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Introduction: The effect of shared decision-making (SDM) regarding the choice of renal replacement therapy (RRT) for chronic kidney disease (CKD) patients on their mortality after the induction of dialysis therapy has not been adequately investigated., Methods: Patients who initiated dialysis at our hospital were divided into two groups according to whether they participated in SDM in the outpatient clinic, and survival analysis was performed. We also examined the effect of SDM in the outpatient clinic on mortality., Results: Of the 554 patients, 123 (22.2%) were in the SDM group. The survival rate was significantly higher in the SDM group (p = 0.001, log-rank test). Multivariate analysis excluding ADL, which competed with SDM, showed that SDM was significantly associated with mortality (HR 0.593, 95% CI: 0.353-0.997, p = 0.049)., Conclusion: SDM regarding RRT selection in the outpatient clinic may be associated with a better patient prognosis after dialysis induction., (© 2024 International Society for Apheresis and Japanese Society for Apheresis.)

Familial pseudohyperkalemia (FP) is an underrecognized cause of pseudohyperkalemia, caused by the leaking of potassium from red blood cells. This extracellular shift of potassium is temperature-dependent and occurs when blood samples are stored below room temperature or for a long time, manifesting as apparent hyperkalemia. We report a suspicious case of FP, which demonstrated an apparent seasonal trajectory of serum-potassium levels at the local clinic. At first, laboratory test results did not show an increase in the serum-potassium levels in our tertiary hospital. However, by replicating the clinic's storage conditions, the patient's serum sample showed hyperkalemia only when it was stored at a temperature of 4 °C or 20 °C for 4-8 h. Hyperkalemia was not observed in the patient's sample when it was stored at 37 °C, or in the healthy control's sample at a temperature of 20 °C or 37 °C. When encountering hyperkalemia without an obvious cause and symptoms, physicians should consider pseudohyperkalemia in the differential diagnosis. In particular, if a seasonal trajectory of serum-potassium levels is observed, FP should be suspected as a potential cause of pseudohyperkalemia. Although a genetic test is needed to properly diagnose FP, confirming it by verifying the sample storage conditions or proving it by replicating the test using different storage conditions is easy and very important, as it can prevent unnecessary treatment., (© 2024. The Author(s), under exclusive licence to Japanese Society of Nephrology.)

Key Messages: The majority of dialysis patients and clinicians favor early advance care planning in our sample. Yet, there is a disconnect: only 11% of patients discussed future care with their clinicians. Our findings indicate Japanese dialysis patients and clinicians support proactive advance care planning at or before dialysis initiation., Background: Little is known about the optimal timing of discussions about advance care planning among dialysis patients and clinicians engaged in dialysis care. We aimed to explore the preferred timing for advance care planning and assess actual participation in advance care planning among dialysis patients and their clinicians., Methods: A scenario-based survey on Japanese patients aged ≥65 years on dialysis and clinicians involved in their dialysis care was performed. Participants were asked if they would feel prepared to engage in advance care planning with their clinicians, offering a choice among four hypothetical stages within the illness trajectory, extending from the initiation of dialysis to a later phase characterized by the patient's extreme frailty., Results: Overall, 181 patients and 128 clinicians participated in the study. Among these, 131 (72%) patients, and 84 (66%) clinicians indicated that they would prefer to initiate advance care planning around the time of dialysis initiation. Only 20 patients (11%) indicated that they had participated in advance care planning with at least one clinician, including 11 (6%) who indicated that they had discussed their preferences around life-sustaining treatments and 8 (4%) who had discussed their preferences around dialysis continuation., Conclusions: While fewer than 11% of patients undergoing dialysis and their clinicians enrolled in our study had participated in advance care planning, most indicated that they would be comfortable initiating the discussion around the time of dialysis initiation. These findings suggest untapped opportunities to engage patients in advance care planning early in the course of their dialysis., (© 2024. The Author(s), under exclusive licence to Japanese Society of Nephrology.)

Background: The aim of present study was to evaluate the effects of one-hour discussion on the choice of dialysis modality at the outpatient clinic., Methods: Charts of consecutive patients who had started maintenance dialysis from May 2013 to April 2021 were retrospectively reviewed. Characteristics at the start of dialysis were compared between patients participated and not participated in the discussion., Results: Of the 620 incident dialysis patients, 128 patients had participated in the discussion. After propensity score matching (1:1), 127 patients who participated in the discussion tended to have fewer urgent hospitalizations (13.4% vs. 21.3%, p = 0.068). In addition, more patients who initiated peritoneal dialysis (PD) (30.7% vs. 9.4%, p < 0.001). On multivariate analysis, participation in the discussion (OR 4.81, 95% CI 2.807-8.24; p < 0.001) was related to PD initiation., Conclusion: One-hour discussion on the choice of dialysis modality may increase PD initiations and decrease the number of urgent hospitalizations., (© 2022 International Society for Apheresis and Japanese Society for Apheresis.)

Background: Cytotoxic T lymphocyte antigen-4 (CTLA-4) is an essential inhibitory regulator of immune activation. CTLA-4 haploinsufficiency is known to be associated with dysregulation of FOXP3 + regulatory T cells, hyperactivation of effector T cells, and lymphocytic infiltration of multiple organs. However, there have only been a few reports of renal involvement with CTLA-4. Herein, we present a case of acute granulomatous tubulointerstitial nephritis (TIN) in a patient with CTLA-4 haploinsufficiency., Case Presentation: A 44-year-old man presented with a 3-week history of fever and malaise, and subsequently developed acute kidney injury (AKI) a few days after treatment with levofloxacin (LVFX). A kidney biopsy and immunohistochemical staining revealed granulomatous TIN with dominantly infiltrating CD4 + T cells. General symptoms and renal impairment showed improvement after discontinuation of LVFX and initiation of oral steroids. However, they worsened following steroid tapering. Further, a colon biopsy analysis showed similar findings to the renal tissue analysis. We suspected that granulomatous TIN was possibly associated with CTLA-4 haploinsufficiency. Therefore, the patient was transferred to another hospital for further treatment of CTLA-4 haploinsufficiency using immunosuppressive agents., Conclusions: There have been few reports regarding renal involvement of CTLA-4 haploinsufficiency. In the present case, granulomatous TIN could have arisen due to instability of immune regulatory functions, such as CTLA-4 haploinsufficiency, and treatment with LVFX could have triggered immunologic activation and severe inflammation as well as renal dysfunction., (© 2022. The Author(s).)

Whether dietary salt intake affects chronic kidney disease (CKD) progression remains unclear. We conducted a retrospective cohort study to analyze the effects of both daily salt intake (DSI) and volume status on renal outcomes in 197 CKD patients. DSI was estimated by 24-h urinary sodium excretion and volume status was assessed by the ratio of extracellular water (ECW) to total body water (TBW) measured by bioelectrical impedance analysis (BIA). We divided patients into two groups according to DSI (6 g/day) or median ECW/TBW (0.475) and compared renal outcomes of each group. Furthermore, we classified and analyzed four groups according to both DSI and ECW/TBW. The higher DSI group showed a 1.69-fold (95% confidence interval (CI) 1.12-2.57, p = 0.01) excess risk of outcome occurrence compared to the lower group. Among the four groups, compared with Group 1 (low DSI and low ECW/TBW), Group 3 (high DSI and low ECW/TBW) showed a 1.84-fold (95% CI 1.03-3.30, p = 0.04) excess risk of outcome occurrence; however, Group 2 (low DSI and high ECW/TBW) showed no significant difference. High salt intake appears to be associated with poor renal outcome independent of blood pressure (BP), proteinuria, and volume status.

Background: Peritonitis is one of the major complications of peritoneal dialysis (PD). Although several reports have indicated seasonality of peritonitis, the observation periods were short, and there were no reports from Japan. Therefore, the purpose of this study was to investigate the long-term seasonality of peritonitis in a single institution in Japan., Methods: Of 126 patients who started PD in our hospital between January 1, 2009, and December 31, 2018, 25 patients (15 men, 10 patients with diabetes) developed peritonitis with a total of 42 episodes. The median age at onset was 63 years, and the median duration from the start of PD to the onset of peritonitis was 22 months., Results: The 10-year incidence of peritonitis was 0.12 episodes per patient-year. Compared with the reference season of winter (December-February), the incidence rate ratios (95% confidence interval) for spring (March-May), summer (June-August), and autumn (September-November) were 1.75 (0.65-4.75), 1.56 (0.57-4.31), and 2.42 (0.94-6.23), respectively. In addition, no seasonality of Gram-positive and Gram-negative organisms was observed., Conclusion: No seasonality was evident in the incidence of PD-related peritonitis in our hospital over a 10-year period. These findings suggest that the development of peritonitis in Japanese PD patients is not affected by seasonality.

Background: Preeclampsia (PE) refers to the development of hypertension and new-onset proteinuria or progressive organ damage (especially kidney) in a previously normotensive pregnant women after 20 weeks of gestation. Thus, new-onset nephrotic syndrome due to PE before 20 weeks of gestation seems to be rare, making its diagnosis difficult in this time period., Case Presentation: A 28-year-old woman presented with a new-onset nephrotic syndrome at 16 weeks of gestation. A high dose of oral glucocorticoids (prednisolone, 40 mg) was initiated for presumed glomerulonephritis since she presented with severe nephrotic syndrome before 20 weeks of gestation, however, the treatment was not effective. At 21 weeks of gestation, we confirmed that the soluble fms-like tyrosine kinase-1 (sFlt-1)/placental growth factor (PlGF) ratio was very high (sFlt-1, 13,400 pg/mL; PlGF, 21.9 pg/mL; serum sFlt-1/PlGF ratio 611.9). Therefore, we diagnosed nephrotic syndrome due to PE, and oral glucocorticoids were discontinued. After she underwent a cesarean section at 24 weeks & 3 days, we performed a kidney biopsy. Focal segmental sclerotic lesions with epithelial cell hyperplasia and foam cells in the tubular poles were seen on light microscopy. On immunofluorescence tests, C4d staining showed linear peripheral patterns in the glomeruli. Electron microscopy revealed diffuse subendothelial edema with focal foot process effacement. The histological diagnosis was severe glomerular endotheliosis with focal segmental glomerulosclerosis. Furthermore, the histology of placenta was consistent with PE. Eight months after delivery, her proteinuria disappeared completely., Conclusions: We not only confirmed an abnormal serum sFlt-1/PlGF ratio but also presented the histology compatible with pure PE in the kidney and placenta in a case of nephrotic syndrome before 20 weeks of gestation. The serum sFlt-1/PlGF ratio may be useful in determining the treatment strategy for atypical cases of pregnant women with nephrotic syndrome, particularly before 20 weeks of gestation.

Introduction: Minimal change disease (MCD) and primary focal segmental glomerulosclerosis (FSGS) are representative podocyte diseases. The clinical cause of MCD and FSGS has not been clearly elucidated yet. However, it is important to distinguish MCD and FSGS because their prognoses and responses to treatment are quite different., Objective: This study aimed to examine whether parietal epithelial cell (PEC) marker and repeat biopsy are useful for diagnosing primary FSGS., Methods: Clinicopathological features of 17 patients with the nephrotic syndrome, who underwent kidney biopsy ≥2 times from 1975 to 2017, and had MCD or FSGS were analyzed using PAX8. We defined patients with PAX8+ cells as PAX8+ and the remainder as PAX8- patients. Three cases of sample insufficiency and 1 non-steroid-resistant or frequently relapsing case indicated for repeat biopsy were excluded., Results: Among the 13 patients studied, 4 were PAX8+ and 9 were PAX8- (median age: 41 and 46 years, -respectively, at first biopsy). PAX8+ and PAX8- patients showed no significant differences in clinical data and histological diagnosis except for a significant difference in histological diagnosis at the second biopsy. The number of PAX8+ patients increased to 6. Unlike the first biopsy results, FSGS was present in 5 of 6 (83.3%) PAX8+ patients; MCD occurred in all 7 (100%) PAX8- patients. Three of 6 (50.0%) PAX8+ patients undergoing repeat biopsy were steroid resistant; no (0%) PAX8- patient was steroid resistant. All cases of final FSGS diagnosis were PAX8+ at the first or second biopsy. Only 1 PAX8+ MCD patient was steroid resistant. All PAX8- MCD patients were frequently relapsing., Conclusions: More PAX8+ patients were diagnosed with FSGS than PAX8- patients. Clinical presentation of MCD in PAX8- patients was frequently relapsing. PEC marker staining in patients with the nephrotic syndrome, e.g., MCD, may help to diagnose FSGS., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2020 by S. Karger AG, Basel.)

A retrospective chart review was undertaken of 204 patients who underwent dynamic urethroscopy. Ninety-nine patients with a diagnosis of genuine stress incontinence were evaluated urodynamically and urethroscopically to determine the predictive accuracy of the sensitivity and specificity of each of these diagnostic modalities. The urodynamic cough profile was both highly reactive and specific for genuine stress incontinence, with sensitivity and specificity of 95% and 100%, respectively, whereas dynamic urethroscopy yielded sensitivity and specificity of 60.2% and 79.1%, respectively. Dynamic urethroscopy is a relatively insensitive predictor of genuine stress incontinence, with many equivocal findings (11%) as well. The urodynamic cough urethral pressure profile is recommended for diagnosing this condition.