title: Encapsulating peritoneal sclerosis in paediatric peritoneal dialysis patients: the experience of the Italian Registry of Pediatric Chronic Dialysis.
abstract: BACKGROUND
  Paediatric literature about encapsulating peritoneal sclerosis (EPS) is limited and comes primarily from anecdotic experiences. In this study, we described the incidence and characteristics of EPS in a large paediatric chronic peritoneal dialysis (CPD) patient population.
  
  
  METHODS
  We reviewed files of patients starting CPD at <16 years of age, recorded from January 1986 to December 2011 by the Italian Registry of Pediatric Chronic Dialysis (n = 712). Moreover, in December 2011, a survey was performed involving all the Italian Pediatric Nephrology Units to report such EPS cases that occurred after CPD withdrawal.
  
  
  RESULTS
  Fourteen EPS cases were reported, resulting in a prevalence of 1.9%. The median age of EPS cases was 4.8 years (range 0.6-14.4) at the start of CPD and 14.3 years (6.5-26.8) at EPS diagnosis. Eleven EPS cases received CPD for longer than 5 years. At diagnosis, nine patients were still on CPD, two were on haemodialysis and three were transplanted. In eight patients, the primary renal disease was represented by glomerulopathy, mainly focal segmental glomerulosclerosis (n = 5). In the last 6 months prior to CPD discontinuation, 10 patients were treated with solutions containing more than 2.27% glucose. Peritonitis incidence was 1:26.8 CPD-months, similar to that calculated in children >12 months of age from the same registry (1:28.3 CPD-months). The mortality rate was 43%. A more aggressive course and an association with calcineurin inhibitors were observed in transplanted patients.
  
  
  CONCLUSIONS
  Surveillance for EPS should be maintained in high-risk children who received long-term PD even after years from CPD withdrawal.
authors.affiliation: Pediatric Nephrology, Dialysis and Transplantation Unit, Department of Pediatrics, University of Padova, Padova, Italy. enrico.vidal@inwind.it
authors.forename: Enrico
authors.initials: E
authors.lastname: Vidal
authors.forename: Alberto
authors.initials: A
authors.lastname: Edefonti
authors.forename: Flora
authors.initials: F
authors.lastname: Puteo
authors.forename: Roberto
authors.initials: R
authors.lastname: Chimenz
authors.forename: Bruno
authors.initials: B
authors.lastname: Gianoglio
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Lavoratti
authors.forename: Giovanna
authors.initials: G
authors.lastname: Leozappa
authors.forename: Silvio
authors.initials: S
authors.lastname: Maringhini
authors.forename: Francesca
authors.initials: F
authors.lastname: Mencarelli
authors.forename: Carmine
authors.initials: C
authors.lastname: Pecoraro
authors.forename: Ilse Maria
authors.initials: IM
authors.lastname: Ratsch
authors.forename: Rossella
authors.initials: R
authors.lastname: Cannavò
authors.forename: Tommaso
authors.initials: T
authors.lastname: De Palo
authors.forename: Sara
authors.initials: S
authors.lastname: Testa
authors.forename: Luisa
authors.initials: L
authors.lastname: Murer
authors.forename: Enrico
authors.initials: E
authors.lastname: Verrina
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D008297:Male; D010530:Peritoneal Dialysis / Q000009:adverse effects* / Q000401:mortality; D056627:Peritoneal Fibrosis / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D010538:Peritonitis / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D011379:Prognosis; D012042:Registries; D051436:Renal Insufficiency, Chronic / Q000150:complications*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate
keywords: children; encapsulating peritoneal sclerosis; peritoneal dialysis