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Abstract Pheochromocytoma and paraganglioma (PPGL) represent a group of rare neuroendocrine tumors known for their potential to metastasize. This study provides a comprehensive retrospective evaluation of 15 patients diagnosed with metastatic or recurrent PPGL at our institution over a two-decade span (2000–2020). Our primary objectives were to delineate the long-term clinical outcomes and pinpoint key prognostic determinants. Median duration from initial PPGL diagnosis to the onset of metastasis or recurrence stood at 5.8 years. Predominant sites for metastasis included the bone, lung, lymph nodes, and peritoneum. A salient finding was that surgical interventions targeting metastatic lesions significantly improved prognosis. Further analysis revealed that a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) exceeding 7 closely associated with unfavorable outcomes. These insights not only underscore the clinical variability of PPGL’s progression but also highlight the pivotal role of surgical management for metastatic or recurrent cases. The value of the PASS score as an informative prognostic tool was evident, suggesting its utility in shaping future therapeutic approaches. Given the intricacies of PPGL, collaborative studies involving larger patient cohorts will be crucial to optimize management strategies and prognostication.

IntroductionCytomegalovirus (CMV) is well established to be an independent risk factor for graft loss after kidney transplantation (KTx). Monitoring for CMV in the chronic phase is not defined in the current guideline. The effects of CMV infection, including asymptomatic CMV viremia, in the chronic phase are unclear.MethodsWe performed a single-center retrospective study to investigate incidence of CMV infection in the chronic phase, defined as more than 1 year after KTx. We included 205 patients who received KTx between April 2004 and December 2017. The CMV pp65 antigenemia assays to detect CMV viremia were continuously performed every 1–3 months.ResultsThe median duration of the follow-up was 80.6 (13.1–172.1) months. Asymptomatic CMV infection and CMV disease were observed in 30.7% and 2.9% in the chronic phase, respectively. We found that 10–20% of patients had CMV infections in each year after KTx which did not change over 10 years. The history of CMV infection in the early phase (within 1 year after KTx) and chronic rejection were significantly associated with CMV viremia in the chronic phase. CMV viremia in the chronic phase was significantly associated with graft loss.DiscussionThis is the first study to examine the incidence of CMV viremia for 10 years post KTx. Preventing latent CMV infection may decrease chronic rejection and graft loss after KTx.

In Japan, since the revision of the Organ Transplant Law in July 2010, donation after brain death (DBD) has increased, while donation after cardiac death (DCD), which has been mainly used by kidney transplantation, has decreased. The number of DCD donors decreased from 98 in 2009 to 28 in 2019. There is no clear reason for the decrease in DCD donors. Furthermore, since 2020, there has been a marked decrease in DCD due to the influence of the COVID-19 pandemic. On the other hand, the outcomes after kidney transplantation from DCD donors are improving year by year. The outcomes of kidney transplantation from DCD donors in Japan are comparable to those of kidney transplantation from DBD donors in Western countries. In order to further improve transplantation outcomes from DCD donors, the clinical introduction of continuous machine perfusion preservation technology, for the purpose of reducing ischemic reperfusion injury, is expected in Japan.

Introduction: Lethal symptoms due to graft rejection warrant rapid removal of the transplanted graft to save the patient's life. We report a case of massive pancreas graft bleeding due to chronic rejection that necessitated graft removal after hemostasis by stent graft insertion. Case presentation: A 39-year-old woman underwent simultaneous pancreas-kidney transplantation for type I diabetes and chronic renal failure nine years ago. She suffered irreversible kidney damage from severe chronic rejection due to drug non-compliance. She was admitted to the emergency department for abdominal pain and bloody stools. She presented with signs of shock based on her vitals due to massive bleeding in the stool a day after hospitalization and required systemic management in the intensive care unit (ICU). Enhanced computed tomography (CT) scan revealed active bleeding from the duodenal portion of the pancreas graft. Hemostasis was achieved by inserting a stent graft into the right external iliac artery. The respiratory and circulatory status of the patient improved after the intervention, and she was transported to our hospital day after treatment. The graft was removed along with the part of the anastomosed intestine, which was reconstructed with a functional end-to-end anastomosis. Conclusion: We encountered a patient with hemorrhagic shock due to bleeding from a rejected pancreas graft. The patient was successfully treated and saved using stent-graft hemostasis followed by graft removal. Clinicians and surgeons should be mindful of chronic rejection, which could lead to life-threatening hemodynamic complications.

Abstract Renal anemia is a complication of chronic kidney disease. Guidelines for safe and effective treatment in patients with renal anemia are needed. The Japanese Society for Dialysis Therapy (JSDT) published guidelines for the treatment of renal anemia in chronic hemodialysis patients in 2004 and in hemodialysis, peritoneal dialysis, predialysis, and pediatric patients in 2008. These two publications provide excellent guidance with respect to clinical practice issues, including the definition and diagnosis of renal anemia, the criteria for the initiation of treatment, target hemoglobin levels, iron supplementation therapy, blood transfusion, and side effects. The guidelines significantly improved the treatment of renal anemia in Japan. However, since 2008, many studies have assessed the treatment of renal anemia, and erythropoiesis-stimulating agents (ESAs) are now available. Therefore, the Executive Board of the JSDT decided that it was time to revise the guidelines to make them more appropriate to the situation of chronic kidney disease patients in Japan. This is the third edition of the guidelines for renal anemia published by the JSDT. The purpose is to improve the prognosis of chronic kidney disease patients, including after renal transplantation, through the treatment of renal anemia. The intended users of the guidelines are all healthcare professionals engaged in the treatment of chronic kidney disease. Regarding the treatment of adult dialysis and predialysis patients, statements and commentary are provided in the context of answers to clinical questions in Chapter 2 (Target Hb level and criteria for starting renal anemia treatment) and Chapter 4 (Evaluation of iron status and iron therapy). Furthermore, the essential information is provided alongside the critical issues in Chapter 1 (Diagnosis of renal anemia), Chapter 3 (Administration of ESAs—administration route and dose), Chapter 5 (ESA hyporesponsiveness), Chapter 6 (Side effects and concomitant symptoms of ESAs), and Chapter 7 (Red blood cell transfusion). In addition, the treatment of pediatric patients and post-renal transplant patients is discussed in Chapter 8 and Chapter 9, respectively.

The Japanese ABO-Incompatible Transplantation Committee officially collected and analyzed data on pediatric ABO-incompatible living-donor kidney transplantation in July 2012. The age of a child was defined as

Basiliximab is a recently developed immunosuppressive agent for the prevention of acute allograft rejection in renal transplant recipients. The combination use of basiliximab and a calcineurin inhibitor was suggested to be more effective in comparison to immunosuppressive therapy using calcineurin inhibitor without basiliximab. Cyclosporine has been generally administered with basiliximab for renal transplant recipients. However, in cases of tacrolimus-based immunosuppressive regimen, the clinical efficacy and safety of combined use of tacrolimus and basiliximab remains to be elucidated. This study evaluated the tacrolimus pharmacological efficacy using a lymphocyte immunosuppressant sensitivity test (LIST) with MTT assay procedures in 16 cases of renal transplant recipients treated by tacrolimus without basiliximab and in 13 cases treated by tacrolimus in combination with basiliximab. The rate of acute rejection episodes in the recipients treated with tacrolimus plus basiliximab was 1/13 (7.7%), whereas the rate in the recipients treated with tacrolimus without basiliximab was 6/16 (37.5%). The recipients were divided into two groups according to their peripheral blood mononuclear cell (PBMC) sensitivity to tacrolimus [i.e., including a tacrolimus high sensitivity group (IC 50 1.0 ng/ml). In the recipients treated with tacrolimus without basiliximab, the rate of acute rejection episodes in the tacrolimus high sensitivity group was 1/10 (10.0%), which was significantly lower than the rate in the low sensitivity group of 5/6 (83.3%; p = 0.008). The incidence of cytomegalovirus infection was not significantly different between the tacrolimus high and the low sensitivity groups of the recipients treated with tacrolimus with and without basiliximab. Therefore, in the case of selected tacrolimus-based immunosuppressive therapy for renal transplant recipients, the tacrolimus pharmacological efficacy should be evaluated using LIST at a time just before the transplant procedure in order to accurately predict allograft rejection. The data also suggested that low tacrolimus sensitivity recipients should be treated with tacrolimus-based immunosuppressive therapy in combination with basiliximab.

Isohemagglutinin assays employing red blood cells (RBCs) are the most common assays used to measure antibody titer in ABO-incompatible kidney transplantation (ABOi KTx). However, ABO antigens expressed on RBCs are not identical to those of kidney and antibody titers do not always correlate with clinical outcome. We previously reported that CD31 was the main protein linked to ABO antigens on kidney endothelial cells (KECs), which was different from those on RBCs. We developed a new method to measure antibody titer using a microarray of recombinant CD31 (rCD31) linked to ABO antigens (CD31-ABO microarray). Mass spectrometry analysis suggested that rCD31 and native CD31 purified from human kidney had similar ABO glycan. To confirm clinical use of CD31-ABO microarray, a total of 252 plasma samples including volunteers, hemodialysis patients, and transplant recipients were examined. In transplant recipients, any initial IgG or IgM antibody intensity >30,000 against the donor blood type in the CD31-ABO microarray showed higher sensitivity, specificity, positive predictive value, and negative predictive value of AABMR, compared to isohemagglutinin assays. Use of a CD31-ABO microarray to determine antibody titer specifically against ABO antigens expressed on. [ABSTRACT FROM AUTHOR]

ABSTRACT Purpose: The purposes of the present study were to evaluate growth rate of nonfunctioning adrenal incidentalomas (AIs) and their development to hormonal hypersecretion on follow-up. Materials and methods: A retrospective study was conducted from the electronic medical records. A total of 314 patients were diagnosed with adrenal tumors between 2000 and 2016. After excluding patients who had overt adrenal endocrine disorders or whose adrenal tumors were clinically diagnosed as metastatic malignancies, we investigated 108 patients with nonfunctioning AIs including characteristics, the treatment, the way of follow-up and pathology. Results: Fifteen patients received immediate adrenalectomy because of the initial tumor size or patient's preference. Pathological examination revealed malignancy in 2 patients. In the remaining 93 patients, radiological examinations were performed periodically. Tumor enlargement of ≥ 1.0cm was observed in 8.6% of the patients who were followed up as nonfunctioning AIs with a median follow-up period of 61.5 months (range: 4-192). Eleven patients underwent adrenalectomy. On the pathological examinations, all of the tumors, which showed a size increase, were diagnosed as benign tumors. Regarding the followed up patients without adrenalectomy, only 2.4% of the patients had tumor enlargement during the prolonged follow-up. Furthermore, none of the patients developed hormonal hypersecretion or clinical signs such as obesity, glucose intolerance or poorly controlled hypertension. Conclusions: Tumor enlargement of AIs did not correlate with malignancy. The value of repeat radiological and hormonal examinations may be limited in the long-term follow-up of patients whose AIs are not enlarged.

Background: The hand-assisted technique enables the rapid extraction of the graft, shortening the warm ischemia time (WIT), and the retroperitoneoscopic approach is potentially associated with a less incidence of postoperative ileus in donor nephrectomy for living kidney transplantation. The aim of this study was to assess the efficacy and safety of retroperitoneoscopic donor nephrectomy with a gel-sealed hand-assist access device (GelPort), which is a wound sealing device that permits the access of the hand to the surgical field, free trocar site choice within it, and rapid conversion to open surgery if necessary, while preserving the pneumoperitoneum/pneumoretroperitoneum. Methods: Seventy-five consecutive donors receiving this procedure were retrospectively studied. A 2-cm skin incision was made at the midpoint between the tip of the 12th rib and superior border of the iliac bone in the midaxillary line, through which retroperitoneal space was made. Preperitoneal wound with a 6 - 7-cm pararectal incision in the upper abdominal region was connected to the retroperitoneal space. A GelPort was put inside the pararectal surgical wound. The principle was pure retroperitoneoscopic surgery; hand-assist was applied for retraction of the kidney in the renal vessel control and graft extraction. Results: The mean operation time including waiting time for recipient preparation was 242.2±37.0 (range: 214.0- 409.0) min, and the mean amount of blood loss was 164.3±146.6 (range: 10.0-1020.0) ml. The mean WIT was 2.8±1.0 (range: 1.0-6.0) min. The shortage of renal vessels or ureter was observed in none of the grafts. No donor experienced blood transfusion, open conversion, or injury of other organs. Blood loss was greater in patients with body mass index (BMI) of 25 kg/m² or higher than in those with BMI of <25 kg/m² (218.4±98.8 vs. 154.8±152.1 ml, P=0.031). No donor had postoperative ileus or reported wound pain leading to decreased activity of daily life or wound cosmetic problem. Conclusions: Retroperitoneoscopic hand-assisted donor nephrectomy with the mentioned approach was suggested to be a feasible option without compromising safety, although further improvement in surgical techniques is warranted. [ABSTRACT FROM AUTHOR]

Background. Complement 4d (C4d) deposition in the peritubular capillary (PTC) in the kidney allograft is a useful diagnostic marker for humoral rejection. C4d is produced not only by the classical pathway but also by the lectin pathway of the complement activation cascade. We have recently reported the in situ role of the later phase of the complement cascade in renal allografts with C4d deposition; however, the initial process prior to C4d deposition is yet to be resolved.Methods. To clarify the early phases of the complement activation cascade, we evaluated the deposition of initial proteins of the above two pathways; IgG, IgM, mannose-binding lectin (MBL), H-ficolin, L-ficolin, MBL-associated serine protease (MASP)-1 and MASP-2 in kidney allografts with PTC C4d deposition.Results. Sixty kidney allograft specimens were divided into two groups on the basis of the presence of C4d deposition in PTC. The C4d-positive group (n = 18) included nine ABO-identical and nine ABO-incompatible cases, and the C4d-negative group (n = 42) had 34 ABO-identical and eight ABO-compatible (but not identical) cases. In the C4d-positive group, 16 of 18 cases showed diffuse H-ficolin and IgM deposition in PTC. In contrast, H-ficolin and IgM were not detected in PTC in the C4d-negative group. Other initial proteins were not detected in all cases.Conclusions. Our study suggested for the first time that the lectin pathway activated by H-ficolin may be involved in C4d deposition on PTC in the kidney allograft. [ABSTRACT FROM AUTHOR]