Encapsulating peritoneal sclerosis in paediatric peritoneal dialysis patients

Encapsulating peritoneal sclerosis (EPS) is a serious complication of chronic peritoneal dialysis (CPD). In contrast to the adult population, there are few studies regarding EPS in paediatric CPD patients, and the majority of reported patients are from Japan. The aim of the present report is to define the incidence of EPS in our paediatric CPD patients and to describe the clinical and laboratory characteristics. A total of 104 paediatric patients were followed from November 1989 to November 2003 and two were diagnosed as EPS (1.9%). The dialysis periods of these patients were 45 and 53 months with 6 and 8 peritonitis episodes, respectively. Clinical signs of EPS developed 7 and 14 days after the removal of the dialysis catheter, and CPD was replaced by haemodialysis because of persistent peritonitis. One patient was well after surgical management but died 6 months later. The second patient who was treated with prednisolone remained well at 16 months. In conclusion, EPS is a rare but important complication of CPD. We recommend that all patients on CPD who develop ultrafiltration failure be evaluated radiologically for the occurrence of EPS. Management should be tailored to the individual patient.     

Development of gene therapy for encapsulating peritoneal sclerosis by a chimeric DNA-RNA hammerhead ribozyme targeting TGF-beta1 mRNA

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a rare and devastating fibrotic complication in patients treated with peritoneal dialysis. Transforming growth factor-beta1 (TGF-beta1) has been reported to be a pivotal factor in the induction of EPS. Ribozymes are RNA molecules that enzymatically cleave the target mRNAs and are expected to be utilized as a novel nucleic acid-based therapy. We examined the effects of the chimeric DNA-RNA hammerhead ribozyme targeting TGF-beta1 mRNA on a peritoneal sclerosis rat model to develop a possible gene therapy for EPS. METHODS: To create an animal model of peritoneal sclerosis, rats were given a daily intraperitoneal injection of chlorhexidine gluconate and ethanol dissolved in saline (CHX) for 14 days. On day 4, the chimeric ribozyme or mismatch ribozyme was intraperitoneally injected. On day 15, samples of peritoneum were obtained from the rats, and expression of TGF-beta1 mRNA and fibronectin mRNA in peritoneal tissues were evaluated by quantitative real-time PCR analysis. RESULTS: Injections of CHX significantly increased the submesothelial thickness, and increased the expression of TGF-beta1 and fibronectin mRNA in the rat peritoneum. Treatment with the chimeric ribozyme significantly reduced the CHX-induced peritoneal thickness, and expression of TGF-beta1, and fibronectin mRNA in peritoneal tissues. CONCLUSIONS: These results indicate that the chimeric DNA-RNA hammerhead ribozyme targeting TGF-beta1 mRNA has the potential for use as a gene therapy agent for EPS.     

Encapsulating peritoneal sclerosis

Since the first peritoneal dialysis (PD) patients with encapsulating peritoneal sclerosis (EPS) were reported in 1980, EPS has been considered primarily a fatal complication. The incidence of EPS in PD patients has been reported to be from 0.7% to 7.3%, and the rate appears to be higher in patients receiving long-term treatment. Most data from Japan has shown an overall incidence of 2.5% with an evident negative effect of increasing duration of PD, which also augments mortality. Since EPS occurred after withdrawal from PD in more than half of the patients, strict monitoring is necessary when a long-term PD patient is withdrawn from PD. Maintaining patients on standard PD for more than 8 years using conventional solutions is associated with a substantial risk for development of EPS. Appropriate treatment according to the disease stage is most important in EPS treatment. Therefore, when examining a PD patient complaining of gastrointestinal symptoms, the possibility of EPS has to be kept in mind. Basic therapeutic tactics for EPS include appropriate use of steroids. If the state of bowel obstruction persists, laparotomy and enterolysis should be performed to obtain a complete cure. It is now recognized that EPS is not a fatal complication of PD.     

A case of encapsulating peritoneal sclerosis at the clinical early stage with high concentration of matrix metalloproteinase-2 in peritoneal effluent

In encapsulating peritoneal sclerosis (EPS), matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases are involved in the remodeling of peritoneal tissue. We measured the MMP-2 concentration in the peritoneal effluents of a patient with EPS who discontinued continuous ambulatory peritoneal dialysis (CAPD) therapy because of ultrafiltration failure and/or underdialysis. First, we report a 58-year-old female patient who discontinued CAPD therapy because of underdialysis. Several months after cessation of CAPD, she complained of slightly blood-colored ascites and had an elevated level of C-reactive protein (CRP) in plasma. She was diagnosed as having clinical early-stage EPS. Peritoneal effluents drained from this case, and from 11 patients who discontinued CAPD therapy because of ultrafiltration failure and/or underdialysis, and who underwent peritoneal lavage with 1.5% dextrose peritoneal dialysis fluid for several months, were analyzed by gelatin zymography. MMP-2 concentration was also measured by enzyme-linked immunosorbent assay (ELISA). MMP-2 concentration in peritoneal effluent of this patient was highest compared with that of the other patients. There was some tendency of a positive correlation between MMP-2 concentration per 1g protein and D/Pcr, and was negative correlation between MMP-2 concentration per 1g of protein and D/D0 glucose. We concluded that MMP-2 is involved in the peritoneal remodeling of long-term CAPD therapy and the progression of EPS.     

Single-center experience of encapsulating peritoneal sclerosis in patients on peritoneal dialysis for end-stage renal failure

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication in patients undergoing continuous ambulatory peritoneal dialysis (CAPD) or automated peritoneal dialysis (APD). It is characterized by a progressive, intra-abdominal, inflammatory process resulting in sheets of fibrous tissue that cover, bind, and constrict the viscera, thereby compromising the motility and function of the bowel. Although recent therapeutic approaches have been reported with variable success, the ability to detect reliably at an early stage patients at risk for EPS would be beneficial and allow treatment standardization. The aim of this study was to evaluate the clinical features of EPS and identify possible risk factors for its development in CAPD and APD patients. METHODS: This was a review of all cases of EPS in a single center over the last 5 years. RESULTS: There were 810 CAPD and APD patients, managed in our program over this period. We identified 27 cases of EPS, giving an overall of 3.3% in this population. The mean duration of CAPD before diagnosis of EPS was 72.6 +/- 39.7 months (range 16-172). Sixteen cases required surgical treatment and were classified as severe; others were treated conservatively (mild to moderate group). Ten patients received tamoxifen treatment with apparent benefit. The overall mortality rate was 29.6%. Eight patients from the severe group and the entire moderate group survived on hemodialysis or transplantation at 48.71 and 27.63 months follow-up, respectively. Peritonitis rates were not different between the 2 groups and peritoneal history was unremarkable compared to overall peritonitis rates in the unit. Data on small solute transport were not available in all patients in this retrospective analysis. CONCLUSION: EPS is a serious, life-threatening complication of CAPD. Most cases had PD duration of more than 4 years. Careful monitoring by CT scans of the peritoneal membrane in patients beyond 5 years, and early catheter removal in patients with peritoneal thickening should be considered for long-term CAPD patients. Treatment with tamoxifen may be of benefit in these patients.     

Long-term peritoneal dialysis and encapsulating peritoneal sclerosis in children

Encapsulating peritoneal sclerosis (EPS) is the most serious complication of long-term peritoneal dialysis (PD), with a mortality rate that exceeds 30%. There have been many reports of the incidence of EPS being strongly correlated to the duration of PD. Patients on PD for longer than 5 years, and especially those receiving this treatment for more than 8 years, should undergo careful and repeated surveillance for risk factors associated with the development of EPS. The development of ultrafiltration failure, a high dialysate/plasma creatinine ratio, as determined by the peritoneal equilibration test, peritoneal calcification, a persistently elevated C-reactive protein level, and severe peritonitis in patients on PD for longer than 8 years are signals that should prompt the clinician to consider terminating PD as a possible means of preventing the development of EPS. The impact of the newer, biocompatible PD solutions on the incidence of EPS has not yet been determined.     

Encapsulating peritoneal sclerosis: what have we learned?

Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD), but carries significant morbidity and mortality. We review the clinical features and radiologic and histologic changes found at diagnosis of EPS. Although EPS is strongly associated with the duration of PD, the pathogenesis remains only partly understood. We discuss the mechanisms thought to underlie the abnormally thickened, sclerotic peritoneal membrane seen in long-term PD patients including epithelial to mesenchymal transition and the molecular mediators of fibrosis and angiogenesis. We review how exposure to high-glucose, nonphysiological dialysis fluids, peritonitis, and uremia may be responsible for these changes. Much remains to be learned about optimal management of EPS, both medical and surgical, because the literature lacks controlled studies. Future research challenges include defining the role of surgery, immunosuppression, and antifibrotic agents in the management of EPS. We also need to understand why some patients progress from asymptomatic peritoneal sclerosis to the extreme levels of fibrin deposition and bowel encapsulation seen in EPS. Screening PD patients for potential future EPS remains difficult, and we need strategies for monitoring patients on longer-term PD that enable us to better quantify the risk of EPS for the individual patient.     

Pathophysiology of encapsulating peritoneal sclerosis: lessons from findings of the past three decades in Japan

Encapsulating peritoneal sclerosis (EPS), a condition with a high mortality rate, is a serious complication of peritoneal dialysis (PD). In Japan, EPS became a central issue in the clinical setting during the mid-90s and the beginning of this century. However, following the introduction of biocompatible neutral PD solutions containing lower levels of glucose degradation products, the incidence and clinical severity of EPS has been greatly lessened. During the past three decades, the etiology of EPS has been elucidated by findings obtained by peritoneal biopsy, laparoscopy, and surgical intervention. Accumulating findings suggest the need for a paradigm change on the nature of EPS pathophysiology; notably, EPS appears not to reflect peritoneal sclerosis per se, but rather the formation of a neo-membrane as a biological reaction to peritoneal injury. This narrative review looks back on the history of EPS in Japan, and discusses EPS pathophysiology, the impact of neutral PD solution on peritoneal protection, and a future novel diagnostic approach, ultra-fine endoscope, for the identification of patients at high risk of EPS.

     

Additive effectiveness of everolimus plus tamoxifen therapy in treatment of encapsulating peritoneal sclerosis

Peritoneal dialysis (PD) is one of the commonly used choices of continuous renal replacement therapies. Peritoneal membrane is damaged by using solutions with lower biocompatibility, peritonitis episodes, and vintage of PD therapy. Encapsulating peritoneal sclerosis (EPS) is a rare complication of PD and is presented by progressive fibrosis of the peritoneum. Fibrous tissue entrapment of the intestine, leading to complete intestinal obstruction, is referred to as EPS, the most severe form of sclerosing peritonitis. EPS is irreversible fibrosis of the peritoneal membrane usually associated with high rates of morbidity and mortality. Preventive strategies are the best choice of treatment. Also there is no proven effective therapy for EPS; there are only small-sized trials. Herein we present a case of EPS who improved with everolimus plus tamoxifen therapy.     

Update on potential medical treatments for encapsulating peritoneal sclerosis; human and experimental data

Encapsulating peritoneal sclerosis (EPS) is an infrequent but serious complication of peritoneal dialysis (PD). The pathogenesis is unknown but speculation is ongoing. The current management of EPS focuses on prevention and treatment of the inflammatory and fibrotic changes at the level of the peritoneal membrane with immunosuppressive and antifibrotic agents, respectively. This article reviews the currently available human and animal data on potential agents to prevent and/or treat EPS. We propose a strategy for early diagnose EPS in an attempt to avoid the development of the full-blown and potentially life-threatening clinical syndrome of EPS. Future research should focus on studying potential prophylactic and therapeutic agents in humans in large, multicenter, randomized trials but also on early detection of EPS in the inflammatory phase by means of biomarkers and the establishment of a composite EPS score.     

Restoration of peritoneal integrity after withdrawal of peritoneal dialysis: characteristic features of the patients at risk of encapsulating peritoneal sclerosis

Background The epidemiological characteristics of encapsulating peritoneal sclerosis (EPS), such as its high incidence in patients with long-term peritoneal dialysis (PD) treatment, and the onset of EPS after patients are switched to hemodialysis (HD) may indicate an activated pathological process after PD withdrawal, especially in long-term PD patients. Accordingly, we aimed to observe changes in peritoneal function after the stoppage of PD, and to clarify the characteristic features of the patients at risk of EPS. Methods Thirty-three patients who were switched from continuous ambulatory peritoneal dialysis (CAPD) to HD were enrolled in this trial. Changes in the dialysate/plasma creatinine (D/P Cr) and CA125 levels in the effluent of the peritoneal equilibration test were observed for 6 months. Furthermore, each patient was followed-up for 36 months after PD withdrawal to monitor for the development of EPS. Results D/P Cr decreased significantly, while CA125 levels tended to increase. Nine patients developed EPS during the follow-up period and they specifically showed significant increases of D/P Cr levels and significantly lower levels of CA125 at PD withdrawal. The accumulation of high transporters in the EPS group at 0 and 6 months after PD withdrawal was significant. Conclusions Peritoneal recovery may take place after withdrawal from PD treatment and such recover indicated by improvement of transport states and a rise of the CA125 level. The present study revealed that a high-transport state and lack of increase of CA125 in the effluent were associated with EPS development after PD withdrawal. This may suggest that the lack of peritoneal recovery after PD withdrawal is predictive for EPS development.     

Interstitial Fibrosis Restricts Osmotic Water Transport in Encapsulating Peritoneal Sclerosis

Encapsulating peritoneal sclerosis (EPS) is a rare but severe complication of peritoneal dialysis (PD) characterized by extensive fibrosis of the peritoneum. Changes in peritoneal water transport may precede EPS, but the mechanisms and potential predictive value of that transport defect are unknown. Among 234 patients with ESRD who initiated PD at our institution over a 20-year period, 7 subsequently developed EPS. We evaluated changes in peritoneal transport over time on PD in these 7 patients and in 28 matched controls using 3.86% glucose peritoneal equilibration tests. Compared with long-term PD controls, patients with EPS showed early loss of ultrafiltration capacity and sodium sieving before the onset of overt EPS. Multivariate analysis revealed that loss of sodium sieving was the most powerful predictor of EPS. Compared with long-term PD control and uremic peritoneum, EPS peritoneum showed thicker submesothelial fibrosis, with increased collagen density and a greater amount of thick collagen fibers. Reduced osmotic conductance strongly correlated with the degree of peritoneal fibrosis, but not with vasculopathy. Peritoneal fibrosis was paralleled by an excessive upregulation of vascular endothelial growth factor and endothelial nitric oxide synthase, but the expression of endothelial aquaporin-1 water channels was unaltered. Our findings suggest that an early and disproportionate reduction in osmotic conductance during the course of PD is an independent predictor of EPS. This functional change is linked to specific alterations of the collagen matrix in the peritoneal membrane of patients with EPS, thereby validating the serial three-pore membrane/fiber matrix and distributed models of peritoneal transport.     

Long-standing high-transport membrane as a risk factor for EPS development after PD withdrawal: an analysis based on changes in peritoneal function during and after CAPD withdrawal

The pathophysiology of encapsulating peritoneal sclerosis (EPS) that develops after withdrawal from long-standing dependence on CAPD remains unclear. The aim of this study was to clarify the risk factors for EPS as expressed in the peritoneal function. Fourteen patients who had shifted to standard hemodialysis after long-term CAPD (average, 105 months) were studied: 3 developed EPS after PD withdrawal while 11 did not. Analysis of the data obtained from the peritoneal equilibration test (PET) revealed that: (1) the dialysate/plasma creatinine ratio (D/Pcr) was significantly higher in the EPS group than in the non-EPS group during the course of PD as well as after PD withdrawal; and (2) eight patients, including the 3 with EPS, were classified as being in a high-transport membrane state (HTS) at PD withdrawal. The duration of HTS during PD was longer in those patients with EPS. During the periods after PD withdrawal, none of these EPS patients recovered from HTS, whereas 4 of the 5 non-EPS patients did. These data suggest that long-standing HTS during the course of PD as well as post-withdrawal, may be risk factors for EPS development. For this reason, it is indicated that PET has clinical relevance in examining sequential changes in peritoneal function and in detecting those patients at risk of EPS.     

Risk factors for encapsulating peritoneal sclerosis in patients who have experienced peritoneal dialysis treatment

Background The present study was conducted to clarify the clinical risk factors related to the development of encapsulating peritoneal sclerosis (EPS), which is one of the most serious complications in patients undergoing peritoneal dialysis (PD).Methods The records of 78 patients with a history of PD treatment, including 18 with EPS, were retrospectively analyzed (male/female, 51:27; age, 51.8 ± 11.0 years; PD treatment, 94.1 ± 42.7 months). The inclusion criteria were: duration of PD more than 24 months; 36-month follow up after discontinuation of PD; available data for dialysate-to-plasma creatinine ratio (D/P Cr), by fast peritoneal equilibration test within 3 months before PD discontinuation; and absence of EPS at PD discontinuation. Analytical parameters included age, sex, underlying renal disease, duration of PD, membrane transport state (higher transporter or lower transporter: D/P cr ratio more than or less than 0.75), number of episodes of peritonitis during PD treatment, performance of peritoneal lavage after PD discontinuation, and reasons for PD withdrawal (ultrafiltration failure, acute peritonitis, social matters).Results Significant differences were noted regarding the PD duration, D/P cr, higher membrane transport state, and number of peritonitis episodes during PD. On receiver operating characteristic curves, the cutoff points for EPS were: D/P cr ratio, 0.74; number of peritonitis episodes, 2; and PD duration (months), 115.2. Multivariate analysis, employing the factors age, PD duration, higher membrane transport state, and number of peritonitis episodes, which were selected by stepwise analysis, identified the latter two factors as significant for the development of EPS (odds ratio [OR], 4.0; P = 0.046 and OR, 12.0; P = 0.049, respectively).Conclusions A higher transporter membrane state and the number of peritonitis episodes are factors contributing to the occurrence of EPS in patients who have experienced PD treatment.     

Posttransplantation Encapsulating Peritoneal Sclerosis Contributes Significantly to Mortality after Kidney Transplantation

Encapsulating peritoneal sclerosis (EPS) is a severe complication of peritoneal dialysis (PD) and may present after kidney transplantation, a condition known as posttransplantation EPS. The prevalence and impact of posttransplantation EPS on survival after kidney transplantation is unknown. From January 1, 1996 until July 1, 2007, 1241 PD patients were transplanted. Thirty‐eight cases of posttransplantation EPS (3%) were identified from the Dutch multicenter EPS study. In EPS patients the mean pretransplant dialysis duration was longer than in the controls (71.4 ± 37.5 months vs. 34.7 ± 25.5, p < 0.0001). The majority of EPS cases were observed within the first 2 years after transplantation, but some cases appeared many years after transplantation. Two hundred and one (16.2%) patients died after transplantation, of which 17 were EPS patients. After infection (23.9%), cardiovascular disease (21.9%) and malignancy (10.9%), EPS (8.5%) was the fourth known cause of death after transplantation. Kaplan–Meier analysis showed a significant decreased survival for transplanted patients with posttransplantation EPS compared to transplanted patients without EPS. In conclusion, posttransplantation EPS is rare but carries a high mortality. A prolonged clinical vigilance and a high index of suspicion for the diagnosis are warranted, specifically in PD patients with a relatively long cumulative pretransplant duration of PD.     

The potential of matrix metalloproteinase-2 as a marker of peritoneal injury, increased solute transport, or progression to encapsulating peritoneal sclerosis during peritoneal dialysis--a multicentre study in Japan.

BACKGROUND: Long-term peritoneal dialysis (PD) leads to peritoneal injury. At worst, peritoneal injury leads to encapsulating peritoneal sclerosis (EPS), which is a serious complication of PD. The mortality rate of EPS is extremely high. To perform PD safely, monitoring of peritoneal injury that leads to EPS is a necessity. METHODS: A total of 444 PD patients with end-stage renal disease at 60 centres in Japan were analysed (sex, 54% males; median age, 56 years; median PD duration, 55 months). Matrix metalloproteinase (MMP)-2 and MMP-9 in the peritoneal effluents were analysed with gelatin zymography or enzyme-linked immunosorbent assay. Cells expressing MMP-2 in the peritoneal tissue were investigated immunohistologically with anti-MMP-2 antibodies. Peritoneal solute transport was assessed with the peritoneal equilibration test (PET). RESULTS: The MMP-2 levels in peritoneal effluents obtained with the PET were significantly correlated with the D/P Cr ratio (R = 0.69, P < 0.001) and the D/D0 glucose ratio (R = -0.59, P < 0.001). The MMP-2 levels in patients with mild peritoneal injury, moderate peritoneal injury, severe peritoneal injury (EPS) and infectious peritonitis were significantly higher than those in control patients (P < 0.001, P < 0.001, P < 0.01 and P < 0.05, respectively). MMP-2 was produced by myofibroblast-like mesenchymal cells and macrophages in the peritoneum. The peritoneal effluents from patients with infectious peritonitis showed strong MMP-9 signals. CONCLUSIONS: From these results, MMP-2 levels in peritoneal effluents reflect peritoneal solute transport and changes in MMP-2 levels are associated with peritoneal injury that leads to EPS. MMP-2 may be a useful marker of peritoneal injury, increased solute transport or progression to EPS.     

Possible pathologic involvement of receptor for advanced glycation end products (RAGE) for development of encapsulating peritoneal sclerosis in Japanese CAPD patients

Encapsulating peritoneal sclerosis (EPS) is a serious complication of PD. The cause(s) of EPS are unknown but may include peritonitis and long duration of PD treatment. However, EPS may also develop in some patients without a history of peritonitis or with rather short duration of PD therapy. It has been suggested that an increasing peritoneal solute transport rate (PSTR) as a function of time on PD treatment is a risk factor for EPS development after transfer to hemodialysis, and that high PSTR is associated with an increased peritoneal microvessels surface area. Other putative mechanisms might include advanced glycated end products (AGE) and their receptors, RAGE. The purpose of this study was to investigate genetic variations in PD patients developing EPS in comparison to PD patients without EPS. SNPs in genes related to angiogenesis as well as RAGE were analyzed. Twenty patients (M/F: 12/8, mean age at start of PD 42.2 years, mean duration of PD 8.4 years) who were diagnosed as EPS during the period 1982 - 2002 at Jikei University Hospital and a matched control group (n = 20) of nonEPS PD patients were studied. The following 5 SNPs were analyzed: VEGF 936 C/T, ecNOS -786 T/C, 298 Glu/Asp, and RAGE -374 T/A, and -429 T/C. The SNPs were analyzed by the pyrosequencing method. The C allele (T/C and C/C) in the RAGE -429T/C genotype was not found in any of the EPS patients (EPS, T/T: 20/20 (100%), nonEPS, T/T: 15/20 (75%), T/C: 4/20 (20%), C/C: 1/2 0(5%), nonC allele vs C allele, p = 0.013), although every allele was found in other SNPs. We conclude that these preliminary data show that whereas genotypes directly related to angiogenesis did not differ between EPS and nonEPS patients, it is noteworthy that no patients in the EPS group had a C allele in the RAGE -429T/C genotype. This might indicate a possible genetic contribution to the development of EPS that is related to RAGE.     

Serum beta2 microglobulin (beta2MG) level is a potential predictor for encapsulating peritoneal sclerosis (EPS) in peritoneal dialysis patients

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a serious complication in patients undergoing continuous ambulatory peritoneal dialysis (CAPD) or automated peritoneal dialysis (APD). The aim of this study was to find a predictor for EPS. METHODS: Patients with EPS who were detected by a historical cohort study using clinical data of 219 CAPD patients at our hospital. We recruited 25 patients with EPS who were compared with the patients without EPS who were matched for age and dialysis period as controls. Differences between the two groups (non-EPS group and EPS group) with respect to age, gender, primary disease, dialysis period, serum urea nitrogen, serum creatinine, beta2MG, CRP and PET (peritoneal equilibration test) category (determined by the peritoneal function testing) were analyzed. RESULTS: According to multiple regression analysis, a high beta2MG level was an independent risk factor for EPS (odds ratio 1.162, 95% confidence interval 1.026 - 1.317, p = 0.018). Other clinical markers did not show positive significance. A ROC (receiver operating characteristic) curve was prepared to evaluate the suitability of I(2)2MG measurement as a screening test. The sensitivity was 64% and the specificity was 80% when a beta2MG level of 37.0 mg/dl was taken as the cut-off value. The odds ratio for occurrence of EPS was 8.8 when beta2MG level was in the range of 35 - 40 mg/dl, 13.5 when I(2)2MG level was > 40 mg/dl and 1 when beta2MG level was < 30 mg/dl. CONCLUSION: These findings suggest that beta2MG is useful as a screening test for the onset of EPS, and that beta2MG and accumulation of middle-molecular uremic substances may be related to the pathophysiology of EPS.     

Bloody ascites in a patient after transfer from peritoneal dialysis to hemodialysis

A 65-year-old woman with end-stage renal disease (ESRD) presented with bloody ascites. She had been maintained on peritoneal dialysis (PD) for 7 years and had eight episodes of peritonitis. She was eventually transferred to hemodialysis (HD) because of ultrafiltration failure. This was associated with "high" peritoneal transport by peritoneal equilibration test (PET). A period of "peritoneal rest" did not improve PET results. Within a year of transfer to HD, ascites developed, which was hemorrhagic upon evaluation. A computed tomography (CT) scan suggested encapsulating sclerosing peritonitis, which was confirmed upon peritonoscopy. The patient was treated with prednisone and tamoxifen. Encapsulating peritoneal sclerosis (EPS) is a devastating complication of PD. Although it is rare and its development often unpredictable, this case demonstrates several clinical features commonly observed in the condition. These include more than 6 years on PD, a high transporter status, recurrent peritonitis, and the development of blood-stained dialysis effluent (or ascites if PD has been discontinued, as was the case in this patient). The initial presentation is often incipient with vague abdominal pain. Symptoms are progressive, however, and EPS has a high mortality rate, with most patients dying within 1 year of diagnosis, usually from malnutrition and sepsis. Treatment options include systemic immunosuppression and regular peritoneal irrigation after transfer to HD. Response to treatment is more likely to occur in the early inflammatory stage of EPS, when symptoms are nonspecific and imaging is relatively normal. Hence a high degree of suspicion for the diagnosis should be present in patients "at risk" of this condition, as early diagnosis is essential if progressive encapsulation of the abdominal viscera is to be prevented.