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Background: Clinical practice guidelines recommend antihypertensive and tolvaptan therapies for patients with autosomal dominant polycystic kidney disease (ADPKD) in Japan. However, tolvaptan therapy may pose an economic burden. The Japanese Ministry of Health, Labour and Welfare supports patients with intractable diseases. This study aimed to confirm the impact of the intractable disease system in Japan on the clinical treatment of ADPKD. Methods: We analyzed the data of 3768 patients with ADPKD having a medical subsidy certificate from the Japanese Ministry of Health, Labour and Welfare in 2015–2016. The following quality indicators were use: the adherence rate to the 2014 clinical practice guideline for polycystic kidney disease (prescription rates of antihypertensive agents and tolvaptan in this cohort) and the number of Japanese patients with ADPKD nationwide started on renal replacement therapy in 2014 and 2020. Results: Compared with new applications from 2015 to 2016, the prescription rates of antihypertensives and tolvaptan for the indicated patients at the 2017 renewal application increased by 2.0% (odds ratio = 1.41, p = 0.008) and 47.4% (odds ratio = 10.1, p > 0.001), respectively. These quality indicators improved with antihypertensive treatment, especially in patients with chronic kidney disease stages 1–2 (odds ratio = 1.79, p = 0.013) and in those aged < 50 years (odds ratio = 1.70, p = 0.003). The number of patients with ADPKD who were started on renal replacement therapy in Japan decreased from 999 in 2014 to 884 in 2020 in the nationwide database (odds ratio = 0.83, p < 0.001). Conclusions: The Japanese public intractable disease support system contributes to improvement of ADPKD treatment. [ABSTRACT FROM AUTHOR]

Background: The prognosis of lupus nephritis (LN) has improved following the introduction of effective immunosuppressive therapy and progress in supportive care. This study examined recent renal and patient prognosis for adults with LN in Japan. Methods: We conducted a nationwide retrospective cohort study of LN patients who received a renal biopsy between 2007 and 2012 that were registered in the Japan Renal Biopsy Registry. Of 623 registered adults with LN from 25 institutions and their affiliated or community hospitals, 489 were eligible for this study. Results: The median age at renal biopsy was 39 years, and 82.2% of patients were female. Renal biopsies were performed in 348 patients with new-onset LN, 106 with relapse LN, and 35 with refractory LN. The distribution of ISN/RPS 2003 Classes was as follows: I 1.6%; II 5.3%; III (± V) 27.0%; IV (± V) 47.0%; V 18.4%; VI 0.6%. During the median observation period of 63.8 months, 36 patients (7.3%) reached a doubling of serum creatinine or end-stage kidney disease (ESKD), and 28 patients (5.7%) died. The 5 year renal and patient survival rates were 93.9% and 94.7%, respectively. Multivariate analysis revealed body mass index (BMI) and estimated glomerular filtration rate (eGFR) were independent risk factors for a doubling of serum creatinine in ESKD. Age and eGFR were independent risk factors for death. Conclusion: Recent prognosis for adults with LN are relatively good in Japan. Risk factors for impaired renal function are BMI and eGFR at renal biopsy, while age and eGFR are risk factors for death. [ABSTRACT FROM AUTHOR]

This document is a correction notice for an article titled "Public support for patients with intractable diseases in Japan: impact on clinical indicators from nationwide registries in patients with autosomal dominant polycystic kidney disease." The correction states that the third author, Tomonori Kimura, was inadvertently left out of the original publication. The corrected article includes the author's affiliation details. The publisher, Springer Nature, remains neutral regarding jurisdictional claims and institutional affiliations. The article is authored by multiple individuals and covers various aspects of the topic. [Extracted from the article]

Background: Practice patterns and bleeding complications of percutaneous native kidney biopsy (PNKB) have not recently been investigated and the Japanese Society of Nephrology performed a nationwide questionnaire survey in 2018. Methods: The survey consisted of nine sections about PNKB: (1) general indications; (2) indications for high-risk patients; (3) informed consent; (4) pre-biopsy evaluation; (5) procedures; (6) sedation; (7) post-biopsy hemostasis, bed rest, and examinations; (8) bleeding complications; and (9) specimen processing. A supplementary survey examined bleeding requiring transcatheter arterial embolization (TAE). Results: Overall, 220 directors of facilities (nephrology facility [NF], 168; pediatric nephrology facility [PF], 52) completed the survey. Indications, procedures, and monitoring protocols varied across facilities. Median lengths of hospital stay were 5 days in NFs and 6 days in PFs. Gauge 14, 16, 18 needles were used in 5%, 56%, 33% in NFs and 0%, 63%, 64% in PFs. Mean limits of needle passes were 5 in NFs and 4 in PFs. The bed rest period was 16–24 h in 60% of NFs and 65% of PFs. Based on 17,342 PNKBs, incidence rates of macroscopic hematuria, erythrocyte transfusion, and TAE were 3.1% (NF, 2.8%; PF, 6.2%), 0.7% (NF, 0.8%; PF, 0%), and 0.2% (NF, 0.2%; PF, 0.06%), respectively. Forty-six percent of facilities processed specimens all for light microscopy, immunofluorescence, and electron microscopy, and 21% processed for light microscopy only. Timing of bleeding requiring TAE varied among PNKB cases. Conclusion: Wide variations in practice patterns of PNKB existed among facilities, while PNKBs were performed as safely as previously reported. [ABSTRACT FROM AUTHOR]

Background: Hyperuricemia is a known risk factor for end-stage renal disease. Although xanthine oxidase (XO) inhibitors are expected to protect the kidney function, evidence to this end is insufficient at present. Methods: This study was a multi-center, open-labeled, randomized study conducted in Mie Prefecture in Japan. Patients were included if they were between 20 and 80 years old and had a serum uric acid (sUA) level ≥ 7.0 mg/dl with or without gout, estimated glomerular filtration rate (eGFR) of 15–60 ml/min/1.73 m2, and urinary protein creatinine ratio (uPCR) of 0.15–3.5 g/gCr. Patients were randomly assigned to a Topiroxostat or Febuxostat group, and the treatment target for the sUA level was < 6.0 mg/dl. The primary outcome was the change in the uPCR after 24 weeks. Results: The change in the median uPCR after 24 weeks was not statistically significant after treatment in the Topiroxostat or Febuxostat group (0.05 g/gCr and − 0.04 g/gCr, respectively). However, the sUA levels decreased significantly in both groups (Topiroxostat group: 8.6 ± 1.1 at baseline to 6.0 ± 1.1 mg/dl at 24 weeks, Febuxostat group: 8.4 ± 1.1 mg/dl at baseline to 5.9 ± 1.3 mg/dl at 24 weeks). No significant change in the eGFR after 24 weeks was noted in either the Topiroxostat or Febuxostat group (− 0.04 ± 4.59 ml/min/1.73 m2 and 0.31 ± 4.70 ml/min/1.73 m2, respectively). Conclusions: In this study, XO inhibitors did not significantly reduce the uPCR in chronic kidney disease stage 3 and 4 patients with hyperuricemia. [ABSTRACT FROM AUTHOR]

Renal biopsy is essential for the diagnosis of kidney diseases, but complications, particularly bleeding incidents, remain problematic. To evaluate the frequency of renal biopsy complications, and to reveal clinical and laboratory factors associated with overt bleeding complications, focusing on those available at hospital ward, we conducted a retrospective observational study for the period between 2001 and 2005 at Mie University Hospital in patients who underwent percutaneous renal biopsy of a native kidney. Of a total of 323 patients, 317 met the inclusion criteria. Only one patient (0.3%) required blood transfusion or intervention to stop bleeding. The mean decrease in hemoglobin (Hb) after biopsy was 0.43 ± 0.7 g/dL. Hb decreased ≥1.0 g/dL in 66 patients (20.8%) and ≥10% in 32 patients (10.1%). On ultrasonography, perirenal hematoma was detected immediately after biopsy in 273 patients (86.1%), and 41 patients (12.9%) showed hematoma ≥2 cm in width. Analgesics were required for back pain in 67 patients (21.1%). Vasovagal response developed in 31 patients (9.8%). Macrohematuria occurred in 12 patients (3.8%). Urinary catheter was used in 161 patients (50.8%). For Hb decrease ≥10% after biopsy, multivariate analysis revealed perirenal hematoma (≥2 cm) as a significant factor. Other significant factors were prolonged international normalized ratio of prothrombin time, elevated blood pressure on hospital admission, older age, increased serum creatinine level, and steroid use. Perirenal hematoma ≥2 cm on ultrasonography immediately after biopsy might well represent a predictive factor for bleeding complications. [ABSTRACT FROM AUTHOR]

The corrected text is given below: According to the questionnaire survey results that were performed for the publication of this book, among 15,657 kidney biopsy cases that were performed in Japan, gross hematuria after kidney biopsy was found in 431 patients (2.8%), bladder wash was in 56 cases (0.4%), red blood cell transfusion was in 121 cases (0.8%), renal arterial embolization was in 31 cases (0.2%), and death occurred in one case (0.006%). Correction to: Clinical and Experimental Nephrology (2021) 25:325-364 https://doi.org/10.1007... In the original publication of the article, the author found errors in the values under the title "Chapter 7: Complications". [Extracted from the article]

In the original publication, some errors have been found in the alignment of Table 9. The corrected table is given below [ABSTRACT FROM AUTHOR]

Background and Objectives: The International Society of Renal Nutrition and Metabolism (ISRNM) has recently recommended the use of the term "protein-energy wasting" (PEW). PEW is a state of malnutrition with decreased body stores of protein and energy fuel in hemodialysis patients and is known as a risk factor for morbidity and mortality. We examined the prevalence of PEW and the characteristics of PEW patients in a hemodialysis center in Japan.Methods and Study Design: Fifty-nine outpatients undergoing maintenance hemodialysis at Iga City General Hospital were evaluated. We observed their biochemical data, body composition, dietary intake, and the number of steps prospectively. PEW was defined according to ISRNM criteria.Results: Nine patients (15% of total) were diagnosed as having PEW. Among indicators of PEW criteria, the relevance ratios of "reduced muscle mass" and "unintentional low dietary energy intake" were significantly higher in PEW than in non-PEW. The number of steps was lower, and serum levels of glucose and C-reactive protein were higher in PEW.Conclusion: About 15% of Japanese hemodialysis patients are estimated to have PEW. Our results suggested that major contributing factors to PEW were reduced muscle mass, unintentional low dietary energy intake, lower amount of exercise, insulin resistance, and chronic inflammation. [ABSTRACT FROM AUTHOR]

Background & Aims: In dialysis patients, malnutrition is a poor prognostic factor. In patients with chronic kidney disease (CKD), malnutrition is qualitatively different from general malnutrition, which is defined as "Protein-Energy Wasting (PEW)." Dietary therapy for the enhancement of PEW requires the aggressive intake of protein. Conversely, as protein intake and phosphorus intake correlate positively, increasing the protein intake increases the phosphorus intake, which is a poor prognostic factor in dialysis patients. One of the treatments for hyperphosphatemia in dialysis patients is the intake restriction of phosphorus by dietary counseling. However, protein uptake to maintain and augment the nutritional status and the protein intake restriction to correct hyperphosphatemia are contradictory treatments. Hence, this study aims to investigate the effects of PEW and hyperphosphatemia on the prognosis in hemodialysis patients., Methods: We enrolled 60 outpatients who underwent maintenance hemodialysis for 6 months (May-November 2012) at Iga City General Hospital (Mie, Japan). In November 2012, we assessed the presence or absence of PEW and hyperphosphatemia in patients and evaluated the survival rate over the next 5 years., Results: Overall, 10 patients (17%) were diagnosed as PEW. While 17 patients (28%) exhibited average phosphorus level >6.0 mg/dL (hyperphosphatemia). The 5-year survival rate was 30% in the PEW group, 66% in the non-PEW group, 57% in the hyperphosphatemia group, and 61% in the non-hyperphosphatemia group. A statistically significant difference existed between the PEW and non-PEW groups (P = 0.021). However, we observed no significant difference between the hyperphosphatemia and non-hyperphosphatemia groups., Conclusions: This study suggests that PEW affects the prognosis more than hyperphosphatemia in maintenance hemodialysis patients. The normalization of the serum phosphorus level by the protein intake restriction could prevent secondary hyperparathyroidism and vascular calcification. Conversely, restricting the protein intake poses a risk of malnutrition. In fact, early death occurred in patients with PEW in this study. Perhaps, patients with PEW should prioritize improving their nutritional status rather than controlling the serum phosphorus level., Competing Interests: Declaration of Competing Interest All authors declare no conflict of interest., (Copyright © 2020 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

The composition of the human gut microbiota is related to host health, and it is thought that dietary habits may play a role in shaping this composition. Here, we examined the population size and prevalence of six predominant bacterial genera and the species compositions of genus Bifidobacterium (g-Bifid) and Bacteroides fragilis group (g-Bfra) in 42 healthy Belgian adults by quantitative PCR (qPCR) over a period of one month. The population sizes and prevalence of these bacteria were basically stable throughout the study period. The predominant g-Bifid species were Bifidobacterium adolescentis and Bifidobacterium longum ss. longum, and the predominant g-Bfra species were Bacteroides vulgatus, Bacteroides uniformis, and Bacteroides ovatus. The Belgian gut microbiota data were then compared with gut microbiota data from 46 Japanese subjects collected according to the same protocol (Matsuki et al., Appl. Environ. Microbiol. 70, 167-173, 2004). The population size and prevalence of Bifidobacterium catenulatum group were significantly lower in the Belgian gut microbiota than in the Japanese gut microbiota (P < 0.001); however, the population size and prevalence of g-Bifid did not differ. This species-level qPCR analysis will be helpful for investigating the diversity of gut microbiota among ethnic groups., (Copyright © 2013 The Society for Biotechnology, Japan. Published by Elsevier B.V. All rights reserved.)

Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Approximately 10% of cases are classified as atypical due to the absence of Shiga toxin-producing bacteria as a trigger. Uncontrolled activation of the complement system plays a role in the pathogenesis of atypical HUS (aHUS). Although many genetic studies on aHUS have been published in recent years, only limited data has been gathered in Asian countries. We analyzed the genetic variants of 6 candidate genes and the gene deletion in complement factor H (CFH) and CFH-related genes, examined the prevalence of CFH autoantibodies and evaluated the genotype-phenotype relationship in 10 Japanese patients with aHUS. We identified 7 causative or potentially causative mutations in CFH (p.R1215Q), C3 (p.R425C, p.S562L, and p.I1157T), membrane cofactor protein (p.Y189D and p.A359V) and thrombomodulin (p.T500M) in 8 out of 10 patients. All 7 of the mutations were heterozygous and four of them were novel. Two patients carried CFH p.R1215Q and 3 other patients carried C3 p.I1157T. One patient had 2 causative mutations in different genes. One patient was a compound heterozygote of the 2 MCP mutations. The patients carrying mutations in CFH or C3 had a high frequency of relapse and a worse prognosis. One patient had CFH autoantibodies. The present study identified the cause of aHUS in 9 out of 10 Japanese patients. Since the phenotype-genotype correlation of aHUS has clinical significance in predicting renal recovery and transplant outcome, a comprehensively accurate assessment of molecular variation would be necessary for the proper management of aHUS patients in Japan., (Copyright © 2012 Elsevier Ltd. All rights reserved.)