Search

Background Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. Methods We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Results Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4-14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction (n = 5) or peritonitis (n = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6 months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6-18) months, and the median age at transplantation was 22 (14-28) months. Conclusions Infants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data., Author(s): Stephanie Dufek [sup.1] , Elisa Ylinen [sup.2] , Agnes Trautmann [sup.3] , Harika Alpay [sup.4] , Gema Ariceta [sup.5] , Christoph Aufricht [sup.6] , Justine Bacchetta [sup.7] , Sevcan [...]

Background Increased plasma cell infiltration in renal allograft biopsies is a rare finding associated with poor outcome in adult renal transplant recipients. The clinical impact of increased plasma cell infiltrates in paediatric renal transplant recipients (pRTR) remains unknown. Methods We conducted a retrospective case-control study from April 1996 to March 2014 comparing the outcome of pRTR with increased (>10 % of infiltrate) plasma cells in renal transplant biopsies to a control cohort of pRTR without increased plasma cell infiltration but similar grade of rejection according to Banff classification. Results Increased plasma cell infiltrates were present in 14 of 162 (9 %) reviewed pRTR aged 3.2-17.5 (median 13.4) years at time of transplantation. Compared with 14 pRTR renal transplant biopsies without significantly increased plasma cells, there were no significant differences in mismatch and baseline estimated glomerular filtration rate (eGFR). Plasma cells were present in case biopsies at a maximal density of 14-116 (median 33) plasma cells/HPF. Increased plasma cells were associated with decreased eGFR at biopsy (22 vs. 49 ml/min/1.73 m.sup.2; p < 0.001) and 4 weeks post-biopsy (26 vs. 56 ml/min/1.73 m.sup.2; p < 0.001) despite comparable eGFR 4 weeks prior to biopsy. Increased plasma cells were further associated with significantly increased frequency of renal allograft loss (71 % vs. 7 %; p < 0.001) at 0-27 (median 2) months after biopsy. Conclusion Increased plasma cell infiltrates in pRTR are uncommon but associated with significantly reduced renal allograft survival as well as significantly reduced allograft function in surviving grafts., Author(s): Stephanie Dufek [sup.1] , Azaz Khalil [sup.1] , Nizam Mamode [sup.2] , Neil J. Sebire [sup.3] , Stephen D. Marks [sup.1] Author Affiliations: (1) 0000 0004 0426 7394grid.424537.3Department of [...]

Background Steroid-sensitive nephrotic syndrome is the most common form of nephrotic syndrome in childhood, defined by the response to treatment with glucocorticoids with consequent remission. While most children eventually experience spontaneous resolution of the disease, some have a difficult course with frequent relapses or steroid dependence nephrotic syndrome (FRSDNS). The consequent steroid toxicity often prompts administration of other immunosuppressive drugs, traditionally cyclophosphamide. Recently, rituximab has been reported as effective in this disorder, but long-term experience is lacking. Methods Retrospective note review of all children with FRSDNS treated with a first course of cyclophosphamide and/or rituximab in our center between December 2006 and April 2015. We reviewed time to first relapse after treatment, co-medications, and side effects. Results A total of 102 children were treated with cyclophosphamide (79) and/or rituximab (42). Of these, 34 received cyclophosphamide prior to rituximab. Median time to first relapse was 7 months after cyclophosphamide and 14 months after rituximab. Documented side effects of cyclophosphamide included neutropenia, hair loss, and hemorrhagic cystitis (1). Rituximab was associated with an allergic reaction at infusion in two patients. Conclusions Rituximab was used in children with the most difficult to treat FRSDNS, yet was associated with longer remission time and less side effects than cyclophosphamide. A randomized controlled trial is needed to directly compare these drugs., Author(s): Hazel Webb[sup.1] , Graciana Jaureguiberry[sup.1] , Stephanie Dufek[sup.1] , Kjell Tullus[sup.1] , Detlef Bockenhauer[sup.1] Author Affiliations: (1) Department of Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust [...]

Background Pleural or pericardial effusions secondary to pleuro-peritoneal fistula (PPF) and pericardio-peritoneal fistula (PcPF) are rare but serious complications of peritoneal dialysis (PD). Methods We conducted a 10-year survey across all participating centres in the European Paediatric Dialysis Working Group to review the incidence, diagnostic techniques, therapeutic options and outcome of children on chronic PD with PPF and/or PcPF. Results Of 1506 children on PD there were ten cases (8 of PPF, 1 each of PcPF and PPF + PcPF), with a prevalence of 0.66 %. The median age at presentation was 1.5 [inter-quartile range (IQR) 0.4-2.4] years, and nine children were Conclusion In conclusion, PPF and PcPF are rare in children on chronic PD, but are associated with significant morbidity, requiring a change of dialysis modality in all cases. Risk factors for PPF development include age of, Author(s): Stephanie Dufek[sup.1] , Tuula Holtta[sup.2] , Michel Fischbach[sup.3] , Gema Ariceta[sup.4] , Augustina Jankauskiene[sup.5] , Rimante Cerkauskiene[sup.5] , Claus Peter Schmitt[sup.6] , Betti Schaefer[sup.6] , Christoph Aufricht[sup.7] , Elizabeth [...]

Background Sudden blindness caused by anterior ischemic optic neuropathy (AION) is a rare complication for patients undergoing peritoneal dialysis (PD). Prognosis is generally poor, with AION commonly resulting in permanent visual loss. Methods We first describe four case reports of children with AION during PD treatment. We then review ten additional AION cases reported in the literature and compare these 14 affected patients with a control cohort of 59 non-affected patients in the Vienna PD registry. Results Significant risk factors for AION were identified as median age (4 vs. 27 months; p Conclusions Young age, autosomal recessive polycystic kidney disease, anephric status and hypotension are substantial risk factors for AION. Early hospitalization with vascular refilling within a few hours following onset of blindness leads to improved visual outcome. Keywords AION * Blindness in children * Dehydration * Polycystic kidney disease * Nephrectomy, Introduction Peritoneal dialysis (PD) is one of the most widely used therapeutic options for children with end-stage renal disease (ESRD) awaiting renal transplantation. Compared to hemodialysis, PD has a number [...]