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van Boven E, Coebergh van den Braak RRJ, van Laar J, Betjes MGH. Unexpected finding of encapsulating peritoneal sclerosis: mind the cocoon. BMJ Case Rep 2025; 18:e264334. [PMID: 39870474 DOI: 10.1136/bcr-2024-264334] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/29/2025] Open
Abstract
Here, we present a fatal case of a man in his 40s with encapsulating peritoneal sclerosis (EPS). In retrospect, a spot diagnosis on the abdominal CT scan. The patient presented with progressive abdominal complaints of pain and vomiting over the last 2 months. He had a history of therapy-refractory sarcoidosis. A CT scan was performed, and loculated ascites was noted. The patient was admitted with suspected spontaneous peritoneal peritonitis but did not improve on antibiotics. Data from the microbiology, pathology and radiology investigations lead to no diagnosis. Eventually, laparoscopy revealed the diagnosis of EPS. Treatment with tamoxifen to counteract fibrosis and a pulse dose of methylprednisolone was started. Unfortunately, the patient developed a bowel perforation and died. In retrospect, the radiological sign of a cocoon encasing the small bowels should have been a clue to an early diagnosis.
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Affiliation(s)
- Eva van Boven
- Erasmus MC Universitair Medisch Centrum Rotterdam, Rotterdam, Zuid-Holland, The Netherlands
| | | | - Jan van Laar
- Clinical Immunology, Internal Medicine, Erasmus MC, Rotterdam, The Netherlands
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Neirouz K, Haifa BR, Mehdi TM, Faicel BS, Mohamed BH, Hichem J. Idiopathic encapsulating peritoneal sclerosis: A case report. Int J Surg Case Rep 2025; 126:110694. [PMID: 39631118 PMCID: PMC11652888 DOI: 10.1016/j.ijscr.2024.110694] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2024] [Revised: 11/25/2024] [Accepted: 11/28/2024] [Indexed: 12/07/2024] Open
Abstract
INTRODUCTION Encapsulating peritoneal sclerosis is a rare disease, characterized by subocclusive symptoms. Idiopathic forms could be challenging, as they are often mistaken for other conditions. We present a case mistaken for an internal hernia. CASE REPORT A 52-year-old male patient, who was complaining of abdominal pain and bloating for 6 months. He presented due to worsening of his symptoms. A CT scan was then performed evoking an internal hernia. Emergency surgery via laparoscopy revealed encapsulating peritoneal sclerosis with a cocoon-like fibrous tissue encasing the bowel. A conversion by a midline incision was performed for enterolysis and fibrous tissue resection. DISCUSSION Encapsulating peritoneal sclerosis (EPS), is a rare disease. Because of the rigid capsule surrounding the loops, symptomatology includes recurrent occlusive symptoms. The incidence of this pathology is unknown. EPS is often associated with peritoneal dialysis(PD), infections, and systemic inflammatory disorders. Idiopathic cases, like in our patient, are often misdiagnosed pre-operatively. The treatment focuses on the management of the underlying cause if identified. Corticoids are proposed in inflammatory diseases while switching to hemodialysis or the use of tamoxifen and immunosuppressive therapies are proposed in case of PD. In case of failure of the conservative approaches or in case of emergency, a peritonectomy and enterolysis could be performed despite being at high risk of recurrence and mortality. CONCLUSION We aimed to describe a case misdiagnosed as an internal hernia to raise awareness among practitioners about this condition.
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Affiliation(s)
- Kammoun Neirouz
- B Surgery Department, Charles Nicolle Hospital, Tunis, Tunisia.
| | | | | | | | | | - Jerraya Hichem
- B Surgery Department, Charles Nicolle Hospital, Tunis, Tunisia
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Srisajjakul S, Prapaisilp P, Bangchokdee S. Imaging pearls and differential diagnosis of encapsulating peritoneal sclerosis: Emphasis on computed tomography. Clin Imaging 2023; 94:116-124. [PMID: 36527797 DOI: 10.1016/j.clinimag.2022.12.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Revised: 11/20/2022] [Accepted: 12/05/2022] [Indexed: 12/14/2022]
Abstract
Encapsulating peritoneal sclerosis (EPS) is a severe peritoneal fibrotic reaction most frequently identified as a complication of peritoneal dialysis. EPS is a complex condition whose management requires multidisciplinary input from radiologists, gastroenterologists, nephrologists, surgeons, and dietitians. EPS carries significant morbidity and mortality, primarily due to bowel obstruction that results in intestinal failure, malnutrition, and sepsis. The nondialysis causes of EPS include tuberculous peritonitis, prior abdominal surgery, beta-blocker medication use, and endometriosis. The clinical symptoms of EPS are nausea, vomiting, and abdominal pain, all of which appear to be associated with bowel obstruction. The diagnosis of EPS needs three pillars to be met: clinical features, radiological evaluation, and histopathological analysis. The disease is frequently progressive and can be fatal. Computed tomography is the gold standard imaging modality for the detection of peritoneal abnormalities and encapsulation of bowel loops by thick adhesions or fibrosis (cocooning). Computed tomography also aids in making a differential diagnosis. Unfortunately, the diagnosis of EPS is often delayed because clinical findings are not specific and may resemble other peritoneal diseases. Radiologists should be familiar with the clinical impacts and related imaging features of EPS and realize when to seek them to facilitate timely and proper treatment.
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Affiliation(s)
- Sitthipong Srisajjakul
- Department of Radiology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
| | - Patcharin Prapaisilp
- Department of Radiology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
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Deng P, Xiong LX, He P, Hu JH, Zou QX, Le SL, Wen SL. Surgical timing for primary encapsulating peritoneal sclerosis: A case report and review of literature. World J Gastrointest Surg 2022; 14:352-361. [PMID: 35664367 PMCID: PMC9131833 DOI: 10.4240/wjgs.v14.i4.352] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2021] [Revised: 01/06/2022] [Accepted: 03/26/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Primary encapsulating peritoneal sclerosis (EPS) is a rare but devastating disease that causes fibrocollagenous cocoon-like encapsulation of the bowel, resulting in bowel obstruction. The pathogenesis, prevention, and treatment strategies of EPS remain unclear so far. Since most patients are diagnosed during exploratory laparotomy, for the non-surgically diagnosed patients with primary EPS, the surgical timing is also uncertain. CASE SUMMARY A 44-year-old female patient was referred to our center on September 6, 2021, with complaints of abdominal distention and bilious vomiting for 2 d. Physical examination revealed that the vital signs were stable, and the abdomen was slightly distended. Computerized tomography scan showed a conglomerate of multiple intestinal loops encapsulated in a thick sac-like membrane, which was surrounded by abdominal ascites. The patient was diagnosed with idiopathic EPS. Recovery was observed after abdominal paracentesis, and the patient was discharged on September 13 after the resumption of a normal diet. This case raised a question: When should an exploratory laparotomy be performed on patients who are non-surgically diagnosed with EPS. As a result, we conducted a review of the literature on the clinical manifestations, intraoperative findings, surgical methods, and therapeutic effects of EPS. CONCLUSION Recurrent intestinal obstructions and abdominal mass combined with the imaging of encapsulated bowel are helpful in diagnosing idiopathic EPS. Small intestinal resection should be avoided.
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Affiliation(s)
- Peng Deng
- Department of General Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
| | - Long-Xin Xiong
- Department of General Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
| | - Ping He
- Department of General Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
| | - Jian-Hua Hu
- Department of Emergency Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
| | - Qi-Xu Zou
- Department of General Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
| | - Shi-Lian Le
- Department of General Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
| | - Sen-Lin Wen
- Department of General Surgery, The First Hospital of Nanchang, Nanchang 330008, Jiangxi Province, China
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Hamada C, Tomino Y. Recent Understanding of Peritoneal Pathology in Peritoneal Dialysis Patients in Japan. Blood Purif 2021; 50:719-728. [PMID: 33567422 DOI: 10.1159/000510282] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2020] [Accepted: 07/09/2020] [Indexed: 11/19/2022]
Abstract
The thin peritoneum covering the peritoneal cavity has been used as a dialysis membrane for peritoneal dialysis (PD) because it is highly vascularized and has a large body surface area. However, it has been reported that peritoneal membranes affected by peritonitis, as well as those exposed to the nonphysiological high glucose levels containing PD dialysate, may undergo histological and functional changes. Patients undergoing PD may experience encapsulating peritoneal sclerosis (EPS), which is a life-threatening serious complication of PD that can significantly impair activities of daily living. The incidence of EPS was 1.4-7.3% of maintenance PD patients in the 1980s. The incidence has improved to 1.0% after a neutral dialysate became the standard PD treatment in Japan. Furthermore, the pathogenesis of EPS is uncertain although its onset may be explained by the "two-hit theory," in which some factors leading to impairment had an additive effect on simple peritoneal sclerosis. The evaluation of histopathological findings has shown the impact of the neutral dialysate on peritoneal deterioration as well as its role in the development of functional changes. In the present report, we discuss the advances in the understanding of peritoneal deterioration based on histological and macroscopic evaluations of the peritoneum of patients undergoing PD. We also discuss the recent treatment for PD patients.
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Affiliation(s)
- Chieko Hamada
- Advanced Research Institute for Health Science, Faculty of Medicine, Juntendo University, Tokyo, Japan,
| | - Yasuhiko Tomino
- Asian Pacific Renal Research Promotion Office, Medical Corporation SHOWAKAI, Tokyo, Japan
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Zvizdic Z, Summers A, Moinuddin Z, Van Dellen D, Pasic-Sefic I, Skenderi F, Vranic S, Augustine T. A Successful Treatment of Encapsulating Peritoneal Sclerosis in an Adolescent Boy on Long-term Peritoneal Dialysis: A Case Report. Prague Med Rep 2021; 121:254-261. [PMID: 33270013 DOI: 10.14712/23362936.2020.22] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022] Open
Abstract
Encapsulating peritoneal sclerosis (EPS) is a rare life-threatening complication associated with peritoneal dialysis (PD). EPS is characterized by progressive fibrosis and sclerosis of the peritoneum, with the formation of a membrane and tethering of loops of the small intestine resulting in intestinal obstruction. It is very rare in children. We present a case of a 16-year-old adolescent boy who developed EPS seven years after being placed on continuous ambulatory peritoneal dialysis (CAPD) complicated by several episodes of bacterial peritonitis. The diagnosis was based on clinical, radiological, intraoperative and histopathological findings. The patient was successfully treated with surgical enterolysis. During a 7-year follow-up, there have been no further episodes of small bowel obstruction documented. He still continues to be on regular hemodialysis and is awaiting a deceased donor kidney transplant. EPS is a long-term complication of peritoneal dialysis and is typically seen in adults. Rare cases may be seen in the pediatric population and require an appropriate surgical approach that is effective and lifesaving for these patients.
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Affiliation(s)
- Zlatan Zvizdic
- Department of Pediatric Surgery, University Clinical Center Sarajevo, Sarajevo, Bosnia and Herzegovina
| | - Angela Summers
- Department of Transplant and Endocrine Surgery, Manchester Royal Infirmary, Manchester University Foundation Trust; An United Kingdom National Specialized Centre for Surgery for Encapsulating Peritoneal Sclerosis, Manchester, United Kingdom
| | - Zia Moinuddin
- Department of Transplant and Endocrine Surgery, Manchester Royal Infirmary, Manchester University Foundation Trust; An United Kingdom National Specialized Centre for Surgery for Encapsulating Peritoneal Sclerosis, Manchester, United Kingdom
| | - David Van Dellen
- Department of Transplant and Endocrine Surgery, Manchester Royal Infirmary, Manchester University Foundation Trust; An United Kingdom National Specialized Centre for Surgery for Encapsulating Peritoneal Sclerosis, Manchester, United Kingdom
| | - Irmina Pasic-Sefic
- Department of Radiology, University Clinical Center Sarajevo, Sarajevo, Bosnia and Herzegovina
| | - Faruk Skenderi
- Department of Pathology, University Clinical Center Sarajevo, Sarajevo, Bosnia and Herzegovina
| | - Semir Vranic
- College of Medicine, QU Health, Qatar University, Qatar.
| | - Titus Augustine
- Department of Transplant and Endocrine Surgery, Manchester Royal Infirmary, Manchester University Foundation Trust; An United Kingdom National Specialized Centre for Surgery for Encapsulating Peritoneal Sclerosis, Manchester, United Kingdom
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Ryu JH, Lee KY, Koo TY, Kim DK, Oh KH, Yang J, Park KJ. Outcomes of the surgical management of encapsulating peritoneal sclerosis: A case series from a single center in Korea. Kidney Res Clin Pract 2019; 38:499-508. [PMID: 31640301 PMCID: PMC6913596 DOI: 10.23876/j.krcp.19.044] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2019] [Revised: 08/21/2019] [Accepted: 09/04/2019] [Indexed: 12/17/2022] Open
Abstract
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.
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Affiliation(s)
- Jung-Hwa Ryu
- Transplantation Center, Seoul National University Hospital, Seoul, Republic of Korea
| | - Kil-Yong Lee
- Department of Surgery, Seoul National University Hospital, Seoul, Republic of Korea
| | - Tai Yeon Koo
- Transplantation Center, Seoul National University Hospital, Seoul, Republic of Korea
| | - Dong Ki Kim
- Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Kook-Hwan Oh
- Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Jaeseok Yang
- Transplantation Center, Seoul National University Hospital, Seoul, Republic of Korea.,Department of Surgery, Seoul National University Hospital, Seoul, Republic of Korea
| | - Kyu Joo Park
- Department of Surgery, Seoul National University Hospital, Seoul, Republic of Korea
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Shimomura Y, Sakai S, Ueda H, Fujikura K, Imai Y, Ishikawa T. Encapsulating peritoneal sclerosis in a patient after allogeneic hematopoietic stem cell transplantation: a case report. BMC Gastroenterol 2019; 19:12. [PMID: 30665354 PMCID: PMC6341688 DOI: 10.1186/s12876-019-0933-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/12/2017] [Accepted: 01/16/2019] [Indexed: 11/21/2022] Open
Abstract
BACKGROUND Encapsulating peritoneal sclerosis (EPS) is a chronic clinical syndrome of acute or subacute gastrointestinal obstruction seen mainly in patients undergoing peritoneal dialysis. Although there are a few reports on EPS developing in non-peritoneal dialysis patients, it has not been reported in patients undergoing allogeneic haematopoietic stem cell transplantation (HSCT). Here, we report a case of EPS after a second HSCT. CASE PRESENTATION A 46-year-old man with myelodysplastic syndrome showed relapse after HSCT and received a second HSCT. The patient was diagnosed with chronic graft-versus-host disease (cGVHD)-associated serositis because of persistent ascites. His ascites improved gradually and disappeared without immunosuppressive therapy. He presented with nausea, weight loss, and constipation 1 year after improvement of ascites. Computed tomography revealed no organic obstruction, but did reveal dilated, thickened, and adhered small bowel loops with a mass-like appearance. He was diagnosed with EPS on the basis of clinical symptoms and image findings. He received corticosteroid therapy (20 mg/body) without any improvement in symptoms. He developed recurrence of myelodysplastic syndrome at 1 month after initiation of corticosteroid therapy. This progressed into acute myeloid leukaemia after 3 months. He died 31 months after the second HSCT. At autopsy, the small and large intestines had formed extensive adhesions and showed signs of progressive fibrosis with peritoneal sclerosis, fibroblast swelling, fibrin deposition, and inflammatory cell infiltration, which confirmed the diagnosis of EPS. CONCLUSION This case suggests that EPS may complicate patients with cGVHD-associated serositis. Although the mechanism of EPS development is not clear, clinicians should be aware of this eventuality.
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Affiliation(s)
- Yoshimitsu Shimomura
- Department of Hematology, Kobe City Hospital Organization, Kobe City Medical Center General Hospital, 2-1-1 Minatojima-Minamimachi, Chuo-ku, Kobe, 650-0047 Japan
| | - Shinsuke Sakai
- Department of Nephrology, Osaka University Graduate School of Medicine, Osaka, Japan
| | - Hiroyuki Ueda
- Department of Diagnostic Radiology, Kobe City Hospital Organization, Kobe City Medical Center General Hospital, Kobe, Japan
| | - Kohei Fujikura
- Department of Clinical Pathology, Kobe City Hospital Organization, Kobe City Medical Center General Hospital, Kobe, Japan
| | - Yukihiro Imai
- Department of Clinical Pathology, Kobe City Hospital Organization, Kobe City Medical Center General Hospital, Kobe, Japan
| | - Takayuki Ishikawa
- Department of Hematology, Kobe City Hospital Organization, Kobe City Medical Center General Hospital, 2-1-1 Minatojima-Minamimachi, Chuo-ku, Kobe, 650-0047 Japan
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Danford CJ, Lin SC, Smith MP, Wolf JL. Encapsulating peritoneal sclerosis. World J Gastroenterol 2018; 24:3101-3111. [PMID: 30065556 PMCID: PMC6064970 DOI: 10.3748/wjg.v24.i28.3101] [Citation(s) in RCA: 88] [Impact Index Per Article: 12.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/16/2018] [Revised: 06/07/2018] [Accepted: 06/25/2018] [Indexed: 02/06/2023] Open
Abstract
Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents (i.e. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPS-specific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is time-consuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease.
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Affiliation(s)
- Christopher J Danford
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
| | - Steven C Lin
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
| | - Martin P Smith
- Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
| | - Jacqueline L Wolf
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
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Brown EA, Bargman J, van Biesen W, Chang MY, Finkelstein FO, Hurst H, Johnson DW, Kawanishi H, Lambie M, de Moraes TP, Morelle J, Woodrow G. Length of Time on Peritoneal Dialysis and Encapsulating Peritoneal Sclerosis - Position Paper for ISPD: 2017 Update. Perit Dial Int 2018; 37:362-374. [PMID: 28676507 DOI: 10.3747/pdi.2017.00018] [Citation(s) in RCA: 95] [Impact Index Per Article: 13.6] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2017] [Accepted: 04/04/2017] [Indexed: 12/11/2022] Open
Affiliation(s)
- Edwina A Brown
- Imperial College Renal and Transplant Centre, Hammersmith Hospital, London, UK
| | - Joanne Bargman
- University Health Network and the University of Toronto, Toronto, ON, Canada
| | - Wim van Biesen
- Renal Division, Ghent University Hospital, Ghent, Belgium
| | - Ming-Yang Chang
- Kidney Research Center, Department of Nephrology, Chang Gung Memorial Hospital, Taoyuan, Taiwan
| | | | - Helen Hurst
- Central Manchester and Manchester Children's NHS Foundation Trust, Manchester, UK
| | - David W Johnson
- Department of Nephrology, University of Queensland at Princess Alexandra Hospital, Brisbane, Australia
| | - Hideki Kawanishi
- Tsuchiya General Hospital, Faculty of Medicine, Hiroshima University, Japan
| | - Mark Lambie
- Institute for Applied Clinical Sciences, Keele University, Stoke-on-Trent, UK
| | | | - Johann Morelle
- Division of Nephrology, Cliniques universitaires Saint-Luc, Brussels, Belgium, et Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
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Wilson RB. Hypoxia, cytokines and stromal recruitment: parallels between pathophysiology of encapsulating peritoneal sclerosis, endometriosis and peritoneal metastasis. Pleura Peritoneum 2018; 3:20180103. [PMID: 30911653 PMCID: PMC6405013 DOI: 10.1515/pp-2018-0103] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2018] [Accepted: 03/01/2018] [Indexed: 02/06/2023] Open
Abstract
Peritoneal response to various kinds of injury involves loss of peritoneal mesothelial cells (PMC), danger signalling, epithelial-mesenchymal transition and mesothelial-mesenchymal transition (MMT). Encapsulating peritoneal sclerosis (EPS), endometriosis (EM) and peritoneal metastasis (PM) are all characterized by hypoxia and formation of a vascularized connective tissue stroma mediated by vascular endothelial growth factor (VEGF). Transforming growth factor-β1 (TGF-β1) is constitutively expressed by the PMC and plays a major role in the maintenance of a transformed, inflammatory micro-environment in PM, but also in EPS and EM. Persistently high levels of TGF-β1 or stimulation by inflammatory cytokines (interleukin-6 (IL-6)) induce peritoneal MMT, adhesion formation and fibrosis. TGF-β1 enhances hypoxia inducible factor-1α expression, which drives cell growth, extracellular matrix production and cell migration. Disruption of the peritoneal glycocalyx and exposure of the basement membrane release low molecular weight hyaluronan, which initiates a cascade of pro-inflammatory mediators, including peritoneal cytokines (TNF-α, IL-1, IL-6, prostaglandins), growth factors (TGF-α, TGF-β, platelet-derived growth factor, VEGF, epidermal growth factor) and the fibrin/coagulation cascade (thrombin, Tissue factor, plasminogen activator inhibitor [PAI]-1/2). Chronic inflammation and cellular transformation are mediated by damage-associated molecular patterns, pattern recognition receptors, AGE-RAGE, extracellular lactate, pro-inflammatory cytokines, reactive oxygen species, increased glycolysis, metabolomic reprogramming and cancer-associated fibroblasts. The pathogenesis of EPS, EM and PM shows similarities to the cellular transformation and stromal recruitment of wound healing.
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Affiliation(s)
- Robert Beaumont Wilson
- Upper GI Surgery Department, Liverpool Hospital, Elizabeth St, Liverpool, 2170, NSW, Australia
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12
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Abstract
BACKGROUND 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. METHODS 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. RESULTS 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. CONCLUSIONS 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.
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Allam H, Al Yahri O, Mathew S, Darweesh A, Suliman AN, Abdelaziem S, Khairat M, Toro A, Di Carlo I. The enigma of primary and secondary encapsulating peritoneal sclerosis. BMC Surg 2016; 16:81. [PMID: 27964725 PMCID: PMC5153754 DOI: 10.1186/s12893-016-0198-2] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/13/2016] [Accepted: 11/30/2016] [Indexed: 12/21/2022] Open
Abstract
BACKGROUND Encapsulating Peritoneal Sclerosis (EPS) describes a variety of diseases that are frequently confused with different names and different etiopathogeneses. The aim of this article is to report personal experience of focusing on correct classification and the status of current diagnosis and treatment. METHODS A retrospective analysis was performed. Age, sex, ethnic origin, past medical history, symptoms and their duration, radiological tools and signs, laboratory tests, preoperative diagnosis, surgical approach, intraoperative findings, pathological findings, hospital stay, morbidity and mortality were studied. RESULTS A total of seven patients, including six males and one female, aged from 24 to 72 years were observed. Four patients had recurrent abdominal colic pain for 3 months, 1, 2 and 9 years; two patients also reported recurrent attacks but without any specification of the duration. All seven patients presented at the emergency department with abdominal pain that was mainly diffused over the entire abdomen. Six patients were submitted to a CT scan. Only in two patients was the diagnosis of EPS made preoperatively. All seven patients were submitted to open surgery. The hospital stay was between 4 and 60 days. One patient had morbidity, and one patient died of MOF. CONCLUSIONS Currently, the correct identification of EPS is more easily possible than in the past, but the diagnosis is still a challenge. Surgery must be performed as soon as possible to avoid a poorer quality of life.
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Affiliation(s)
- Hisham Allam
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
| | - Omer Al Yahri
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
| | - Sharon Mathew
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
| | - Adham Darweesh
- Department of Radiology, Hamad General Hospital, Doha, Qatar
| | - Ahmed Nafea Suliman
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
| | - Sherif Abdelaziem
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
| | - Mohamed Khairat
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
| | - Adriana Toro
- Department of General Surgery, Barone I. Romeo Hospital, Patti, Messina Italy
| | - Isidoro Di Carlo
- Departments of General Surgery, Hamad General Hospital, Al Rayyan Road, 3050 Doha, Qatar
- Department of Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, Catania, Italy
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14
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Oguz EG, Okyay GU, Merhametsiz O, Ersoz S, Canbakan B, Ayli D. Long-Term Success with Adhesiolysis in Post-Transplant Encapsulating Peritoneal Sclerosis: A Retrospective Case Series of 4 Patients and Review of the Literature. Intern Med 2016; 55:269-72. [PMID: 26831022 DOI: 10.2169/internalmedicine.55.5461] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
Encapsulating peritoneal sclerosis (EPS) is an occasional and serious complication for peritoneal dialysis (PD) patients for whom no evidence-based management strategies have yet been established. Encapsulating peritoneal sclerosis could appear after kidney transplantation in patients who previously underwent long-term PD. In this report, we present our experience in four PD patients diagnosed with EPS after kidney transplantation. Adhesiolysis provided improvement in their acute clinical conditions and allograft functions, despite the long-term follow-up. Surgical intervention may be a safe modality for this specific group of patients.
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Affiliation(s)
- Ebru Gok Oguz
- Department of Nephrology, Diskapi Yildirim Beyazit Education And Research Hospital, Turkey
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15
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Kitterer D, Segerer S, Steurer W, Dippon J, Geissler A, Ulmer C, Braun N, Alscher MD, Latus J. C-reactive protein levels in combination with abdominal CT scans is a useful tool to predict the macroscopic appearance in late-stage EPS patients prior to surgery. Int J Nephrol Renovasc Dis 2015; 8:83-90. [PMID: 26316796 PMCID: PMC4540114 DOI: 10.2147/ijnrd.s84910] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
Background Diagnosis of encapsulating peritoneal sclerosis (EPS) is based on clinical symptoms, radiologic findings, and macroscopic or histological criteria. Two diagnostic scores for radiologic findings in computed tomography (CT) scans of patients with EPS have been established in the past (by Tarzi et al and Vlijm et al). The macroscopic appearance of EPS has previously been separated into three types. The use of CT scan as a tool to predict different macroscopic phenotypes, leading to specific surgical techniques and different medical treatment, has not yet been investigated. Methods We retrospectively analyzed 30 patients with late-stage EPS who underwent major surgery with peritonectomy and enterolysis. The preoperative CT scans were scored according to the two aforementioned established diagnostic CT scores. The macroscopic phenotype, surgical procedure, and laboratory values at the time of surgery were evaluated. CT findings in the different macroscopic phenotypes were analyzed. Results All patients had highly predictive CT scores for EPS. The macroscopic Type III had significantly higher CT scores compared with the other macroscopic phenotypes. Patients with macroscopic Type I had significantly higher C-reactive protein values compared to EPS Type III. Operation time was significantly longer, and repeated surgery and intraoperative complications were more frequent in EPS Type I compared with EPS Type III (P<0.05). Using the CT score and CRP level, the sensitivities for prediction of EPS I and III were 78% and 87% with corresponding specificities of 67% and 93%. Conclusion Abdominal CT scans might help to identify patients with a higher risk of complications and provide important information for the surgical intervention prior to surgery.
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Affiliation(s)
- Daniel Kitterer
- Department of Internal Medicine, Division of Nephrology, Robert-Bosch-Hospital, Stuttgart, Germany
| | - Stephan Segerer
- Division of Nephrology, University Hospital, Zurich, Switzerland
| | - Wolfgang Steurer
- Department of General, Visceral and Trauma Surgery, Robert-Bosch-Hospital, University of Stuttgart, Stuttgart, Germany
| | - Juergen Dippon
- Department of Mathematics, University of Stuttgart, Stuttgart, Germany
| | - Angela Geissler
- Department of Radiology and Nuclear Medicine, Robert-Bosch-Hospital, Stuttgart, Germany
| | - Christoph Ulmer
- Department of General, Visceral and Trauma Surgery, Robert-Bosch-Hospital, University of Stuttgart, Stuttgart, Germany
| | - Niko Braun
- Department of Internal Medicine, Division of Nephrology, Robert-Bosch-Hospital, Stuttgart, Germany
| | - Mark Dominik Alscher
- Department of Internal Medicine, Division of Nephrology, Robert-Bosch-Hospital, Stuttgart, Germany
| | - Joerg Latus
- Department of Internal Medicine, Division of Nephrology, Robert-Bosch-Hospital, Stuttgart, Germany
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16
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Messina M, Ariaudo C, Mella A, Cantaluppi V, Segoloni GP, Biancone L. mTOR inhibitors for medical treatment of post-transplantation encapsulating peritoneal sclerosis: a favourable single center experience. J Nephrol 2015; 28:245-9. [PMID: 25585825 DOI: 10.1007/s40620-014-0168-7] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2014] [Accepted: 12/09/2014] [Indexed: 01/01/2023]
Abstract
BACKGROUND 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. METHODS We performed a retrospective analysis on 1,048 kidney transplanted patients at our center between 11/2001 and 12/2011. RESULTS 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. CONCLUSION 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.
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Affiliation(s)
- Maria Messina
- Renal Transplantation Unit "A. Vercellone", Division of Nephrology Dialysis and Transplantation, Department of Medical Sciences, Città della Salute e della Scienza Hospital and University of Torino, Corso Bramante, 88-10126, Turin, Italy,
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17
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Moinuddin Z, Summers A, Van Dellen D, Augustine T, Herrick SE. Encapsulating peritoneal sclerosis-a rare but devastating peritoneal disease. Front Physiol 2015; 5:470. [PMID: 25601836 PMCID: PMC4283512 DOI: 10.3389/fphys.2014.00470] [Citation(s) in RCA: 35] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2014] [Accepted: 11/16/2014] [Indexed: 01/08/2023] Open
Abstract
Encapsulating peritoneal sclerosis (EPS) is a devastating but, fortunately, rare complication of long-term peritoneal dialysis. The disease is associated with extensive thickening and fibrosis of the peritoneum resulting in the formation of a fibrous cocoon encapsulating the bowel leading to intestinal obstruction. The incidence of EPS ranges between 0.7 and 3.3% and increases with duration of peritoneal dialysis therapy. Dialysis fluid is hyperosmotic, hyperglycemic, and acidic causing chronic injury and inflammation in the peritoneum with loss of mesothelium and extensive tissue fibrosis. The pathogenesis of EPS, however, still remains uncertain, although a widely accepted hypothesis is the "two-hit theory," where, the first hit is chronic peritoneal membrane injury from long standing peritoneal dialysis followed by a second hit such as an episode of peritonitis, genetic predisposition and/or acute cessation of peritoneal dialysis, leading to EPS. Recently, EPS has been reported in patients shortly after transplantation suggesting that this procedure may also act as a possible second insult. The process of epithelial-mesenchymal transition of mesothelial cells is proposed to play a central role in the development of peritoneal sclerosis, a common characteristic of patients on dialysis, however, its importance in EPS is less clear. There is no established treatment for EPS although evidence from small case studies suggests that corticosteroids and tamoxifen may be beneficial. Nutritional support is essential and surgical intervention (peritonectomy and enterolysis) is recommended in later stages to relieve bowel obstruction.
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Affiliation(s)
- Zia Moinuddin
- Department of Transplantation, Manchester Royal Infirmary Manchester, UK ; Faculty of Medical and Human Sciences, Institute of Inflammation and Repair, University of Manchester, Manchester Academic Health Science Centre Manchester, UK
| | - Angela Summers
- Department of Transplantation, Manchester Royal Infirmary Manchester, UK
| | - David Van Dellen
- Department of Transplantation, Manchester Royal Infirmary Manchester, UK
| | - Titus Augustine
- Department of Transplantation, Manchester Royal Infirmary Manchester, UK
| | - Sarah E Herrick
- Faculty of Medical and Human Sciences, Institute of Inflammation and Repair, University of Manchester, Manchester Academic Health Science Centre Manchester, UK
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18
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Hurst H, Summers A, Beaver K, Caress AL. Living with encapsulating peritoneal sclerosis (EPS): the patient's perspective. Perit Dial Int 2014; 34:758-65. [PMID: 24497592 PMCID: PMC4269501 DOI: 10.3747/pdi.2013.00053] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2013] [Accepted: 08/18/2013] [Indexed: 12/19/2022] Open
Abstract
BACKGROUND Although relatively rare, encapsulating peritoneal sclerosis (EPS) is nonetheless a major concern within the renal community. Risk of developing EPS is associated with long-term peritoneal dialysis (PD). High mortality was previously reported, although surgery has since improved outcomes. Research into EPS focuses on imaging and early detection methods, genetics, biomarkers and preventive strategies. No previous studies have examined patients' experiences of EPS. AIMS The aim of the present study was to explore the experience of patients who have undergone surgery for EPS in one center in the North of England. METHODS A qualitative phenomenological approach, involving in-depth interviews, was adopted. Nine participants were recruited out of a total of 18 eligible. Most participants were interviewed twice over a 12-month period (October 2009 to October 2010). ANALYSIS Interpretive data analysis was conducted, following the philosophical tradition of hermeneutics, to draw out themes from the data. Data collection and analysis took place concurrently and participants were sent a summary of their first interview to allow a period of reflection prior to the subsequent interview. RESULTS EPS presented the most serious challenge participants had faced since developing chronic kidney disease (CKD). Three major themes were identified, each with subcategories. The key issues for patients were related to identification of early symptoms and lack of understanding. The patients' sense of 'not being heard' by health care professionals led to a loss of trust and enhanced their feelings of uncertainty. The enormity of the surgery, the suffering, and what they had to endure had an enormous impact, but an overriding aspect of this experience was also the loss they felt for their independence and for the PD therapy over which they had control. CONCLUSIONS The findings of this study highlight a number of important issues relevant to clinical practice, including lack of information and understanding of EPS, particularly its early symptoms At the time patients transfer from peritoneal to hemodialysis, the provision of adequate information about the risks and potential early signs of EPS may not only improve their experiences, but may also assist in early detection.
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Affiliation(s)
- Helen Hurst
- Manchester University, Manchester, UK; Department of Renal Medicine Manchester Royal Infirmary (CMFT), Manchester, UK; University of Central Lancashire, Preston, UK; and University Hospital of South Manchester NHSFT, Manchester, UK
| | - Angela Summers
- Manchester University, Manchester, UK; Department of Renal Medicine Manchester Royal Infirmary (CMFT), Manchester, UK; University of Central Lancashire, Preston, UK; and University Hospital of South Manchester NHSFT, Manchester, UK
| | - Kinta Beaver
- Manchester University, Manchester, UK; Department of Renal Medicine Manchester Royal Infirmary (CMFT), Manchester, UK; University of Central Lancashire, Preston, UK; and University Hospital of South Manchester NHSFT, Manchester, UK
| | - Ann-Louise Caress
- Manchester University, Manchester, UK; Department of Renal Medicine Manchester Royal Infirmary (CMFT), Manchester, UK; University of Central Lancashire, Preston, UK; and University Hospital of South Manchester NHSFT, Manchester, UK
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19
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Habib SM, Dor FJMF, Korte MR, Hagen SM, Betjes MGH. Post-transplantation encapsulating peritoneal sclerosis without inflammation or radiological abnormalities. BMC Nephrol 2013; 14:203. [PMID: 24067112 PMCID: PMC3849826 DOI: 10.1186/1471-2369-14-203] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2013] [Accepted: 09/14/2013] [Indexed: 11/10/2022] Open
Abstract
Background Post-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 presentation A 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. Conclusion EPS 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.
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Affiliation(s)
- Sayed Meelad Habib
- Department of Internal Medicine, Division of Nephrology and Transplantation, Erasmus Medical Center, D-414, P,O, Box 2040, Rotterdam 3000, CA, The Netherlands.
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20
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Shroff R, Stefanidis CJ, Askiti V, Edefonti A, Testa S, Ekim M, Kavaz A, Ariceta G, Bakkaloglu S, Fischbach M, Klaus G, Zurowska A, Holtta T, Jankauskiene A, Vondrak K, Walle JV, Schmitt CP, Watson AR. Encapsulating peritoneal sclerosis in children on chronic PD: a survey from the European Paediatric Dialysis Working Group. Nephrol Dial Transplant 2013; 28:1908-14. [DOI: 10.1093/ndt/gfs603] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
Affiliation(s)
- Rukshana Shroff
- Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
| | | | | | | | - Sara Testa
- Clinica Pediatrica De Marchi, Milan, Italy
| | | | - Asli Kavaz
- Ankara University Hospital, Ankara, Turkey
| | | | | | | | | | | | | | | | | | | | | | - Alan R. Watson
- Nottingham University Hospitals NHS Trust, Nottingham, UK
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