Encapsulating peritoneal sclerosis (EPS) is a rare but severe complication of peritoneal dialysis. A total of 50% of the patients died within 12 months after being diagnosed. There are no obvious clinical symptoms in the early stage of EPS, which is easy to be missed. And there are few case reports of EPS in early stage. On December 22, 2018, a 70-year-old male patient undergoing peritoneal dialysis for 17 months, who was diagnosed as EPS, was admitted to the Department of Nephrology, the Third Xiangya Hospital, Central South University. The patient's peritoneal dialysis catheter was obstructed after peritonitis. The peritoneal dialysis fluid couldn't be drain in and out of the abdominal cavity. Therefore, the laparoscopy was performed to repair the catheter. The operation in progress showed that the peritoneum was slightly thickened and the ileocecal intestinal tube was closely adhered to the parietal peritoneum where the catheter was wrapped, indicating the early stage of EPS. Peritoneal relaxation was performed. The patient's catheter was normal after adhesiolysis. He underwent hemodialysis, nutritional supporting as well as peritoneal dialysis transition, etc. The peritonitis was controlled after 10 days and the peritoneal dialysis was resumed. After discharge from hospital, the patient took moxifloxacin for 2 more weeks. We followed up the patient for 6 months. The automated peritoneal dialysis is maintained, and everything remains normal. Clinicians need to improve understanding of EPS. Early diagnosis and laparoscopic adhesiolysis is helpful to continue peritoneal dialysis treatment.
Aged ; Early Diagnosis ; Humans ; Male ; Peritoneal Dialysis/adverse effects* ; Peritoneal Fibrosis/pathology* ; Peritoneum ; Peritonitis/pathology* ; Sclerosis/pathology*

BACKGROUND: Encapsulating 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.METHODS: We 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.RESULTS: Thirteen 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.CONCLUSION: The high mortality rate of EPS suggests the importance of appropriate surgical intervention in young symptomatic male EPS patients with relatively low CT scan scores.
Adhesives ; Diagnosis ; Dilatation ; Female ; Fistula ; Humans ; Kidney Transplantation ; Korea ; Male ; Mortality ; Peritoneal Dialysis ; Peritoneal Fibrosis ; Reoperation ; Retrospective Studies ; Seoul ; Tomography, X-Ray Computed

BACKGROUND: We investigated the effects of tranilast on epithelial-to-mesenchymal transition (EMT) in an animal model and on the EMT signaling pathway in human peritoneal mesothelial cells (HPMCs).METHODS: We performed in vitro studies (cytotoxicity, cell morphology, and western blot analyses) on HPMCs from human omenta, along with in vivo studies (peritoneal membrane function and morphometric and immunohistochemical analyses) on Sprague Dawley rats. Thirty-two rats were divided into three groups: control (C) group (peritoneal dialysis [PD] catheter but not infused with dialysate), PD group (4.25% glucose-containing dialysate), and PD + tranilast group (4.25% glucose-containing dialysate along with tranilast).RESULTS: In in vitro experiments, transforming growth factor-beta 1 (TGF-β1) increased α-smooth muscle actin and Snail expression and reduced E-cadherin expression in HPMCs. TGF-β1 also reduced cell contact, induced a fibroblastoid morphology, and increased phosphorylation of Akt, Smad2, and Smad3 in HPMCs. Tranilast significantly inhibited TGF-β1-induced EMT and attenuated these morphological changes in HPMCs. In in vivo studies, after 6 weeks of experimental PD, the peritoneal membrane was significantly thicker in the PD group than in the C group. Tranilast protected against PD-induced glucose mass transfer change and histopathological changes in rats.CONCLUSION: Tranilast prevented EMT both in HPMCs triggered with TGF-β1 and in rats with PD-induced peritoneal fibrosis. Thus, tranilast may be considered a therapeutic intervention that enables long-term PD by regulating TGF-β1 signaling pathways.
Actins ; Animals ; Blotting, Western ; Cadherins ; Catheters ; Dialysis ; Epithelial-Mesenchymal Transition ; Fibrosis ; Glucose ; Humans ; In Vitro Techniques ; Membranes ; Models, Animal ; Peritoneal Dialysis ; Peritoneal Fibrosis ; Peritoneum ; Phosphorylation ; Rats ; Rats, Sprague-Dawley ; Snails

BACKGROUND: Peritoneal fibrosis is the primary reason that patients with end-stage renal disease (ESRD) have to cease peritoneal dialysis. Peritonitis caused by Gram-negative bacteria such as Escherichia coli (E. coli) were on the rise. We had previously shown that matrine inhibited the formation of biofilm by E. coli. However, the role of matrine on the epithelial-mesenchymal transition (EMT) in peritoneal mesothelial cells under chronic inflammatory conditions is still unknown. METHODS: We cultured human peritoneal mesothelial cells (HPMCs) with lipopolysaccharide (LPS) to induce an environment that mimicked peritonitis and investigated whether matrine could inhibit LPS-induced EMT in these cells. In addition, we investigated the change in expression levels of the miR-29b and miR-129-5p. RESULTS: We found that 10 μg/ml of LPS induced EMT in HPMCs. Matrine inhibited LPS-induced EMT in HPMCs in a dose-dependent manner. We observed that treatment with matrine increased the expression of E-cadherin (F = 50.993, P < 0.01), and decreased the expression of alpha-smooth muscle actin (F = 32.913, P < 0.01). Furthermore, we found that LPS reduced the expression levels of miR-29b and miR-129-5P in HPMCs, while matrine promoted the expression levels of miR-29b and miR-129-5P. CONCLUSIONS Matrine could inhibit LPS-induced EMT in HPMCs and reverse LPS inhibited expressions of miR-29 b and miR-129-5P in HPMCs, ultimately reduce peritoneal fibrosis. These findings provide a potential theoretical basis for using matrine in the prevention and treatment of peritoneal fibrosis.
Actins ; metabolism ; Alkaloids ; therapeutic use ; Cadherins ; metabolism ; Cells, Cultured ; Epithelial-Mesenchymal Transition ; drug effects ; Epithelium ; drug effects ; Fibrosis ; chemically induced ; genetics ; metabolism ; Humans ; Lipopolysaccharides ; toxicity ; MicroRNAs ; metabolism ; Peritoneal Fibrosis ; drug therapy ; Quinolizines ; therapeutic use

Sclerosing mesenteritis is a rare benign disease with a prevalence of 0.16–3.4% and is characterized by chronic nonspecific inflammation and extensive fibrosis in the adipose tissue of the mesentery although the exact pathogenesis is still elusive. A 65-year-old woman was referred with suspicion of an abdominal mass and biliary stones on abdominal ultrasonography and CT. Bile duct stones were confirmed by endoscopic ultrasonography and successfully treated by endoscopic retrograde cholangiography with stone removal. Furthermore, a 4.7 cm conglomerated mass on small intestinal mesentery was suspected as sclerosing mesenteritis based on the features on abdominal MRI. However, because it could not be differentiated from malignancy without histologic examination, laparoscopic excisional biopsy was performed; it showed only inflammatory cells with extensive fibrosis. Therefore, the abdominal mass was confirmed as sclerosing fibrosis and the patient was followed-up without any treatments because no mass-related symptoms accompanied the findings. Six months later, abdominal CT showed no significant change in the mass. Herein, we report a rare case of incidentally found idiopathic sclerosing mesenteritis.
Adipose Tissue ; Aged ; Bile Ducts ; Biopsy ; Cholangiography ; Endosonography ; Female ; Fibrosis ; Humans ; Immunoglobulins* ; Inflammation ; Magnetic Resonance Imaging ; Mesentery ; Panniculitis, Peritoneal ; Prevalence ; Sclerosis* ; Tomography, X-Ray Computed ; Ultrasonography

Encapsulating peritoneal sclerosis (EPS) is a rare cause of intestinal obstruction by a thick fibrous membrane wrapping around the small intestine. It is a possible complication after liver transplantation (LT) that can be fatal. This report describes 2 cases of EPS after LT that were successfully treated with surgery, corticosteroids, tamoxifen, and mammalian target of rapamycin inhibitor. After treatment in both cases, the patients were able to start oral feeding and have been symptom free for more than 1 year. These cases suggests that for the management of EPS, surgical treatment is mandatory when the patients present with symptoms of intestinal obstruction or if there are findings suggestive of decreased mural perfusion. Surgery should be accompanied with medical treatment to prevent the relapse of EPS.
Adrenal Cortex Hormones ; Humans ; Intestinal Obstruction ; Intestine, Small ; Liver Transplantation ; Liver* ; Membranes ; Perfusion ; Peritoneal Fibrosis* ; Recurrence ; Sirolimus ; Tamoxifen ; Transplant Recipients*

To investigate the role of glucose transporter 1 (GLUT1) and sodium-glucose cotransporter 1 (SGLT1) in high glucose dialysate-induced peritoneal fibrosis.Thirty six male SD rats were randomly divided into 6 groups (6 in each):normal control group, sham operation group, peritoneal dialysis group (PD group), PD+phloretin group (PD+T group), PD+phlorizin group (PD+Z group), PD+phloretin+phlorizin group (PD+T+Z group). Rat model of uraemia was established using 5/6 nephrotomy, and 2.5% dextrose peritoneal dialysis solution was used in peritoneal dialysis. Peritoneal equilibration test was performed 24 h after dialysis to evaluate transport function of peritoneum in rats; HE staining was used to observe the morphology of peritoneal tissue; and immunohistochemistry was used to detect the expression of GLUT1, SGLT1, TGF-β1 and connective tissue growth factor (CTGF) in peritoneum. Human peritoneal microvascular endothelial cells (HPECs) were divided into 5 groups:normal control group, peritoneal dialysis group (PD group), PD+phloretin group (PD+T group), PD+phlorezin group (PD+Z group), and PD+phloretin+phlorezin group (PD+T+Z group). Real time PCR and Western blotting were used to detect mRNA and protein expressions of GLUT1, SGLT1, TGF-β1, CTGF in peritoneal membrane and HPECs., compared with sham operation group, rats in PD group had thickened peritoneum, higher ultrafiltration volume, and the mRNA and protein expressions of GLUT1, SGLT1, CTGF, TGF-β1 were significantly increased (all<0.05); compared with PD group, thickened peritoneum was attenuated, and the mRNA and protein expressions of GLUT1, SGLT1, CTGF, TGF-β1 were significantly decreased in PD+T, PD+Z and PD+T+Z groups (all<0.05). Pearson's correlation analysis showed that the expressions of GLUT1, SGLT1 in peritoneum were positively correlated with the expressions of TGF-β1 and CTGF (all<0.05)., the mRNA and protein expressions of GLUT1, SGLT1, TGF-β1, CTGF were significantly increased in HPECs of peritoneal dialysis group (all<0.05), and those in PD+T, PD+Z, and PD+T+Z groups were decreased (all<0.05). Pearson's correlation analysis showed that the expressions of GLUT1, SGLT1 in HPECs were positively correlated with the expressions of TGF-β1 and CTGF (all<0.05).High glucose peritoneal dialysis fluid may promote peritoneal fibrosis by upregulating the expressions of GLUT1 and SGLT1.
Animals ; Cells, Cultured ; Connective Tissue Growth Factor ; analysis ; drug effects ; Dialysis Solutions ; adverse effects ; chemistry ; pharmacology ; Gene Expression Regulation ; drug effects ; Glucose ; adverse effects ; pharmacology ; Glucose Transporter Type 1 ; analysis ; drug effects ; physiology ; Hemodiafiltration ; adverse effects ; methods ; Humans ; Male ; Peritoneal Dialysis ; adverse effects ; methods ; Peritoneal Fibrosis ; chemically induced ; genetics ; physiopathology ; Peritoneum ; chemistry ; drug effects ; pathology ; Phloretin ; Phlorhizin ; RNA, Messenger ; Rats ; Rats, Sprague-Dawley ; Sodium-Glucose Transporter 1 ; analysis ; drug effects ; physiology ; Transforming Growth Factor beta1 ; analysis ; drug effects ; Uremia ; chemically induced

Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is characterized by a thick fibrotic membrane encasing the small intestine like a cocoon. Accurate preoperative diagnosis is often difficult. We present 2 cases of SEP that were diagnosed preoperatively by contrast-enhanced computed tomography scan. A 38-year-old man and a 56-year-old woman were admitted to Daegu Catholic University Medical Center because of recurrent intestinal obstruction. We performed exploratory laparotomy with doubt of the preoperative diagnosis of SEP. We confirmed the diagnosis of SEP on laparotomy and performed adhesiolysis. Both patients recovered successfully and had no signs of recurrence. A better awareness of SEP and its radiological features should lead to more correct preoperative diagnosis and result in more appropriate management, including surgery.
Academic Medical Centers ; Adult ; Daegu ; Diagnosis ; Female ; Humans ; Intestinal Obstruction* ; Intestine, Small ; Laparotomy ; Membranes ; Middle Aged ; Peritoneal Fibrosis ; Peritonitis* ; Recurrence

Peritoneal fibrosis is one of the major complications occurring in long-term peritoneal dialysis patients as a result of injury. Peritoneal fibrosis is characterized by submesothelial thickening and fibrosis which is associated with a decline in peritoneal membrane function. The myofibroblast has been identified as the key player involved in the development and progression of peritoneal fibrosis. Activation of the myofibroblast is correlated with expansion of the extracellular matrix and changes in peritoneal membrane integrity. Over the years, epithelial to mesenchymal transition (EMT) has been accepted as the predominant source of the myofibroblast. Peritoneal mesothelial cells have been described to undergo EMT in response to injury. Several animal and in vitro studies support the role of EMT in peritoneal fibrosis; however, emerging evidence from genetic fate-mapping studies has demonstrated that myofibroblasts may be arising from resident fibroblasts and pericytes/perivascular fibroblasts. In this review, we will discuss hypotheses currently surrounding the origin of the myofibroblast and highlight the experimental systems predominantly being used to investigate this.
Animals ; Extracellular Matrix ; Fibroblasts ; Fibrosis ; Humans ; In Vitro Techniques ; Membranes ; Myofibroblasts* ; Peritoneal Dialysis ; Peritoneal Fibrosis*

Pediatric mesenteric panniculitis is an extremely rare disease of unknown etiology characterized by chronic inflammation, fat necrosis, and fibrosis in the mesenteric adipose tissue. A previously healthy 13-year-old boy was admitted because of right upper abdominal pain. An abdominal computed tomography scan revealed increased attenuation and enhancement in the left upper abdominal omental fat and anterior peritoneal wall thickening. A laparoscopic biopsy showed mesenteric panniculitis with chronic inflammation, adiponecrosis, and septal fibrosis. Serological tests for autoimmune diseases, nested polymerase chain reaction for Mycobacterium tuberculosis, and special immunohistochemical stains for malignancy were all negative. Symptomatic improvement and improved abnormal findings were achieved after an 8-month treatment with prednisolone according to a follow-up abdominal computed tomography scan. Here, we report a case of pediatric mesenteric panniculitis treated with prednisolone.
Abdominal Pain ; Adipose Tissue ; Adolescent ; Autoimmune Diseases ; Biopsy ; Child ; Coloring Agents ; Fat Necrosis ; Fibrosis ; Follow-Up Studies ; Humans ; Inflammation ; Male* ; Mycobacterium tuberculosis ; Panniculitis, Peritoneal* ; Polymerase Chain Reaction ; Prednisolone* ; Rare Diseases ; Serologic Tests