been performed. RESULTS: In total, 30 patients were treated with DFPP. Data were available for 436 sessions in 23 patients. Patients had a median of 6 (1-161) sessions. Age at start of treatment was 13.9 years (2.2-19.2) and weight 46 kg (13.4-82.8). Six patients were treated for antibody mediated rejection; 1 had full recovery, 1 CKD stage 4 and 4 required dialysis. Two patients were treated for recurrence of focal segmental glomerulosclerosis (FSGS) with good response. One successfully had an ABO-incompatible kidney transplantation. Five had anti-glomerular basement membrane disease; 3 had complete recovery, 1 CKD and 1 required transplantation. Three had granulomatosis with polyangiitis; 1 with full recovery, 1 had CKD and 1 required dialysis. Two had Type-2 Membrano-proliferative glomerulonephritis, 1 successfully treated, the other needing dialysis. One treated for rapidly progressive MPO-glomerulonephritis required dialysis. Other indications were Myasthenia Gravis, Guillain-Barré disease and autoimmune limbic encephalitis. Excluding FSGS patients (with >100 sessions), one patient had cryoprecipitate, 2 had blood transfusions, no other blood products were required. Minor complications were seen in 13 sessions (8.4%). No major complications were seen. CONCLUSION: DFPP is a safe, well tolerated form of apheresis that appears to have comparable outcomes to that of PE, without the routine need of replacement blood products.

Positive trends in paediatric renal biopsy service provision in the UK: a national survey and re-audit of paediatric renal biopsy practice.

BACKGROUND: Paediatric renal biopsy standards introduced in the UK in 2010 were intended to reduce variation and improve practice. A concurrent national drive was aimed at building robust paediatric nephrology networks to ensure services cater for the needs of the family and minimise time away from home. We aimed to identify current national practice since these changes on behalf of the British Association for Paediatric Nephrology. METHODS: All UK paediatric nephrology centres were invited to complete a survey of their biopsy practice, including advance preparation. From 1 January to 30 June 2012, a national prospective audit of renal biopsies was undertaken at participating centres comparing practice with the British Association for Paediatric Nephrology (BAPN) standards and audit results from 2005. RESULTS: Survey results from 11 centres demonstrated increased use of pre-procedure information leaflets (63.6 % vs 45.5 %, P = 0.39) and play preparation (90.9 % vs 9.1 %, P = 0.0001). Audit of 331 biopsies showed a move towards day-case procedures (49.5 % vs 32.9 %, P = 0.17) and reduced major complications (4.5 % vs 10.4 %, P = 0.002). Biopsies with 18-gauge needles had significantly higher mean pass rates (3.2 vs 2.3, P = 0.0008) and major complications (15.3 % vs 3.3 %, P = 0.0015) compared with 16-gauge needles. CONCLUSIONS: Percutaneous renal biopsy remains a safe procedure in children, thus improving family-centered service provision in the UK.

Anemia in children following renal transplantation-results from the ESPN/ERA-EDTA Registry.

BACKGROUND: Our aim was to determine the prevalence of sub-target hemoglobin (Hb) levels in children with a renal allograft and to identify potential determinants associated with these Hb levels. METHODS: Data from 3669 children with a functioning renal allograft, aged <18 years between 1 January 2000 and 31 December 2012, from 20 European countries were retrieved from the ESPN/ERA-EDTA Registry, providing 16,170 Hb measurements. RESULTS: According to the NKF/KDOQI classification and the UK-NICE guidelines, 49.8 and 7.8% of the patients, respectively, were anemic. Hb levels were strongly associated with graft function, with Hb levels of 12.6 g/dl in children with chronic kidney disease (CKD) stage 1, declining to 10.7 g/dl in children with CKD stage 5 (P < 0.001). Higher Hb levels were associated with the use of tacrolimus compared to ciclosporin (0.14 g/dl; 95% confidence interval 0.02-0.27; P = 0.002). Low Hb levels were associated with an increased risk of graft failure (P = 0.01) or combined graft failure and death (P < 0.01), but not with death alone (not significant). CONCLUSIONS: Anemia is present in a significant proportion of European pediatric kidney transplant recipients and is associated with renal allograft dysfunction and type of immunosuppressants used. In our patient cohort, higher Hb levels were associated with better graft and patient survival and less hypertension.

UK Renal Registry 13th Annual Report (December 2010): Chapter 5: demography of the UK paediatric renal replacement therapy population in 2009.

AIMS: To describe the demographics of the paediatric RRT population under the age of 16 years in the UK and to analyse changes in demography with time. METHODS: Extraction and analysis of data from the UK Renal Registry (UKRR). RESULTS: There were 751 children <16 years old with established renal failure (ERF) in the UK in December 2009. The reported prevalence under the age of 16 years was 65 per million age related population (pmarp) and the reported incidence 9.3 pmarp. The incidence and prevalence for South Asian patients was much higher than that of the White and Black populations. Of the patients for whom a primary renal diagnosis had been reported, renal dysplasia ± reflux was the most common cause of ERF accounting for 34.0% of prevalent cases. There has been growth in treatment numbers in all paediatric renal centres between 1995 and 2010. Whilst the rate of transplantation within 90 days of commencing RRT has remained at around 25-30% of patients, the use of HD has increased by 4% at the expense of PD. CONCLUSIONS: The paediatric ERF population continued to expand with a slow increase in both incidence and prevalence rates. The high incidence in patients from ethnic minority groups will lead to a greater proportion of the population being from these groups in time. To maintain the high proportion of engrafted patients it will be necessary to encourage living donation in the ethnic minority population.

UK national study of barriers to renal transplantation in children.

AIMS: To investigate access to paediatric renal transplantation and examine potential barriers within the process. METHODS: Cross-sectional, multicentre, observational study where paediatric nephrology centres in the UK were requested to provide data on transplantation plans for all children (<18 years) with end-stage kidney disease (ESKD). RESULTS: 308 children with ESKD were included in this study from 12 out of 13 UK paediatric nephrology centres. 139 (45%) were being prepared for living donor transplantation and 82 (27%) were listed for deceased donor transplantation. The most common cited factors delaying transplantation from occurring in children were disease factors (36%), donor availability (27%) and size of the child (20%). Psychosocial factors were listed as a barrier in 19% of children. CONCLUSIONS: In this study we have documented the main barriers to renal transplantation in children. Some identified factors may be modifiable through local or national intervention, including donor availability and patient psychosocial factors.

Renal biopsies in children: current practice and audit of outcomes.

BACKGROUND: There is considerable variation in the way that children are prepared for and the techniques employed in a renal biopsy. There was national agreement between UK paediatric renal centres to review current practice and audit outcomes METHODS: An initial questionnaire survey was undertaken and a 12-month prospective audit performed of renal biopsies against agreed standards for the number of needle passes, adequacy of biopsy material and complication rates. RESULTS: Eleven of 13 centres participated. Information leaflets are sent pre-biopsy in five centres with only one using play preparation. Six of 11 routinely perform biopsies as day-case (DC) procedures and 6 use general anaesthesia (GA). Real-time ultrasound is the favoured method in eight centres. Biopsies are performed by nephrologists only in four centres, nephrologists with radiologists in five and radiology alone in two. Of 531 biopsies (352 native), 31% were performed as a DC with 49% being done under GA. The standard for the number of passes of native kidneys (95%). The major complication rate was higher than the standard of

UK Renal Registry 12th Annual Report (December 2009): chapter 15: clinical, haematological and biochemical parameters in patients receiving renal replacement therapy in paediatric centres in the UK in 2008: national and centre-specific analyses.

BACKGROUND: The British Association for Paediatric Nephrology Registry was established thirteen years ago to analyse data related to renal replacement therapy for children. The registry receives data from the 13 paediatric nephrology centres in the UK. In 2008 the registry was relocated to the UK Renal Registry (UKRR). AIM: To provide centre specific data so that individual centres can reflect on the contribution that their data makes to the national picture and to determine the extent to which their patient parameters meet nationally agreed audit standards for the management of children with established renal failure. METHOD: Data were submitted to the UKRR for analysis electronically via renal IT systems from 5 centres and on paper-based returns from the remaining centres. Data were analysed to calculate summary statistics and where applicable the percentage achieving an audit standard. The standards used were those set out by the Renal Association and the National Institute for Health and Clinical Excellence. RESULTS: Data were received from all but one centre. Anthropometric data confirmed that children with ERF in the UK are short compared with their peers with no change in recent trends. In the UK as a whole, the control of blood pressure, anaemia and bone biochemistry is suboptimal, but for some parameters these appear to be better in the 2008 cohort than in the 1999-2008 cohort. CONCLUSIONS: Key features of this report are the provision of centre specific data and comparison of data to audit standards. It is hoped that this information will provide a basis for discussion and a stimulus to improve the care of children with ERF.

UK Renal Registry 12th Annual Report (December 2009): chapter 14: demography of the UK paediatric renal replacement therapy population in 2008.

AIMS: To describe the demographics of the paediatric RRT population in the UK and analyse changes in demographics with time. METHODS: Extraction and analysis of data from the UK Paediatric Renal Registry and the UK Renal Registry (UKRR). RESULTS: The UK paediatric established renal failure (ERF) population in December 2008 was 905 patients. The prevalence under the age of 16 years was 56 per million age related population (pmarp) and the incidence 7.4 pmarp. The incidence and prevalence for South Asian patients was much higher than that of the White and Black populations. Renal dysplasia was the most common cause of ERF accounting for 33% of prevalent cases. Diseases with autosomal recessive inheritance were a common cause of ERF in all ethnic groups, 23.5% of prevalent and 18% of incident cases. Whilst the incidence and prevalence of diseases with autosomal recessive inheritance in the South Asian population was 3 times that of the white population, this was not the sole reason for the increased proportion of South Asian patients with ERF, as diseases with no defined inheritance were twice as common in this ethnic group than in White patients. Prevalent mortality stood at 9.4%. Most deaths were in patients presenting with ERF early in life and mortality varied markedly according to the aetiology of ERF. The proportion with new grafts from living donors has steadily risen to 54%. Children from ethnic minority groups were less likely to have an allograft and living donation was less frequent in this population. For those on dialysis, 56% were receiving peritoneal dialysis. This was the main treatment modality for patients under 4 years of age. CONCLUSIONS: The paediatric ERF population continued to expand slowly. Incidence and prevalence rates were stable and similar to other developed nations. The high incidence in patients from ethnic minority groups will lead to a greater proportion of the population being from these groups in time. To maintain the high proportion of engrafted patients it will be necessary to encourage living donation in the ethnic minority population. Case note analysis of the factors involved in mortality would be valuable.

UK Renal Registry 11th Annual Report (December 2008): Chapter 13 Demography of the UK paediatric renal replacement therapy population.

AIMS: To describe the demographics of the paediatric RRT population in the UK and analyse changes in demographics with time. METHODS: Extraction and analysis of data from the UK paediatric Renal Registry. RESULTS: The UK paediatric established renal failure (ERF) population in April 2008 was 875 patients. The prevalence under the age of 16 years was 55 per million age related population (pmp) and the incidence 7.92 pmp. The incidence and prevalence for South Asian and Other ethnic groups were 3 times that of the White and Black populations. Renal dysplasia was the most common cause of ERF accounting for 33% of prevalent cases. Diseases with autosomal recessive inheritance were more common in patients from ethnic minority groups. The spectrum of diseases seen has changed over a generation. Overall 5 year survival for children with ERF was 91.8%. Five year survival of infants starting dialysis was just 62%. Transplanted patients accounted for 74% of the current population. The proportion with grafts from living donors has steadily risen to 34%. Children from ethnic minority groups were less likely to have an allograft and living donation was less frequent in this population. For those on dialysis, 57% were receiving peritoneal dialysis. This was the main treatment modality for patients under 4 years of age. CONCLUSIONS: The paediatric ERF population continued to expand slowly. Incidence and prevalence rates were stable and similar to other developed nations. The high incidence in patients from ethnic minority groups will lead to a greater proportion of the population being from these groups in time. To maintain the high proportion of engrafted patients it will be necessary to encourage living donation in the ethnic minority population. The spectrum of diseases seen has already changed over a generation with the treatment of young children with diseases such as congenital nephrosis. The incidence of cystinosis causing ERF was reduced, probably reflecting better early treatment.

Treatment of Renal Artery Stenosis in a Solitary Kidney Using Cutting Balloon Angioplasty in a Paediatric Patient.

AIMS: Renovascular hypertension is a rare cause of paediatric hypertension. It is however, a potentially treatable cause particularly when caused by renal artery stenosis (RAS). MATERIALS AND METHODS: We present the case of an 11-month-old girl presenting with cardiac dysfunction. She was found to be hypertensive with a systolic blood pressure >180mmHg. DMSA demonstrated a small right kidney and a divided renal function of 6% on the right and 94% on the left. Spectral analysis demonstrated abnormal waveforms suggestive of RAS of the left kidney. RESULTS: Angioplasty with a cutting balloon was successful. Blood pressure measurements, renal function and left ventricular function improved. CONCLUSION: RAS can be successfully treated with cutting balloon angioplasty after failure of convention balloon angioplasty to relieve the narrowing. In our case, there was an immediate successful angiographic result that on mid-term follow-up demonstrated significant improvement in clinical and biochemical outcomes and cessation of all anti-hypertensive medication.

Chronic peritoneal dialysis in children.

The incidence of end-stage renal disease in children is increasing. Peritoneal dialysis (PD) is the modality of choice in many European countries and is increasingly applied worldwide. PD enables children of all ages to be successfully treated while awaiting the ultimate goal of renal transplantation. The advantages of PD over other forms of renal replacement therapy are numerous, in particular the potential for the child to lead a relatively normal life. Indications for commencing PD, the rationale, preparation of family, technical aspects, and management of complications are discussed.

Standards for renal biopsies: comparison of inpatient and day care procedures.

There are no national standards for the adequacy and complications of percutaneous renal biopsies. We developed local standards that have been used in a prospective audit of biopsies undertaken in a tertiary pediatric nephrology unit between January 1997 and December 2000. We compared outcomes of biopsies performed on inpatients with day care procedures. A total of 251 biopsies (113 transplant) were undertaken, 114 (46%) as day care procedures. Adequate tissue for diagnosis was obtained in 245 (97.6%), with a standard set at >95%. This was also achieved for a mean number of passes in native (<3 in 80%) and transplanted (<2 in 80%) kidneys. Eleven patients (4%) developed macroscopic hematuria (standard <5%) and none required transfusion. Delay in discharge occurred in 4 patients, and a further 4 returned to the ward post discharge. There was no significant difference in complication rates between inpatient and day care patients. Our local biopsy standards were met in this audit. Such standards could provide useful comparisons between units in national audit programs, as well as permitting the monitoring of individual performance as part of clinical governance. Day care procedures benefit the patient and family, as well as significantly reducing costs.