Randomized Prospective Controlled Open-labeled Trial of Cyclosporine with/without Low-dose Oral Corticosteroids in Idiopathic Membranous Nephropathy in Adults with Nephrotic Syndrome.

Objective We conducted a prospective, randomized controlled, open-label study to investigate the efficacy of a combination of cyclosporine and low-dose prednisolone in patients with idiopathic membranous nephropathy (IMN). Methods We recruited biopsy-proven IMN patients with nephrotic syndrome who had never been treated with immunosuppressants. The patients were randomized into 2 groups treated after randomization with cyclosporine (3 mg/kg/day) alone or with an oral low-dose corticosteroid (prednisolone 15 mg/day) for 24 months.

Does old-to-young kidney transplantation rejuvenate old donor kidneys?

Background: The number of older organ donors is increasing due to the aging population. Aged kidneys often face problems such as delayed graft function but previous murine experiments suggested the possibilities of rejuvenation, for example, in a parabiosis setting between old and young mice. To investigate kidney-graft rejuvenation, we compared an old-to-young (O-Y) patient transplantation group and a transplantation group with donors/recipients of approx.

Survey of Japanese nephrologists' use of clinical practice guidelines for rapidly progressive glomerulonephritis.

Background: The guidelines in Japan for the treatment of rapidly progressive glomerulonephritis (RPGN) have been revised; the latest update was released in 2020. We investigated the actual usage of the new guidelines in Japan.

Methods: We distributed a survey electronically to board-certified nephrologists throughout Japan from December 15, 2021 to January 31, 2022.

Cardiorenal damages in mice at early phase after intervention induced by angiotensin II, nephrectomy, and salt intake.

The interconnection of heart performance and kidney function plays an important role for maintaining homeostasis through a variety of physiological crosstalk between these organs. It has been suggested that acute or chronic dysfunction in one organ causes dysregulation in another one, like patients with cardiorenal syndrome. Despite its growing recognition as global health issues, still little is known on pathophysiological evaluation between the two organs.

All reported non-canonical splice site variants in GLA cause aberrant splicing.

Background: Fabry disease is an X-linked lysosomal storage disorder caused by insufficient α-galactosidase A (GLA) activity resulting from variants in the GLA gene, which leads to glycosphingolipid accumulation and life-threatening, multi-organ complications. Approximately 50 variants have been reported that cause splicing abnormalities in GLA. Most were found within canonical splice sites, which are highly conserved GT and AG splice acceptor and donor dinucleotides, whereas one-third were located outside canonical splice sites, making it difficult to interpret their pathogenicity.

Corrigendum to "Detecting MUC1 Variants in Patients Clinicopathologically Diagnosed With Having Autosomal Dominant Tubulointerstitial Kidney Disease"Kidney International Reports, Volume 7, Issue 4, April 2022, Pages 857-866.

[This corrects the article DOI: 10.1016/j.ekir.

A Case of Castleman's Disease during the Long-Term Course of Membranous Nephropathy.

Concomitant with nephrotic syndrome and multicentric castleman's disease (MCD) has only been described in a limited number of small studies and case reports. Among those, none confirmed the renal pathology prior to the onset of MCD, and none of the cases had a history of nephrotic syndrome. A 76 year-old Japanese man visited a nephrologist because of incident nephrotic syndrome.

Prediction of early graft function after living donor kidney transplantation by quantifying the "nephron mass" using CT-volumetric software.

Early renal function after living-donor kidney transplantation (LDKT) depends on the "nephron mass" in the renal graft. In this study, as a possible donor-recipient size mismatch parameter that directly reflects the "nephron mass," the cortex to recipient weight ratio (CRWR) was calculated by CT-volumetric software, and its ability to predict early graft function was examined. One hundred patients who underwent LDKT were enrolled.

Aberrant splicing caused by exonic single nucleotide variants positioned 2nd or 3rd to the last nucleotide in the COL4A5 gene.

Background And Objectives: The evident genotype-phenotype correlation shown by the X-linked Alport syndrome warrants the assessment of the impact of identified gene variants on aberrant splicing. We previously reported that single nucleotide variants (SNVs) in the last nucleotide of exons in COL4A5 cause aberrant splicing. It is known that the nucleotides located 2nd and 3rd to the last nucleotides of exons can also play an essential role in the first step of the splicing process.

The efficacy and safety of mizoribine for maintenance therapy in patients with myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis: the usefulness of serum mizoribine monitoring.

Background: The life prognosis of elderly patients with myeloperoxidase-anti-neutrophil cytoplasmic antibodies-associated vasculitis (MPO-AAV) has been improved by reducing the corticosteroid or cyclophosphamide dose to avoid opportunistic infection. However, many elderly MPO-AAV patients experience recurrence and renal death. An effective and safer maintenance treatment method is necessary to improve the renal prognosis of MPO-AAV.

Detecting Variants in Patients Clinicopathologically Diagnosed With Having Autosomal Dominant Tubulointerstitial Kidney Disease.

Introduction: Autosomal dominant tubulointerstitial kidney disease (ADTKD)- is predominantly caused by frameshift mutations owing to a single-base insertion into the variable number tandem repeat (VNTR) region in . Because of the complexity of the variant hotspot, identification using short-read sequencers (SRSs) is challenging. Although recent studies have revealed the usefulness of long-read sequencers (LRSs), the prevalence of variants in patients with clinically suspected ADTKD remains unknown.

Levels of Soluble NKG2D Ligands and Cancer History in Patients Starting Hemodialysis.

Background: Immune dysfunction in hemodialysis patients is partially due to NK cell impairment. Ligands for NK activating receptors such as NKG2D expressed on cancer cells are involved in NK cell dysfunction and can lead to cancer development.

Methods: A cohort with 370 patients who started hemodialysis (HD) was investigated.

Study protocol and baseline characteristics of newly induced dialysis patients: a prospective multi-center cohort study with a biological sample bank, the Ibaraki Dialysis Initiation Cohort (iDIC) study.

Background: Patients with end-stage kidney disease (ESKD) face higher risks of life-threatening events including cardiovascular disease. Various risk factors are identified as agents influencing the life prognosis of ESKD patients. Herein, we evaluated the risk factors related to the outcomes of Japanese patients with dialysis induction.

Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989-2015 survey.

Background: Rapidly progressive glomerulonephritis (RPGN) can progress to end-stage kidney disease within a short period. This study is a continuation of the chronological nationwide survey conducted by the Japan-RPGN working group.

Methods: We examined a total of 2793 RPGN cases registered during four periods (1989-1998, 1999-2001, 2002-2008, 2009-2011) plus 1386 cases in 2012-2015.