The aim of this study was to define the incidence and characteristics of sc
lerosing encapsulating peritonitis (SEP) in pediatric peritoneal dialysis (
PD) patients in Japan. A questionnaire was sent to all dialysis units with
at least two pediatric PD patients. Among 687 patients registered, 11 cases
(1.6%) of SEP were diagnosed. The mean age of patients with SEP at the sta
rt of PD was 9.7+/-3.6 years and at SEP diagnosis, 19.1+/-3.8 years. All pa
tients had undergone PD for more than 5 years, and the mean PD duration was
9.6+/-3.3 years. SEP was diagnosed in 6.6% and 12% of patients dialyzed fo
r >5 years and >8 years, respectively. The incidence of peritonitis among p
atients with SEP was not different from that among the Japanese pediatric r
egistry. All patients had virtually no residual urine volume and 9 had impa
ired peritoneal ultrafiltration. Peritoneal calcification was the most-freq
uent radiological finding. Peritoneal biopsy was performed in 7 patients an
d confirmed sclerotic peritonitis in all. Ten patients transferred to hemod
ialysis, and only 1 patient underwent surgery. Three patients died. We reco
mmend that patients on PD for more than 5 years who have impaired peritonea
l ultrafiltration or peritoneal calcification should be carefully managed a
s presumptive cases of SEP.