Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group.
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.
Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. rukshana.shroff@gosh.nhs.uk
Rukshana
R
Shroff
Constantinos J
CJ
Stefanidis
Varvara
V
Askiti
Alberto
A
Edefonti
Sara
S
Testa
Mesiha
M
Ekim
Asli
A
Kavaz
Gema
G
Ariceta
Sevcan
S
Bakkaloglu
Michel
M
Fischbach
Günter
G
Klaus
Aleksandra
A
Zurowska
Tuula
T
Holtta
Augustina
A
Jankauskiene
Karel
K
Vondrak
Johan
J
Vande Walle
Claus Peter
CP
Schmitt
Alan R
AR
Watson
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D006306:Health Surveys; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D008297:Male; D010530:Peritoneal Dialysis / Q000009:adverse effects* / Q000401:mortality; D056627:Peritoneal Fibrosis / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D010538:Peritonitis / Q000209:etiology* / Q000473:pathology; D011379:Prognosis; D012307:Risk Factors; D015996:Survival Rate; D014462:Ultrafiltration; D055815:Young Adult
UF failure; encapsulating peritoneal sclerosis, paediatrics, peritoneal dialysis; peritonitis