Diagnosis Of Encapsulating Peritoneal Sclerosis Research Articles

Inkapsulirajuća peritoneumska skleroza - retka i ozbiljna komplikacija peritoneumske dijalize - prikaz slučaja

Introduction: End-stage renal disease is a terminal illness with a glomerular filtration rate of less than 15 mL/min. when must start one of the methods of renal replacement therapy: hemodialysis, peritoneal dialysis or kidney transplantation. Peritoneal dialysis is based on the processes of diffusion and osmosis, while the transport of substances takes place through the peritoneal membrane. The advantages of peritoneal dialysis compared to hemodialysis include longer-term preservation of residual renal function, preservation of blood vessels and a better quality of life for the patient. Complications of peritoneal dialysis can be infectious, metabolic and mechanical. Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of peritoneal dialysis (PD) treatment. The incidence of EPS is variable between 0.7 and 3.7%, while it increases with the length of PD treatment (0.7-6.4% after 5 years to as much as 17.2% after 15 years). The diagnosis of EPS is based on clinical symptoms. and signs as well as specific, but not pathognomonic, radiological and pathohistological findings. Features of IPS on computed tomography (CT) images are thickening and calcification of the peritoneal membrane, thickening of the intestinal wall, narrowing and dilatation of the in-testine, as well as "trapped" fluid collections. Case report: A 60-year-old patient, who has been treated with peritoneal dialysis for 4 years, was hospitalized due to severe gastrointestinal complaints. Due to clinical findings a peritoneal equilibration test was performed, which determined that it was a moderately fast transporter. Based on the findings of the computed tomography, the diagnosis was confirmed. Treatment is started tamoxifen and prednisone was started, the response to therapy was positive. Despite the control of sclerosing peritonitis, death occurred because of cardiac decompensation. Conclusion: Early diagnosis is necessary to start specific treatment in a timely manner and, if possible, preserve peritoneal dialysis as a treatment method and patient survival.

A case report of encapsulating peritoneal sclerosis followed by cesarean section: Clinical diagnosis and treatment experience

Encapsulating peritoneal sclerosis (EPS), also known as abdominal cocoon syndrome, is an uncommon condition that typically presents with features of bowel obstruction. EPS followed by cesarean section is extremely rare. Intestinal obstruction caused by EPS lacks specificity and poses clinical difficulties for diagnosis and treatment. We present the case of a patient with recurrent intestinal obstruction followed by cesarean section, and the diagnosis of EPS was confirmed intraoperatively. The patient recovered well postoperatively, and achieved satisfactory therapeutic results. Management of this condition tests the surgeon's knowledge and experience of the disease. A 27-year-old woman with recurrent abdominal pain and distention accompanied by reduced anal discharge and defecation there months. The patient had a history of cesarean section 4 months earlier and recovered well after operation. She had no other history of abdominal surgery or diseases. On examination, a 10-cm long transverse incision was made in the lower abdomen, and marking on the intestinal movements were observed in the left mid-abdomen. A long, soft lump with good mobility was touched in the left lower abdomen. The abdominal computed tomography and small bowel barium meal examination revealed incomplete intestinal obstruction. Incomplete small bowel obstruction due to abdominal adhesions after the cesarean section was initially considered. After conservative treatment, the symptom of intestinal obstruction still recurred. Thus, we decided to perform a surgery of repeated decortication of fibrous peritoneal membranes. The operation successfully released the intestinal obstruction and abdominal pain, postoperative course recovered smoothly. After cesarean section could develop EPS. Intestinal obstruction caused by EPS lacks specificity and poses clinical difficulty in diagnosis and treatment. The management of this condition tests the surgeon's knowledge and experience, and surgery is an effective treatment measure.

The Role of Enterolysis and Tamoxifen in Encapsulating Peritoneal Sclerosis: A Successful Case

Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis which mortality can reach 50%. The usual treatment involves suspension of the technique, medical treatment, and surgical enterolysis. We report the case of a 36-year-old female who undergone continuous ambulatory peritoneal dialysis without complications for 10 years. She presented at the emergency department with abdominal pain and a diagnosis of peritonitis was assumed. The patient developed a systemic inflammatory response, and her abdominal computer tomography (CT) scan showed multiple parietal calcifications. After multiple broad-spectrum antibiotics, an undetermined febrile syndrome persisted, and parenteral nutrition was started. An EPS diagnosis was established and treatment with tamoxifen was proposed. Exploratory laparoscopy was decided, and the intraoperative findings were consistent with EPS. The patient was submitted to extensive enterolysis. A clinical improvement led to a discharge after 60 days of hospitalization. After more than 30 months with tamoxifen, she remains on hemodialysis and doing well. There are no randomized controlled trials to guide EPS management and the following procedures are more difficult to decide, who makes these reports important to the reflection. We describe a case of successful treatment of EPS, with a very severe presentation. The management with enterolysis and tamoxifen contributed decisively to this outcome.

Encapsulating peritoneal sclerosis in an AMA-M2 positive patient: A case report

BACKGROUNDEncapsulating peritoneal sclerosis (EPS) is hard to diagnose because of nonspecific symptoms and signs. It is a general consensus that EPS is classified as primary and secondary. There have been several studies discovering some high-risk factors such as liver cirrhosis, of which AMA-M2 is a biomarker, and intra-abdominal surgery such as laparoscopic surgery. Imaging studies help to diagnose EPS and exploratory laparotomy might be an alternative if imaging fails. Nowadays, laparotomy plays a key role in treating EPS, especially when medical treatments do not work and medical therapy fails to ease patients’ symptoms.CASE SUMMARYA 58-year-old man complained of unexplained vomiting and abdominal distension 2 mo after laparoscopic cholecystectomy. Increased alkaline phosphatase and liver enzymes were discovered. An autoimmune liver disease test showed that AMA-M2 was positive. A gastroscopy revealed bile reflux gastritis. A magnetic resonance imaging scan showed a slight dilatation of the intrahepatic bile duct. A colonoscopy showed that there was a mucosal eminence lesion in the sigmoid colon (24 cm away from the anus), with a size of 3 cm × 3 cm and erosive surface. At last, the small intestine and the stomach were found to be encased in a cocoon-like membrane during the surgery. The membrane was dissected and adhesiolysis was done to release the trapped organs. The patient recovered and was discharged 44 d after the operation, and there was no recurrence during a follow-up period of 3 mo.CONCLUSIONAMA-M2 is a marker of primary biliary sclerosis and may help to make a preoperative diagnosis of EPS.

Risk factors and clinical outcomes of encapsulating peritoneal sclerosis: A case-control study from China.

Encapsulating peritoneal sclerosis (EPS) is an uncommon, but serious complication in patients with continuous ambulatory peritoneal dialysis (PD) who have a considerable mortality rate. This study aimed to identify risk factors and outcomes of EPS in Chinese patients on PD. Sixteen patients on PD who met the International Society for Peritoneal Dialysis criteria for diagnosis of EPS in the First Affiliated Hospital of Sun Yat-Sen University from 1997 to 2018 were included. Patients without EPS were matched for age, sex and the duration of PD and selected at a 1:3 ratio for the controls. A case-control study was conducted to analyse the clinical profile and risk factors associated with EPS in patients. The prevalence of EPS in patients on PD in our centre was 0.55%. The percentage of EPS significantly increased with the duration of PD. In univariate regression analysis, a history of peritonitis (odds ratios (OR): 2.83; 95% confidence interval (CI): 0.82-9.68; p = 0.08), peritoneal glucose exposure (OR: 1.12; 95% CI: 1.03-1.22; p < 0.01) and a high peritoneal transport status (OR: 14.70; 95% CI: 1.85-117.02; p < 0.01) were associated with EPS in patients on PD. However in the multivariate model, only a high peritoneal transport status (adjusted odds ratios (aOR): 13.65; 95% CI: 1.69-109.96; p = 0.01) was independently associated with EPS. The rate of EPS significantly increases with the duration of PD. Progressive peritoneal dysfunction, especially a high peritoneal transport status, is associated with a higher risk of EPS in this population.

Long-term outcomes in patients with encapsulating peritoneal sclerosis managed with nutritional support.

Little is known about long-term survivors with encapsulating peritoneal sclerosis (EPS). Published literature focuses on patients managed surgically. We describe our experience of the long-term outcomes in patients with EPS conservatively managed with nutritional support alone. This is a single-centre retrospective observational study of patients who had survived for ≥5 years since diagnosis. EPS survivors were invited for review of symptoms, nutritional assessment and evaluation of quality of life. Radiological progression was assessed based on serial computed tomography (CT) scores for each patient. A total of 23 patients with a diagnosis of EPS for at least 5 years were identified, with 18 patients alive at the time of the study. Of these 18 patients, 10 patients transferred to haemodialysis (HD) and 8 patients received kidney transplants. Commonest symptoms were nausea (91%) and vomiting (73%). Mean body mass index for patients was within the ideal and healthy range, with only 11% suffering from continued weight loss. In all, 70% EPS survivors on HD received nutritional support compared to 15% of those with transplants; 17% required ongoing parenteral nutrition. Of the 11 patients with serial CT scans at least 4 years apart, 10 had an increase in radiological score for EPS but with no apparent correlation to clinical outcomes. There were no significant differences in the reported quality of life between EPS survivors on HD and those transplanted, with self-rated health status equivalent to that reported for the general end-stage kidney disease (ESKD) population. Long-term survival following EPS managed conservatively with nutritional support is feasible, with the majority no longer requiring nutritional support and having a quality of life similar to other patients with ESKD.

Outcomes of the surgical management of encapsulating peritoneal sclerosis: A case series from a single center in Korea.

BackgroundEncapsulating peritoneal sclerosis (EPS) is a rare but near-fatal complication of peritoneal dialysis (PD). Despite the high mortality rate of EPS, the surgical treatment strategy of severe EPS is yet to be established.MethodsWe retrospectively analyzed outcomes of patients with EPS who underwent enterolysis for intractable EPS at Seoul National University Hospital between 2001 and 2018. EPS was diagnosed based on the clinical symptoms and radiological findings of abdominal computed tomography (CT). CT scans were scored according to an EPS scoring system that assessed peritoneal thickening and calcification as well as bowel thickening, tethering, loculation, and dilatation.ResultsThirteen patients (nine males and four females; age, 48 [29–63] years) underwent enterolysis for severe EPS. PD duration (11 [6–21] years) was not associated with survival. Two patients were newly diagnosed with EPS following kidney transplantation. Five patients died of infectious complications immediately after the surgery. Eight patients survived after the first surgery; however, five of them underwent reoperation but died of persistent infection, fistula formation, or adhesive bowel obstruction. Four young (< 60 years) male patients with relatively low CT scan scores (< 13) survived for > 2 years after the first surgery. Median survival duration from EPS diagnosis was 22 (1.3–184) months and that from the first surgery was 9 (0.3–153) months.ConclusionThe high mortality rate of EPS suggests the importance of appropriate surgical intervention in young symptomatic male EPS patients with relatively low CT scan scores.

Surgical Treatment for Encapsulating Peritoneal Sclerosis: 24 Years' Experience.

Encapsulating peritoneal sclerosis (EPS) is a serious complication of long-term peritoneal dialysis. The mortality rate for EPS is high, primarily due to complications related to bowel obstruction. Surgery was previously contraindicated; however, surgical enterolysis is performed for patients in whom bowel obstruction fails to improve. This was a retrospective observational study of patients with EPS who received surgical intervention at a single center between November 1993 and October 2017. The severity of intestine damage was characterized by grade-3 peritoneal calcification on abdominal computed tomography (CT) scan and degeneration of the small intestinal wall in surgery. Two-hundred and forty-three patients with EPS opted for surgery. Among them, 58 had recurrence and required re-surgery; a total of 318 EPS surgeries were performed. Death was related to EPS in 61 patients (25.1%), of whom 15 died postoperatively. Sixty-seven patients (27.6%) died from other causes. The actuarial survival rates at 1, 2, 3, 5, and 8 years after EPS diagnosis were 91%, 83%, 77%, 66%, and 53% respectively. The 50% actuarial survival points after EPS diagnosis and surgery were 104 months and 85 months, respectively. Peritoneal calcification and small intestinal wall degeneration grading showed significant association with the mortality curve for EPS-related death. Excellent outcomes for EPS are achieved with surgery. The degree of peritoneal deterioration affected the clinical outcomes. Currently, EPS is no longer recognized as a fatal complication.

Early Detection of Imminent Encapsulating Peritoneal Sclerosis: Free Water Transport, Selected Effluent Proteins, or Both?

No diagnostic tool or methodology is currently available for early detection of imminent encapsulating peritoneal sclerosis (EPS). The objective of this study was to investigate the predictive value of free water transport (FWT) and construct a panel of peritoneal effluent proteins for EPS alone or in combination with FWT. These parameters could be incorporated in the follow-up of peritoneal dialysis (PD) patients. A case-control study, nested in a longitudinal PD patient cohort, was conducted. Time-specific areas under the receiver operating characteristic (ROC) curve were calculated for FWT and effluent biomarkers at a lag time up to 3 years before EPS diagnosis. Free water transport was combined with appearance rates (AR) of biomarkers to assess their clinical validity. Free water transport volume and AR of effluent biomarkers were investigated in 11 EPS patients and 34 long-term PD patients. Diagnostic performance was best for FWT (area under the curve [AUC] 0.94) followed by plasminogen activator inhibitor (PAI-1) AR. Throughout, diagnostic panels of FWT and AR of cancer antigen 125 (CA125), interleukin-6 (IL-6), or (PAI-1) yielded specificity estimates above 84%. The combination of FWT and PAI-1 AR identified the largest proportion of EPS patients at 1 year prior to diagnosis (sensitivity 100%, specificity 94%). Measurement of FWT is simple and has the highest predictive value for imminent EPS. The addition of effluent biomarkers provides an all-round insight into the state of the peritoneum. Our data indicate that combining FWT with either PAI-1, CA125, or IL-6 has the highest specificity. This is required to avoid unnecessary discontinuation of PD treatment.

Incidence and outcomes of encapsulating peritoneal sclerosis (EPS) and factors associated with severe EPS.

BackgroundEncapsulating peritoneal sclerosis (EPS) is a rare but serious complication of long-term peritoneal dialysis (PD). However, previous studies reported large variations in its mortality rates that may associate with a different degree of EPS severity. This study reports the incidence and outcomes of EPS and identifies the risk factors associated with severe EPS.MethodsWe retrospectively analyzed clinical data of EPS patients from 3 medical centers in Taiwan from January 1982 to September 2015, and classified patients as having mild/moderate or severe EPS. Patients with intractable intestinal obstruction/gut-related sepsis that needed surgical intervention or resulted in mortality were in severe EPS group. Follow-up for outcome was through December 31, 2015. Clinical characteristics, peritoneal dialysis (PD)-related parameters, biochemical and imaging results were analyzed and compared between groups.ResultsFifty-eight of 3202 patients undergoing PD during the study period had EPS (prevalence 1.8%). The incidence of EPS increased for patients on PD for >6–8 years (≤6 yrs. vs. >6–8 yrs., 0.0% vs. 1.8%, p = 0.001). Relative to those on PD for >6–8 years, the risk of EPS significantly increased with PD duration longer than 10 years (>10–12 years vs. >6–8 years: OR: 5.5, 95% CI: 1.7–17.1, p < 0.01). Twenty-three patients fulfilled the criteria for severe EPS. The overall mortality rate of EPS was 35% (20/58), and was 74% (17/23) in the severe EPS group. The average serum levels of C-reactive protein (CRP) and intact-parathyroid hormone (i-PTH), which were checked every 3~6 months within one year before diagnosis of EPS, were higher in severe EPS group than in mild/moderate group (p = 0.02, p = 0.08, respectively). Multivariate analysis revealed severe EPS was independently associated with bowel tethering (based on CT), presentation with bloody ascites, diagnosis of EPS after withdrawal from PD, and i-PTH ≥ 384 pg/mL. Receiver operating characteristic analysis indicated that presentation with 2 or more of the 5 risk factors (EPS diagnosis after PD withdrawal, bloody ascites, bowel tethering, CRP ≥ 29 mg/L, and i-PTH ≥ 384 pg/mL) had a good accuracy (AUC = 0.80, p = 0.001) for prediction of severe EPS.ConclusionsThe incidence of EPS increases with PD duration. Severe EPS has high mortality rate and is associated with bowel tethering, presentation of bloody ascites, diagnosis after PD withdrawal, and higher serum levels of i-PTH before EPS diagnosis. Having 2 or more of the 5 risk factors can provide a good accuracy for prediction of severe EPS.

A prospective, proteomics study identified potential biomarkers of encapsulating peritoneal sclerosis in peritoneal effluent

Encapsulating peritoneal sclerosis (EPS) is a potentially devastating complication of peritoneal dialysis (PD). Diagnosis is often delayed due to the lack of effective and accurate diagnostic tools. We therefore examined peritoneal effluent for potential biomarkers that could predict or confirm the diagnosis of EPS and would be valuable in stratifying at-risk patients and driving appropriate interventions. Using prospectively collected samples from the Global Fluid Study and a cohort of Greek PD patients, we utilized 2D SDSPAGE/ MS and iTRAQ to identify changes in the peritoneal effluent proteome from patients diagnosed with EPS and controls matched for treatment exposure. We employed a combinatorial peptide ligand library to compress the dynamic range of protein concentrations to aid identification of low-abundance proteins. In patients with stable membrane function, fibrinogen γ-chain and heparan sulphate proteoglycan core protein progressively increased over time on PD. In patients who developed EPS, collagen-α1(I), γ-actin and Complement factors B and I were elevated up to five years prior to diagnosis. Orosomucoid-1 and a2-HS-glycoprotein chain-B were elevated about one year before diagnosis, while apolipoprotein A-IV and α1-antitrypsin were decreased compared to controls. Dynamic range compression resulted in an increased number of proteins detected with improved resolution of protein spots, compared to the full fluid proteome. Intelectin-1, dermatopontin, gelsolin, and retinol binding protein-4 were elevated in proteome-mined samples from patients with EPS compared to patients that had just commenced peritoneal dialysis. Thus, prospective analysis of peritoneal effluent uncovered proteins indicative of inflammatory and pro-fibrotic injury worthy of further evaluation as diagnostic/prognostic markers.

CD147 expression in peritoneal injury.

Peritoneal injury is an important cause of technical failure of long-term peritoneal dialysis (PD). Encapsulating peritoneal sclerosis (EPS) is a severe complication of long-term PD with potentially life threatening consequences. CD147 is a glycoprotein with diverse functions including modulation of extracellular matrix via induction of matrix metalloproteinases, cell adhesion, and regulation of immune reactions. We hypothesized that CD 147 plays a role in the peritoneal cavity. In this retrospective study, we localized CD147 by immunohistochemistry in peritoneal biopsies from uremic patients not on PD (n = 8), on PD without signs of EPS (n = 7), and in biopsies in patients with the diagnosis of EPS (n = 7). Double immunofluorescence was used to co-localize α-smooth-muscle actin (α-SMA) and CD147 in selected biopsies from each group. Expression was scored semi-quantitatively. In biopsies from uremic controls, CD147 was prominently expressed in mesothelial cells, focally between fat cells and by some perivascular cells. In patients on PD, a similar distribution was present (although mesothelium was rarely conserved), with some focal accentuation. In EPS, layers of fibroblastic cells were positive for CD147. EPS biopsies demonstrated a significantly higher score in a blinded evaluation, compared to uremic patients. Cells expressing CD147 were α-SMA positive myofibroblasts as demonstrated by double immunofluorescence. Mean CD147 scores did not differ between patients with different transporter status. This is the first study demonstrating CD147 on a major part of fibroblastic cells in EPS. Future studies need to address the role of these cells in this severe complication of long-term PD.

Encapsulating Peritoneal Sclerosis

Encapsulating Peritoneal Sclerosis (EPS) developed in an African American male who had been on Peritoneal Dialysis for nine years. During his hospitalization for peritonitis, he continued to clinically deteriorate with refractory abdominal pain, vomiting, and anorexia requiring Total Parenteral Nutrition. This case demonstrates the importance of prompt EPS diagnosis and the technical challenges associated with surgical treatment.

Encapsulating peritoneal sclerosis in an Italian center: thirty year experience.

Encapsulating peritoneal sclerosis (EPS) is a rare but life-threatening complication of peritoneal dialysis (PD). Its incidence and prevalence are still not clearly defined. No data exist on the prevalence of EPS in Italy. To evaluate the incidence and prevalence of EPS, and identify potential factors useful for prevention or early diagnosis of EPS. A retrospective study in patients starting PD between 1979 and 2013 in one Italian center. Data on demographics, occurrence of EPS, time on PD, peritoneal equilibration test, and therapy for EPS were gathered. EPS occurred in 26/920 patients with a prevalence of 2.8 % and incidence of 1/105 patient-years. The prevalence increased with the time spent on PD: 0.4 % for PD duration <2 years, 3 % (2-4 years), 4 % (4-6 years), 6 % (6-8 years), 8 % (8-10 years), 18 % (10-12 years), 75 % (12-14 years), 67 % (>14 years). EPS prevalence was not higher in PD patients transplanted: 5/172 (2.9 %); only two of them (1.2 %) were diagnosed while with a functioning graft. In only one patient (0.6 %) was the diagnosis made during hemodialysis; the other 23 were diagnosed while still on PD. Mortality due to EPS was 38.5 %, and was associated with PD duration. Therapy with steroids reduced mortality [hazard ratio 0.047 (95 % CI: 0.008-0.273); p < 0.001]. In our experience the prevalence of EPS is low, but increases progressively with the duration of PD. The transfer to hemodialysis or transplantation does not appear to be a key factor for EPS. Therapy with steroids significantly improves the outcome.

MTOR inhibitors for medical treatment of post-transplantation encapsulating peritoneal sclerosis: a favourable single center experience.

Encapsulating peritoneal sclerosis (EPS) is a serious complication in patients on peritoneal dialysis (PD) causing intestinal obstruction. Two different forms of EPS are reported: the classical one observed in patients on PD, and post-transplantation EPS (PostTx-EPS). The first-line therapy of classical and PostTx-EPS remains surgical treatment, but for both the complication rate and mortality are high. Recently, a few cases of EPS were successfully treated with inhibitors of mammalian target of rapamycin (mTORi). The aim of this study was to evaluate PostTx-EPS outcome in our patients, focusing on the potential benefit of mTORi treatment. We performed a retrospective analysis on 1,048 kidney transplanted patients at our center between 11/2001 and 12/2011. In the 226 patients treated with PD at any time before grafting, we found 10 cases of PostTx-EPS (prevalence 4.4%). The mean age was 54.9 years (26-69), with a mean time on PD of 83.1 months (33-156). The interval between kidney transplant and EPS diagnosis was 10.5 months (4-18.9). Five of the ten patients were treated after the diagnosis with mTORi, with a favorable outcome in 4/5 cases. This result was substantially independent of surgical and steroid therapy, performed in 9/10 and 10/10 patients respectively. EPS is a serious complication but susceptible to improvement if early diagnosed. mTORi represent a useful option for EPS treatment. We too suggest adopting an immunosuppressive protocol based on mTORi, mycophenolate mofetil and steroids in order to prevent PostTx-EPS in transplanted patients at high risk.

Peritoneal Effluent MMP-2 and PAI-1 in Encapsulating Peritoneal Sclerosis

Recently, the use of effluent matrix metalloproteinase 2 (MMP-2) and plasminogen activator inhibitor 1 (PAI-1) as potential biomarkers of peritoneal fibrosis has been demonstrated during longitudinal follow-up of incident peritoneal dialysis (PD) patients. This study focuses on effluent MMP-2 and PAI-1 as early diagnostic markers in the preceding years of patients who develop encapsulating peritoneal sclerosis (EPS). Diagnostic test study. PD patients who developed EPS were compared with controls using a 1:3 case-control design with a minimum PD duration of 57 months. Dialysate appearance rates of MMP-2 and PAI-1. EPS cases identified by 2 experienced nephrologists and a radiologist based on predefined criteria. 11 patients developed EPS within our center. The time course of MMP-2 appearance rates, studied by means of a linear repeated-measures model 4 years prior to the diagnosis of EPS, showed no difference between long-term controls and patients with EPS. In contrast, higher PAI-1 appearance rates were found in patients with EPS compared with controls (P=0.01). At a lag time of 1 year prior to EPS diagnosis, time-specific receiver operating characteristic curve analyses indicated a discriminative ability for PAI-1 appearance rate of 0.77 (95% CI, 0.63-0.91). A discriminative capacity was absent for those of MMP-2. Low event rate of EPS prevented independent validation in this single-center study. Elevated levels of PAI-1 appearance rates are present in patients who develop EPS, pointing to progressive peritoneal fibrosis and sclerosis. The PAI-1 appearance rate has fair discriminative capacity from 3 years prior to EPS diagnosis. Therefore, effluent PAI-1 may aid in monitoring peritoneal fibrosis and serve as a biomarker for EPS.

Post-transplantation encapsulating peritoneal sclerosis without inflammation or radiological abnormalities

BackgroundPost-transplantation encapsulating peritoneal sclerosis (EPS) causing bowel obstruction has been identified as a serious complication after kidney transplantation in patients previously treated with peritoneal dialysis. Systemic inflammation and abnormalities on an abdominal computed tomography (CT) scan are important hallmarks of EPS. To our knowledge, this is the first report of a case being diagnosed with late-onset post-transplantation EPS without systemic inflammation or abnormalities on a CT scan which could only be diagnosed by laparotomy.Case presentationA 59-year old female presented because of symptoms of bowel obstruction 33 months after kidney transplantation. The patient had a 26-month history of peritoneal dialysis before her first kidney transplantation and was treated with peritoneal dialysis for 4 years before undergoing a second kidney transplantation. Physical examination was unremarkable and laboratory tests showed no signs of systemic inflammation (C-reactive protein <1 mg/L). An abdominal CT scan did not reveal any abnormalities fitting the diagnosis of EPS, except a “feces sign”. Given the severity of the progressive symptoms, a diagnostic laparotomy was performed, visualizing a classical EPS. Total peritonectomy and enterolysis were performed, leading to restoration of peristalsis.ConclusionEPS may occur several years after kidney transplantation in the absence of inflammation and typical radiological abnormalities. Obtaining a diagnosis of post-transplantation EPS is challenging, however, a low threshold for surgical exploration in case of high clinical suspicion and negative findings on the CT scan is mandatory.

Encapsulating peritoneal sclerosis in paediatric peritoneal dialysis patients: the experience of the Italian Registry of Pediatric Chronic Dialysis

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. 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. 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. Surveillance for EPS should be maintained in high-risk children who received long-term PD even after years from CPD withdrawal.

Lower Mortality and Inflammation from Post-Transplantation Encapsulating Peritoneal Sclerosis Compared to the Classical Form

Background: Encapsulating peritoneal sclerosis (EPS) may occur after kidney transplantation (post-transplantation EPS) or may be diagnosed during or after peritoneal dialysis treatment (classical EPS). The aim of the present study was to investigate to what extent both EPS entities differ in clinical presentation, radiological findings, outcome, and the systemic inflammatory response, as measured by plasma C-reactive protein (CRP) levels both prior to and after EPS diagnosis. Methods: We performed a retrospective analysis of 15 post-transplantation EPS and 19 classical EPS patients who were diagnosed at seven hospitals in the Netherlands between January 1, 2000, and January 1, 2011. Results: There were no inter-group differences in age, duration of peritoneal dialysis, clinical presentation, or radiology findings at diagnosis. Post-transplantation patients had experienced a lower number of peritonitis episodes per patient-year (0.2 (0.0-0.4) vs. 0.7 (0.3-1.2), p = 0.01) with a longer interval between the last peritonitis and EPS diagnosis (18.1 (4.6-34.3) vs. 4.4 (0.89-13.78) months, p = 0.01). Post-transplantation EPS patients showed a remarkably lower mortality rate (40.0 vs. 84.2%, p < 0.05). In both groups a pattern of elevated CRP values was observed, increasing within the year before EPS diagnosis. In the post-transplantation group the median CRP level at diagnosis was lower (56.0 vs. 144.50 mg/l, p < 0.05) than in the classical EPS group. Conclusion: Post-transplantation EPS has a similar clinical presentation as classical EPS but with a lower systemic inflammatory response and better outcome.

Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group

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. 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. 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. 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.