TY - JOUR
T1 - Encapsulating peritoneal sclerosis in children on chronic PD
T2 - A survey from the European Paediatric Dialysis Working Group
AU - Shroff, Rukshana
AU - Stefanidis, Constantinos J.
AU - Askiti, Varvara
AU - Edefonti, Alberto
AU - Testa, Sara
AU - Ekim, Mesiha
AU - Kavaz, Asli
AU - Ariceta, Gema
AU - Bakkaloglu, Sevcan
AU - Fischbach, Michel
AU - Klaus, Günter
AU - Zurowska, Aleksandra
AU - Holtta, Tuula
AU - Jankauskiene, Augustina
AU - Vondrak, Karel
AU - Walle, Johan Vande
AU - Schmitt, Claus Peter
AU - Watson, Alan R.
PY - 2013/7
Y1 - 2013/7
N2 - 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.
AB - 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.
KW - Encapsulating peritoneal sclerosis
KW - Paediatrics
KW - Peritoneal dialysis
KW - Peritonitis
KW - UF failure
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U2 - 10.1093/ndt/gfs603
DO - 10.1093/ndt/gfs603
M3 - Article
C2 - 23348882
AN - SCOPUS:84881520601
VL - 28
SP - 1908
EP - 1914
JO - Nephrology Dialysis Transplantation
JF - Nephrology Dialysis Transplantation
SN - 0931-0509
IS - 7
ER -