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Tomoko Miyakubo, Ryo Mukai, Hidetaka Matsumoto, Masahiro Morimoto, Maki Takahashi, Kazuki Nagai, Kosuke Nakamura, Junki Hoshino, Hideo Akiyama Department of Ophthalmology, Gunma University, Graduate School of Medicine, Maebashi, Gunma, JapanCorrespondence: Ryo Mukai, Department of Ophthalmology, Gunma University Graduate School of Medicine, 3-39-15 Showa-machi, Maebashi, Gunma, 371-8511, Japan, Tel +81-27-220-8338, Fax +81-27-233-3841, Email rmukai@gunma-u.ac.jpPurpose: To compare the efficacies of photodynamic therapy (PDT) combined with intravitreal aflibercept (IVA) injections and IVA monotherapy using a treat-and-extend regimen (TAE) for treatment-naïve polypoidal choroidal vasculopathy (PCV).Patients and Methods: One hundred and nine eyes treated with PDT combined with IVA (PDT+IVA group: 51 eyes) or IVA monotherapy (IVA group: 58 eyes) were assessed for 2 years. The main outcome measures included best-corrected visual acuity (BCVA), central macular thickness (CMT), central choroidal thickness (CCT), number of IVA injections, and macular atrophy (MA). Polypoidal lesions before and after the loading phase were assessed using indocyanine green angiography.Results: In both groups, BCVA significantly improved after the loading phase and was maintained for 2 years. CMT and CCT were significantly reduced in both groups, without significant differences after 2 years between the groups (P=0.2708). The mean number of IVA injections in the IVA and PDT+IVA groups during the 2 years were 13.2± 3.3 and 12.7± 1.8, respectively, without a significant difference (P=0.06). The frequencies of MA expansion in the IVA and PDT+IVA groups during the 2 years were 25.9% and 33.4%, respectively, with no significant difference in the incidence (odds ratio: 1.40, P=0.4253). The ratios of polyp regression after the loading phase in the IVA and PDT+IVA groups were 55.2% and 94.1%, respectively, with a significant difference (P< 0.0001).Conclusion: PDT combined with IVA injections using a TAE regimen is effective for anatomical and visual function improvement, without a significant difference as compared to IVA monotherapy. It can facilitate complete regression of polyps with higher odds.Keywords: polypoidal choroidal vasculopathy, aflibercept, photodynamic therapy, treat-and-extend, macular atrophy

Distinguishing autosomal‐dominant polycystic kidney disease (ADPKD) from other inherited renal cystic diseases in patients with adult polycystic kidney disease and no family history is critical for correct treatment and appropriate genetic counseling. However, for patients with no family history, there are no definitive imaging findings that provide an unequivocal ADPKD diagnosis. We analyzed 53 adult polycystic kidney disease patients with no family history. Comprehensive genetic testing was performed using capture‐based next‐generation sequencing for 69 genes currently known to cause hereditary renal cystic diseases including ADPKD. Through our analysis, 32 patients had PKD1 or PKD2 mutations. Additionally, 3 patients with disease‐causing mutations in NPHP4, PKHD1, and OFD1 were diagnosed with an inherited renal cystic disease other than ADPKD. In patients with PKD1 or PKD2 mutations, the prevalence of polycystic liver disease, defined as more than 20 liver cysts, was significantly higher (71.9% vs 33.3%, P = .006), total kidney volume was significantly increased (median, 1580.7 mL vs 791.0 mL, P = .027) and mean arterial pressure was significantly higher (median, 98 mm Hg vs 91 mm Hg, P = .012). The genetic screening approach and clinical features described here are potentially beneficial for optimal management of adult sporadic polycystic kidney disease patients. [ABSTRACT FROM AUTHOR]

The association of physical activity with renal outcome and mortality in advanced chronic kidney disease (CKD; i.e., estimated glomerular filtration rate [eGFR] < 45 ml/min/1.73m 2 ) is poorly studied. We examined this association in patients with advanced CKD in Japan. We used the Rapid Assessment of Physical Activity to assess baseline physical activity and classify patients as active or inactive. CKD progression was defined as 40% decline in eGFR, eGFR < 10, or requiring dialysis or transplantation. Among the 1,808 eligible patients, after adjusting for possible confounders, hazard ratios (HRs) for poor renal outcome in the active group were 0.68 (95% CI, 0.44-1.04), 1.09 (0.86-1.38), and 1.01 (0.82-1.25) in CKD stage G3b, G4, and G5, respectively, suggesting a renal benefit of exercise in CKD stage G3b. Adjusted HRs for death were 0.79 (0.40-1.57), 0.55 (0.38-0.80), and 0.75 (0.44-1.26) in stage G3b, G4, and G5, respectively. While the adjusted HRs of death were 0.84 (0.52-1.38) and 0.60 (0.43-0.83) in diabetic and non-diabetic patients, suggesting that exercise may reduce mortality in non-diabetic patients. Our study suggests that exercise is associated with better survival in non-diabetic patients with CKD stage G3b-5, and better renal outcome in diabetic and non-diabetic CKD stage G3b., (© 2024. The Author(s).)

A 37-year-old man with autosomal dominant polycystic kidney disease (ADPKD) was admitted to our hospital with a liver volume of 8,000 cm 3 . Hepatic arterial embolization was performed using a microcoil but was ineffective. Eight years later, the hepatomegaly progressed to liver failure and death. At autopsy, the liver weighed 21.5 kg, and the entire liver had been replaced by cysts; in the few remaining areas of liver parenchyma, microscopic, small cysts of various sizes and fibrosis were evident, with only a few normal hepatocytes observed. Hepatic arterial branches developed; however, the portal vein could not be observed.

Epigenetic mechanisms are considered to contribute to diabetic nephropathy by maintaining memory of poor glycemic control during the early stages of diabetes. However, DNA methylation changes in the human kidney are poorly characterized, because of the lack of cell type-specific analysis. We examined DNA methylation in proximal tubules (PTs) purified from patients with diabetic nephropathy and identified differentially methylated CpG sites, given the critical role of proximal tubules in the kidney injury. Hypermethylation was observed at CpG sites annotated to genes responsible for proximal tubule functions, including gluconeogenesis, nicotinamide adenine dinucleotide synthesis, transporters of glucose, water, phosphate, and drugs, in diabetic kidneys, whereas genes involved in oxidative stress and the cytoskeleton exhibited demethylation. Methylation levels of CpG sites annotated to ACTN1 , BCAR1 , MYH9 , UBE4B , AFMID , TRAF2 , TXNIP , FOXO3 , and HNF4A were correlated with the estimated glomerular filtration rate, whereas methylation of the CpG site in RUNX1 was associated with interstitial fibrosis and tubular atrophy. Hypermethylation of G6PC and HNF4A was accompanied by decreased expression in diabetic kidneys. Proximal tubule-specific hypomethylation of metabolic genes related to HNF4A observed in control kidneys was compromised in diabetic kidneys, suggesting a role for aberrant DNA methylation in the dedifferentiation process. Multiple genes with aberrant DNA methylation in diabetes overlapped genes with altered expressions in maladaptive proximal tubule cells, including transcription factors PPARA and RREB1 . In conclusion, DNA methylation derangement in the proximal tubules of patients with diabetes may drive phenotypic changes, characterized by inflammatory and fibrotic features, along with impaired function in metabolism and transport. NEW & NOTEWORTHY Cell type-specific DNA methylation patterns in the human kidney are not known. We examined DNA methylation in proximal tubules of patients with diabetic nephropathy and revealed that oxidative stress, cytoskeleton, and metabolism genes were aberrantly methylated. The results indicate that aberrant DNA methylation in proximal tubules underlies kidney dysfunction in diabetic nephropathy. Aberrant methylation could be a target for reversing memory of poor glycemic control.

Background: Sacubitril/valsartan, being both a neprilysin inhibitor and angiotensin receptor blocker, exhibits a renin-angiotensin-aldosterone system (RAAS) inhibitory effect. However, no study has investigated the administration of sacubitril/valsartan in patients early after surgery using cardiopulmonary bypass., Methods and Results: This was a prospective observational study of 63 patients who underwent open heart surgery and were treated with sacubitril/valsartan. No serious adverse events occurred. Among the 63 patients, sacubitril/valsartan was discontinued in 13 due to hypotension (n=10), renal dysfunction (n=2), and dizziness (n=1). Atrial natriuretic peptide concentrations increased significantly from Day 3 of treatment (P=0.0142 vs. Postoperative Day 1) and remained high thereafter. In contrast, plasma renin activity was significantly suppressed from Day 3 onwards (P=0.00206 vs. Postoperative Day 1). A decrease in creatinine concentrations and an increase in the estimated glomerular filtration rate were observed on Day 3; this improvement in renal function was not observed in the historical control group, in which patients did not receive sacubitril/valsartan. New postoperative atrial fibrillation was less frequent in the study group compared with the historical control (12.7% vs. 38.0%; P=0.0034)., Conclusions: Sacubitril/valsartan administration was safe immediately after open heart surgery in patients without postoperative hypotension. It enhanced serum atrial natriuretic peptide concentrations and suppressed RAAS activation.

We aimed to develop a novel method for measuring the complement-binding ability of anti-blood type antibodies (ab-Abs), the flow cytometry method for the complement C1q test (FCM-C1q) for detecting antibody-mediated rejection (AMR) caused by ab-Abs in ABO-incompatible kidney transplantation (ABOI-KTx). FCM-C1q distribution was surveyed in 44 healthy participants and 43 dialysis patients (Cohort A). The relationship between AMR and FCM-C1q levels was examined along with ab-Ab titers by the flow cytometry method for the IgG test (FCM-IgG) in 62 ABOI-KTx patients (Cohort B). FCM-IgG and C1q levels were significantly higher in type O participants than in A/B participants in Cohort A. There were minimal differences in the distribution of FCM-IgG and C1q between dialysis and healthy participants. Sixteen cases were suspected of acute rejections (ARs) in Cohort B, of whom nine had AR clinically. One patient with severe AMR was highly suspected of hyperacute rejection along with another patient with severe AMR. Their postoperative FCM-C1q and FCM-IgG levels were elevated. Another two patients showed high FCM-IgG and C1q levels before KTx, and these levels remained low after KTx with no or mild rejection. In conclusion, our results suggest that a high positivity rate for FCM-C1q may predict moderate to severe AMR caused by ab-Abs and poor prognosis in ABOI-KTx.

Competing Interests: The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Introduction: The DAPA-CKD study showed a protective effect of dapagliflozin on kidney function in chronic kidney disease (CKD) patients with and without diabetes mellitus. Although dapagliflozin is expected to be effective also in CKD patients with autosomal dominant polycystic kidney disease (ADPKD), its efficacy and safety in this population remain unknown because ADPKD was an exclusion criterion in the DAPA-CKD study. Therefore, we evaluated the effects of dapagliflozin in CKD patients with ADPKD., Methods: We performed a retrospective observational study of seven patients with ADPKD treated with dapagliflozin at Toranomon Hospital, Tokyo, Japan. We analyzed changes in estimated glomerular filtration rate (eGFR) slope and annual height-corrected total kidney volume before and after starting dapagliflozin treatment., Results: The median observation period after starting dapagliflozin was 20 months. Four patients received concomitant tolvaptan. The eGFR slope before and after initiation of dapagliflozin could be calculated in six patients and improved in all of them except the one who did not receive a renin-angiotensin system (RAS) inhibitor. Annual height-corrected total kidney volume increased in all patients. Concurrent tolvaptan treatment had no effect., Conclusion: In CKD patients with ADPKD, dapagliflozin may increase kidney volume but may have a protective effect on kidney function when used concomitantly with RAS inhibitors., Competing Interests: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)

Although the anticoagulant complications of Impella are well known, the timing of heparin administration when using Impella immediately after open heart surgery has not been established. We report a case of a 59-year-old man with Impella-assisted repair of a ventricular septal perforation after acute myocardial infarction who developed thromboembolism of the lower extremity arteries after removal of Impella., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

In April 2022, an additional medical fee for exercise instruction during haemodialysis treatment was approved for insurance claims in Japan. We conducted a questionnaire survey to investigate the current situation regarding exercise therapy during haemodialysis treatment after this change. Questionnaires were mailed to 4257 haemodialysis facilities, almost all the haemodialysis facilities in Japan, on January 31, 2023. In total, 1657 facilities responded, of which 550 (33%) provided exercise instruction during haemodialysis treatment, and 65% of these claimed the new fee. Of the 550 facilities that had claimed the fee at the time of survey, 245 (55%) started exercise instruction in April 2022 or later. Exercise instruction focused on resistance training (81%) and aerobic exercise (62%) for 20-30 min (66%) three times a week (80%). The instructors included physicians in 45% of facilities, nurses in 74%, and physical therapists in 36%. Efficacy was evaluated in 76% of the facilities providing instruction, mainly by assessing change in muscle strength (49%). Overall, 39% of facilities had experienced some adverse events, but none were life-threatening. In conclusion, after the change in the insurance regime, exercise instruction during haemodialysis treatment has become more popular, and more patients on haemodialysis are undergoing exercise therapy., (© 2024. The Author(s).)

Introduction: The association of hemoglobin level at treatment initiation with renal and cardiovascular outcomes in patients with anemia in nondialysis-dependent (NDD) chronic kidney disease (CKD) is unclear., Methods: This retrospective cohort study utilized 2 Japanese databases (Medical Data Vision Co. Ltd., Tokyo, Japan [MDV]; and Real World Data Co. Ltd, Kyoto, Japan [RWD]). Patients initiated on long-acting erythropoiesis-stimulating agent (ESA) treatment were divided into early (hemoglobin levels ≥9.0 g/dl) and delayed (<9.0 g/dl) treatment groups. The primary outcome was a renal composite (renal replacement therapy, ≥50% estimated glomerular filtration rate [eGFR] reduction, eGFR <6.0 ml/min per 1.73 m 2 , and all-cause mortality), and secondary outcomes were a cardiovascular composite (hospitalization by ischemic heart disease, including myocardial infarction, hospitalization by stroke and heart failure, and cardiovascular death) and components of the composite outcomes., Results: After propensity score matching, 1472 (MDV) and 1264 (RWD) patients were evaluated. Delayed treatment was not associated with a risk of the renal composite outcome (MDV: hazard ratio [HR]: 1.15, 95% confidence interval [CI]: 0.99-1.33; RWD: HR: 1.08, 95% CI: 0.92-1.28). However, delayed treatment was associated with higher risks of the cardiovascular composite outcome (MDV: HR: 1.47, 95% CI: 1.16-1.84; RWD: HR: 1.34, 95% CI: 1.09-1.64), heart failure (MDV: HR: 1.50, 95% CI: 1.13-2.00; RWD: HR: 1.53, 95% CI: 1.20-1.96) and all-cause mortality (MDV: HR: 1.83, 95% CI: 1.32-2.54; RWD: HR: 1.64, 95% CI: 1.21-2.22)., Conclusion: Although the risk of renal events was not increased following delayed treatment of anemia in patients with NDD-CKD, the risks of cardiovascular events and all-cause mortality were increased, suggesting the importance of early intervention before hemoglobin falls below 9.0 g/dl., (© 2024 International Society of Nephrology. Published by Elsevier Inc.)

Purpose: Intervortex venous anastomosis is widely recognized as compensating for vortex vein congestion in pachychoroid spectrum diseases. However, determining the blood flow direction within the compensated drainage route is often challenging. Herein, we investigated the morphological patterns of vortex veins in eyes showing retrograde pulsatile vortex venous flow., Design: Retrospective observational case series., Subjects: Six hundred eighty-nine consecutive eyes with treatment-naive central serous chorioretinopathy, pachychoroid neovasculopathy, or polypoidal choroidal vasculopathy., Methods: We reviewed the clinical records of patients with these pachychoroid spectrum diseases. Multimodal images including indocyanine green angiography (ICGA) and en face OCT were analyzed., Main Outcome Measures: Intervortex venous anastomosis between superotemporal and inferotemporal vortex veins and the dominant site of dilated temporal vortex veins were determined in the eyes with retrograde pulsatile vortex venous flow in the temporal vortex veins., Results: Twenty-two eyes with retrograde pulsatile vortex venous flow in the temporal vortex veins were identified utilizing early phase ICGA videos. In 9 eyes, retrograde pulsatile flow was detected in the superotemporal vortex veins, which were connected to the inferotemporal vortex veins via intervortex venous anastomoses. Among these cases, contralateral inferotemporal vortex vein dilatation was dominant in 7 eyes (77.8%), while superotemporal and inferotemporal vortex veins were symmetrically dilated in the other 2 eyes (22.2%). On the other hand, in 13 eyes, the retrograde pulsatile flow was detected in the inferotemporal vortex veins, which were linked to the superotemporal vortex veins via intervortex venous anastomoses. In these eyes, contralateral superotemporal vortex vein dilatation was dominant in 10 eyes (76.9%). Superotemporal and inferotemporal vortex veins were symmetrically dilated in 2 eyes (15.4%), while mainly inferotemporal vortex veins were dilated in 1 eye (7.7%)., Conclusions: In pachychoroid spectrum diseases, there are cases wherein congested venous blood might drain into the contralateral vortex veins via intervortex anastomoses. Overloaded contralateral vortex veins may, as a consequence, become more dilated than the primary congested vortex veins. Inversion of asymmetric vortex vein dilatation might thereby develop in pachychoroid spectrum diseases., Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (© 2024 by the American Academy of Ophthalmology.)

Background: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease that affects multiple organs, including the pancreas, lacrimal glands, salivary glands, periaortic/retroperitoneum, and kidney. Interstitial nephritis is a typical renal disorder associated with IgG4-RD, but membranous nephropathy is also seen in some cases., Case Presentation: Herein we report on the case of a 77-year-old male patient with nephrotic syndrome and IgG4-related lung disease. His serum phospholipase A2 receptor (PLA2R) antibody was positive. His renal biopsy specimen was also positive for PLA2R. The renal biopsy specimen showed membranous nephropathy with equal IgG3 and IgG4 immunofluorescence staining and no interstitial nephritis, suggesting IgG4-RD manifesting as membranous nephropathy., Conclusions: Nephrotic syndrome caused by membranous nephropathy is sometimes associated with IgG4-RD. In such cases, even if serum PLA2R antibody is positive, it should be considered that the membranous nephropathy may be secondary to IgG4-RD., (© 2024. The Author(s).)

We investigated the assessment of blood flow within polypoidal lesions using optical coherence tomography angiography (OCTA) to determine intravitreal brolucizumab (IVBr) efficacy for treating polypoidal choroidal vasculopathy (PCV). We retrospectively studied 46 eyes with PCV that completed 1-year IVBr treatment. Blood flow signals within polypoidal lesions were evaluated using OCTA after loading-phase treatment, and 1-year outcomes were compared between eyes in which blood flow signals disappeared versus persisting. After loading-phase treatment, blood flow signals within polypoidal lesions disappeared in 31 eyes and persisted in 15. In the former group, visual acuity improved significantly throughout the year (P < 0.01), while in the latter there was no significant difference between baseline and after 1 year. The total number of injections was significantly lower with than without disappearance of blood flow signals (6.0 vs. 6.9, P < 0.01). The intended injection interval at the last visit was significantly longer in the former than in the latter group (15.7 weeks vs. 12.5 weeks, P < 0.01). These results indicate that PCV cases showing disappearance of blood flow signals within polypoidal lesions by OCTA after loading-phase treatment had favorable 1-year outcomes of IVBr. Therefore, evaluating blood flow within polypoidal lesions by OCTA may allow noninvasive prediction of PCV treatment outcomes., (© 2024. The Author(s).)

We retrospectively studied 12 eyes of 12 patients with central serous chorioretinopathy (CSC) to investigate choroidal thickness changes following half-fluence photodynamic therapy (PDT) using widefield choroidal thickness maps obtained by optical coherence tomography (OCT). Additionally, we assessed the relationship between choroidal thickness changes and the regional vortex veins as visualized on widefield en face OCT of the choroid. Pre-treatment en face images of the choroidal vasculature were superimposed on subtracted choroidal thickness maps before and 3 months after half-fluence PDT. The choroidal thickness decreased mainly in the irradiated macular area and in the region of vortex veins which function as drainage for the macula in all eyes. Eleven eyes (91.7%) showed choroidal thinning in the nasal area which overlapped with the nasal vortex vein distribution. Moreover, in 10 (90.9%) of those eyes, we observed intervortex venous anastomosis across the vertical watershed zone. Quantitative analysis revealed that the reduction in choroidal thickness was most pronounced in the macular area. Furthermore, the choroidal thickness reduction in the area with macular drainage vortex veins was significantly greater than that in the area without such vortex veins. These results suggest that half-fluence PDT might decrease choroidal thickness due to choriocapillaris occlusion in the irradiated macula, possibly leading to diminished venous drainage from the macula to regional vortex veins. Moreover, venous blood flow through the anastomotic vessels from the macular drainage vortex veins into the nasal vortex veins might be reduced post-treatment., (© 2023. The Author(s).)

Introduction: Data on longitudinal trajectory of kidney function decline and fluctuation in albuminuria leading to end-stage kidney disease (ESKD) is sparse in patients with type 2 diabetes., Methods: Using data from an observational study of patients with type 2 diabetes and biopsy-confirmed diabetic kidney disease (DKD), generalized additive mixed models (GAMMs) were performed to quantify patterns of longitudinal trajectory of estimated glomerular filtration rate (eGFR) decline to ESKD associated with repeated measures of urine albumin-to-creatinine ratio (ACR)., Results: Over a median follow-up period of 3.3 years, 155 of 319 patients progressed to ESKD. Among these patients, 91.6% exhibited a curvilinear pattern in their eGFR trajectory. The median coefficient of variation for ACR, representing the variability in ACR measurements, was 48.9 (interquartile range: 36.9, 68.2). The median compound annual growth rate (CAGR) for ACR, reflecting the variation in ACR progression over time, was 43.6% (interquartile range: 0.0, 102.5); and 84.5% of patients developed nephrotic-range albuminuria, with a majority remaining nephrotic and subsequently progressing to ESKD. There was a positive association between the instantaneous speed of eGFR decline and ACR., Conclusion: The observed curvilinear pattern in eGFR trajectory, high variability in ACR progression over time, and positive correlation between the speed of eGFR decline and ACR highlight the complex dynamics of disease progression and emphasize close monitoring of ACR fluctuation over time in patients with DKD., (© 2023 International Society of Nephrology. Published by Elsevier Inc.)

Aims/introduction: This multicenter cohort study retrospectively assessed the association between polar vasculosis and the progression of diabetic kidney disease (DKD) in type 2 diabetes., Materials and Methods: We enrolled 811 patients with type 2 diabetes, biopsy-proven DKD, and proteinuria (≥0.15 g/g creatinine [g/day]). The association between polar vasculosis and other kidney lesions was explored. The outcome was DKD progression defined as a composite of renal replacement therapy initiation or 50% decline in estimated glomerular filtration rate (eGFR) from baseline., Results: Of the 811 cases, 677 (83.5%) had polar vasculosis. In multivariate logistic regression analysis, subendothelial widening of the glomerular basement membrane, glomerulomegaly, glomerular class in the Renal Pathology Society classification ≥IIb, vascular lesions, age, eGFR, and hemoglobin A1c were positively associated with polar vasculosis, whereas interstitial fibrosis and tubular atrophy (IFTA) was negatively associated with polar vasculosis. During a median follow-up of 5.2 years, progression of DKD occurred in 322 of 677 (7.4 events/100 person-years) and 79 of 134 (11.4 events/100 person-years) cases with and without polar vasculosis, respectively. Kaplan-Meier analysis showed that polar vasculosis was associated with lower cumulative incidences of DKD progression. Multivariate Cox regression analyses showed that polar vasculosis was associated with a lower risk of DKD progression, regardless of eGFR or proteinuria subgroups. These associations between polar vasculosis and better kidney outcome were unchanged considering all-cause mortality before DKD progression as a competing event., Conclusions: This study showed that polar vasculosis of DKD was associated with less advanced IFTA and a better kidney outcome in type 2 diabetes with proteinuria., (© 2023 The Authors. Journal of Diabetes Investigation published by Asian Association for the Study of Diabetes (AASD) and John Wiley & Sons Australia, Ltd.)

Coronary artery calcification (CAC) is associated with cardiovascular disease (CVD). CAC might contain calcium oxalate, and a high serum oxalate (S Ox ) concentration is associated with cardiovascular mortality in dialysis patients. We assessed the associations between S Ox and CAC or CVD events in Japanese hemodialysis patients. This cross-sectional and retrospective cohort study was done in 2011. Seventy-seven hemodialysis patients' Agatston CAC score was measured, and serum samples were collected. S Ox concentrations were measured in 2021 by using frozen samples. Also, new-onset CVD events in 2011-2021 were retrospectively recorded. The association between S Ox concentration and CAC score ≥ 1000, and new-onset CVD events were examined. Median S Ox concentration and CAC score were 266.9 (229.5-318.5) µmol/L and 912.5 (123.7-2944), respectively. CAC score ≥ 1000 was associated with S Ox [adjusted odds ratio (OR) 1.01, 95% confidence interval (CI), 1.00-1.02]. The number of new-onset CVD events was significantly higher in patients with S Ox  ≥ median value [hazard ratio (HR) 2.71, 95% CI 1.26-6.16]. By Cox proportional hazard models, new-onset CVD events was associated with S Ox  ≥ median value (adjusted HR 2.10, 95% CI 0.90-4.91). S Ox was associated with CAC score ≥ 1000 and new-onset CVD events in Japanese hemodialysis patients., (© 2023. The Author(s).)

Cyst infection is a frequent and serious complication of autosomal dominant polycystic kidney disease (ADPKD). Hematogenous spread via bacterial translocation in the intestine is considered to be the main cause, so intestinal flora may be involved. However, the exact role of the intestinal flora in cyst infection in ADPKD is unknown. We report a 66-year-old woman and a 56-year-old man with ADPKD who had severe hepatic cyst infection. We analyzed the microbiome of infected cyst content, feces, and saliva in these two patients. The microbiome of patient 1 showed various bacteria in an infected cyst, whereas that of patient 2 showed only one bacterium. In both patients, the composition of the microbiome of the cyst content was quite different from those of feces and saliva, and the main bacteria in the infected cyst content represented a small proportion of those in feces and saliva. Lactobacilli were not almost detected in the infected cyst content though some lactobacilli are endemic in the gastrointestinal tract and the saliva. The association between bacteria in cysts and those in feces or saliva remains uncertain, and further research on this topic is needed., (© 2022. The Author(s) under exclusive licence to The Japan Society of Nephrology.)

A 16-year-old Japanese girl developed a fever, thrombocytopenia, and renal dysfunction. Treatment was started with steroids, but cervical lymphadenopathy and ascites developed. A lymph node biopsy indicated TAFRO syndrome. The patient's renal function deteriorated, and dialysis was started. Refractory hypertension and subsequent encephalopathy developed. Treatment was started with an anti-IL-6 receptor antibody and an anti-CD20 monoclonal antibody. A kidney biopsy showed malignant nephrosclerosis-like microangiopathy and glomerular collapse due to narrowing of the small arteries. The majority of TAFRO syndrome cases are adult-onset, with glomerular microangiopathy. To our knowledge, this is the first report of adolescent-onset TAFRO syndrome presenting with malignant nephrosclerosis-like lesions associated with hypertension.

The success of sodium-glucose cotransporter 2 inhibitors and bariatric surgery in patients with chronic kidney disease has highlighted the importance of glomerular hyperfiltration and hypertrophy in the progression of kidney disease. Sustained glomerular hyperfiltration and hypertrophy can lead to glomerular injury and progressive kidney damage. This article explores the relationship between obesity and chronic kidney disease, focusing on the roles of glomerular hyperfiltration and hypertrophy as hallmarks of obesity-related kidney disease. The pathological mechanisms underlying this association include adipose tissue inflammation, dyslipidemia, insulin resistance, chronic systemic inflammation, oxidative stress, and overactivation of the sympathetic nervous system, as well as the renin-angiotensin aldosterone system. This article explains how glomerular hyperfiltration results from increased renal blood flow and intraglomerular hypertension, inducing mechanical stress on the filtration barrier and post-filtration structures. Injured glomeruli increase in size before sclerosing and collapsing. Therefore, using extreme values, such as the maximal glomerular diameter, could improve the understanding of the data distribution and allow for better kidney failure predictions. This review provides important insights into the mechanisms underlying glomerular hyperfiltration and hypertrophy and highlights the need for further research using glomerular size, including maximum glomerular profile, calculated using needle biopsy specimens., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Kataoka, Nitta and Hoshino.)

Background: Hemoglobin A1c (A1c) and glycated albumin (GA) are two blood glycated proteins commonly used to monitor glycemic control in dialysis patients with diabetes. However, little is known about the association between the GA/A1c ratio and mortality in these populations. Here, we examine these associations using a nationwide cohort., Methods: We enrolled 28 994 dialysis patients with diabetes who met our inclusion criteria (female, 32.9%; mean age, 67.4 ± 11.6 years; mean dialysis duration, 6.3 ± 5.8 years). After dividing the patients into groups based on GA/A1c quantiles and adjusting for 18 potential confounders, adjusted hazard ratios (HR) and 95% confidence limits were calculated for 3-year mortality and cause-specific mortalities. Additionally, propensity score matching analyses were used to compare mortalities between the low and high GA/A1c groups., Results: After adjusting for possible confounders, significantly increased mortality was found in patients with GA/A1c ratios of 3.6-4.0 [HR 1.21 (1.10-1.34)] or higher [HR 1.43 (1.30-1.58)] than in those with GA/A1c ratios of 3.0-3.3. The risks of infectious and cardiovascular death were higher in these patients regardless of their nutritional status. In the propensity score matching analyses, significantly increased mortality was consistently found in those with a higher ratio (≥3.3) [HR 1.23 (1.14-1.33)] than in those with a lower ratio., Conclusions: The GA/A1c ratio was significantly associated with 3-year mortality, especially infectious and cardiovascular mortality, in dialysis patients with diabetes. This ratio may be a promising new clinical indicator of survival in these patients, independent of their current glycemic control and nutritional markers., (© The Author(s) 2022. Published by Oxford University Press on behalf of the ERA.)