Sclerosing encapsulating peritonitis in pediatric peritoneal dialysis patients

Abstract

The aim of this study was to define the incidence and characteristics of sclerosing 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 start of PD was 9.7+/-3.6 years and at SEP diagnosis, 19.1+/-3.8 years. All patients 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 for >5 years and >8 years, respectively. The incidence of peritonitis among patients with SEP was not different from that among the Japanese pediatric registry. All patients had virtually no residual urine volume and 9 had impaired peritoneal ultrafiltration. Peritoneal calcification was the most-frequent radiological finding. Peritoneal biopsy was performed in 7 patients and confirmed sclerotic peritonitis in all. Ten patients transferred to hemodialysis, and only 1 patient underwent surgery. Three patients died. We recommend that patients on PD for more than 5 years who have impaired peritoneal ultrafiltration or peritoneal calcification should be carefully managed as presumptive cases of SEP.

Encapsulating Peritoneal Sclerosis: Clinical Significance and Implications

“…Although peritoneal sclerosis can be induced in animal models by infusing a range of sclerosant agents into the peritoneal cavity [2] and more recently the introduction of profibrotic agents delivered to the peritoneum via adenoviruses [3], it has been difficult in animals to adequately mimic the human condition where prolonged dialysis in the context of uraemia and inflammation contributes to the formation of the abdominal cocoon. In PD patients, clinical associations have been identified with acetate buffer [4], chlorhexidine, severe PD peritonitis (in particular when due to Staphylococcus aureus , Pseudomonas , Enterococcus ), dialysate glucose exposure [5], low ultrafiltration capacity [6], absence of residual renal function (at least in children) [7] and time on PD as well as, rather paradoxically, discontinuing it [1]. The latter risk factor possibly explains recent reports of an increase in EPS following renal transplantation [8].…”

Chronic abdominal pain in a patient on chronic peritoneal dialysis: answer

“…Sclerosing encapsulating peritonitis is an infrequent complication of PD and is primarily diagnosed in adults with an incidence of 0.5-2.8% [2]. There are few reports of SEP in children: two reports from Japan [3,4], one from Turkey [5] one from Singapore [6] and one from USA [7]-for a total of 17 pediatric patients ( Table 1). The incidence of SEP in children is similar to that in adults [2]: 1.6-1.8% in Japan [3,4] and 1.9% in Turkey [5].…”

“…There are few reports of SEP in children: two reports from Japan [3,4], one from Turkey [5] one from Singapore [6] and one from USA [7]-for a total of 17 pediatric patients ( Table 1). The incidence of SEP in children is similar to that in adults [2]: 1.6-1.8% in Japan [3,4] and 1.9% in Turkey [5]. There is only one report of SEP in children from North America that focuses on the radiological features [7].…”

“…It was first reported in PD patients in 1980 and is characterized by bowel obstruction associated with gross thickening of the peritoneum [1]. The International Society of PD and the Japanese Sclerosing Encapsulating Peritonitis Study Group define SEP as a clinical entity with symptoms of persistent, intermittent or recurrent bowel obstruction with gross thickening of the peritoneum enclosing the small intestine like a cocoon [2,3]. Sclerosing encapsulating peritonitis is an infrequent complication of PD and is primarily diagnosed in adults with an incidence of 0.5-2.8% [2].…”

“…Morbidities associated with SEP include encasement of bowel loops, severe malnutrition, bowel obstruction and perforation [3][4][5]. The mortality rates from SEP are 27% [3] in children and 24-83% in adults [2].…”