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Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group

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Abstract
Background. Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that is associated with significant morbidity and mortality in adults. There are scarce data for children. We performed a 10-year survey to determine the prevalence, risk factors and outcome for EPS in children. Methods. Chronic PD patients in 14 dialysis units participating in the European Paediatric Dialysis Working Group between January 2001 and December 2010 were included in this study. Results. Twenty-two cases of EPS were reported (prevalence 1.5%; 8.7 per 1000 patient-years on PD). Median PD vintage was 5.9 (1.6-10.2) in EPS and 1.7 (0.7-7.7) years in the remainder of the PD population (P < 0.0001). EPS patients had a significantly higher peritonitis rate than non-EPS patients (P = 0.2). EPS was diagnosed while the child was on PD in 17 (77%), after conversion to haemodialysis (HD) in 3 and after transplantation in 2. Fifteen of 17 (88%) developed ultrafiltration (UF) failure. The median interval between UF failure and presentation with bowel obstruction was 2.8 (0.02-5.8) months. Twenty (91%) had clinical and radiological signs of bowel obstruction. Enterolysis was performed in 14 and 19 received immunosuppression or tamoxifen. Nine required parenteral nutrition. At final follow-up 4.8 (1.3-8.7) years after EPS diagnosis, 3 patients died, 11 had a functioning transplant and 8 were on HD. Conclusions. The prevalence of EPS in European children on PD is comparable with that of adult PD patients, but mortality from paediatric EPS is significantly lower. A high index of suspicion is required for the diagnosis of EPS in children with longer dialysis duration, a high peritonitis rate and UF failure.
Keywords
paediatrics, encapsulating peritoneal sclerosis, peritoneal dialysis, peritonitis, UF failure, DUTCH EPS, DIAGNOSIS, EXPERIENCE, MANAGEMENT, MEMBRANE, OUTCOMES, THERAPY, SYSTEM, JAPAN

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MLA
Schroff, Rukshana, Constantinos J Stefanidis, Varvara Askiti, et al. “Encapsulating Peritoneal Sclerosis in Children on Chronic PD: a Survey from the European Paediatric Dialysis Working Group.” NEPHROLOGY DIALYSIS TRANSPLANTATION 28.7 (2013): 1908–1914. Print.
APA
Schroff, R., Stefanidis, C. J., Askiti, V., Edefonti, A., Testa, S., Ekim, M., Kavaz, A., et al. (2013). Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group. NEPHROLOGY DIALYSIS TRANSPLANTATION, 28(7), 1908–1914.
Chicago author-date
Schroff, Rukshana, Constantinos J Stefanidis, Varvara Askiti, Alberto Edefonti, Sara Testa, Mesiha Ekim, Asli Kavaz, et al. 2013. “Encapsulating Peritoneal Sclerosis in Children on Chronic PD: a Survey from the European Paediatric Dialysis Working Group.” Nephrology Dialysis Transplantation 28 (7): 1908–1914.
Chicago author-date (all authors)
Schroff, Rukshana, Constantinos J Stefanidis, Varvara Askiti, Alberto Edefonti, Sara Testa, Mesiha Ekim, Asli Kavaz, Gema Ariceta, Sevcan Bakkalogu, Michel Fischbach, Günter Klaus, Aleksandra Zurowska, Tuula Holtta, Augustina Jankauskiene, Karel Vondrak, Johan Vande Walle, Claus Peter Schmitt, and Alan R Watson. 2013. “Encapsulating Peritoneal Sclerosis in Children on Chronic PD: a Survey from the European Paediatric Dialysis Working Group.” Nephrology Dialysis Transplantation 28 (7): 1908–1914.
Vancouver
1.
Schroff R, Stefanidis CJ, Askiti V, Edefonti A, Testa S, Ekim M, et al. Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group. NEPHROLOGY DIALYSIS TRANSPLANTATION. 2013;28(7):1908–14.
IEEE
[1]
R. Schroff et al., “Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group,” NEPHROLOGY DIALYSIS TRANSPLANTATION, vol. 28, no. 7, pp. 1908–1914, 2013.
@article{3260084,
  abstract     = {Background. Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that is associated with significant morbidity and mortality in adults. There are scarce data for children. We performed a 10-year survey to determine the prevalence, risk factors and outcome for EPS in children. 
Methods. Chronic PD patients in 14 dialysis units participating in the European Paediatric Dialysis Working Group between January 2001 and December 2010 were included in this study. 
Results. Twenty-two cases of EPS were reported (prevalence 1.5%; 8.7 per 1000 patient-years on PD). Median PD vintage was 5.9 (1.6-10.2) in EPS and 1.7 (0.7-7.7) years in the remainder of the PD population (P < 0.0001). EPS patients had a significantly higher peritonitis rate than non-EPS patients (P = 0.2). EPS was diagnosed while the child was on PD in 17 (77%), after conversion to haemodialysis (HD) in 3 and after transplantation in 2. Fifteen of 17 (88%) developed ultrafiltration (UF) failure. The median interval between UF failure and presentation with bowel obstruction was 2.8 (0.02-5.8) months. Twenty (91%) had clinical and radiological signs of bowel obstruction. Enterolysis was performed in 14 and 19 received immunosuppression or tamoxifen. Nine required parenteral nutrition. At final follow-up 4.8 (1.3-8.7) years after EPS diagnosis, 3 patients died, 11 had a functioning transplant and 8 were on HD. 
Conclusions. The prevalence of EPS in European children on PD is comparable with that of adult PD patients, but mortality from paediatric EPS is significantly lower. A high index of suspicion is required for the diagnosis of EPS in children with longer dialysis duration, a high peritonitis rate and UF failure.},
  author       = {Schroff, Rukshana and Stefanidis, Constantinos J and Askiti, Varvara and Edefonti, Alberto and Testa, Sara and Ekim, Mesiha and Kavaz, Asli and Ariceta, Gema and Bakkalogu, Sevcan and Fischbach, Michel and Klaus, Günter and Zurowska, Aleksandra and Holtta, Tuula and Jankauskiene, Augustina and Vondrak, Karel and Vande Walle, Johan and Schmitt, Claus Peter and Watson, Alan R},
  issn         = {0931-0509},
  journal      = {NEPHROLOGY DIALYSIS TRANSPLANTATION},
  keywords     = {paediatrics,encapsulating peritoneal sclerosis,peritoneal dialysis,peritonitis,UF failure,DUTCH EPS,DIAGNOSIS,EXPERIENCE,MANAGEMENT,MEMBRANE,OUTCOMES,THERAPY,SYSTEM,JAPAN},
  language     = {eng},
  number       = {7},
  pages        = {1908--1914},
  title        = {Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group},
  url          = {http://dx.doi.org/10.1093/ndt/gfs603},
  volume       = {28},
  year         = {2013},
}

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