A novel scoring system for the prediction of disease severity in STEC-HUS.

Background: Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is a life-threatening condition complicated by acute kidney injury, acute respiratory distress syndrome, and central nervous system disorders. The early identification of high-risk patients is required to facilitate timely and appropriate treatment.

Methods: The medical records of patients with STEC-HUS treated at 11 hospitals in Hokkaido, Japan, were reviewed retrospectively.

Effects of canagliflozin on brain natriuretic peptide levels in patients with type 2 diabetes on peritoneal dialysis in Japan: protocol for a multicentre, prospective, randomised controlled trial (CARD-PD trial).

Introduction: Patients with type 2 diabetes (T2D) undergoing dialysis exhibit a higher mortality rate compared with those with other conditions, primarily due to vascular complications including coronary artery disease, heart failure and stroke. Sodium-glucose cotransporter 2 (SGLT2) inhibitors, a type of drug for T2D, have reportedly decreased cardiovascular and renal events in patients with heart failure and chronic kidney disease, irrespective of diabetes presence. Nevertheless, the evidence supporting the use of SGLT2 inhibitors in patients undergoing dialysis has been limited.

Common pathological findings in the heart in COVID-19-related sudden death cases: An autopsy case series.

Background: Cardiomyopathy is a leading cause of sudden out-of-hospital death after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and coronavirus disease 2019 (COVID-19) vaccination. Such unexpected COVID-19-related cardiomyopathies are challenging to diagnose as specific pathological findings are not always identified.

Case Summary: We reported the autopsy findings of two cases of sudden death due to COVID-19-related cardiomyopathies.

Efficacy of cognitive behavioral therapy using self-check sheet for patients with nocturia in real-world clinical practice.

Objectives: We previously demonstrated the efficacy of cognitive behavioral therapy (CBT) using a self-check sheet for patients with nocturia in a randomized controlled study. Additionally, we investigated the efficacy of the intervention in real-world clinical practice.

Methods: Two hundred forty-three outpatients with complaint of nocturia who practiced CBT for 4 weeks using a self-check sheet were included in this trial, which took place from April 2021 to March 2022 in 20 institutions.

Changes in bone turnover markers after discontinuing long-term glucocorticoid administration in children with idiopathic nephrotic syndrome: a multicenter retrospective observational study.

Background: Glucocorticoids affect bone turnover. Little is known about how bone turnover changes when glucocorticoids are discontinued following long-term administration.

Methods: This retrospective observational study was conducted on the relationship between discontinuation of long-term administration of glucocorticoid and bone turnover markers (BTMs) in patients with childhood-onset idiopathic nephrotic syndrome.

Distal arthrogryposis in a girl arising from a novel TNNI2 variant inherited from paternal somatic mosaicism.

TNNI2 at 11p15.5 encodes troponin I2, fast skeletal type, which is a member of the troponin I gene family and a component of the troponin complex. Distal arthrogryposis (DA) is characterized by congenital limb contractures without primary neurological or muscular effects.

siRNA against CD40 delivered via a fungal recognition receptor ameliorates murine acute graft-versus-host disease.

Acute graft-versus-host disease (aGvHD) remains a major threat to a successful outcome after allogeneic hematopoietic stem cell transplantation (HSCT). Although antibody-based targeting of the CD40/CD40 ligand costimulatory pathway can prevent aGvHD, side effects hampered their clinical application, prompting a need for other ways to interfere with this important dendritic T-cell costimulatory pathway. Here, we used small interfering RNA (siRNA) complexed with β-glucan allowing the binding and uptake of the siRNA/β-glucan complex (siCD40/schizophyllan [SPG]; chemical modifications called NJA-312, NJA-302, and NJA-515) into Dectin1+ cells, which recognize this pathogen-associated molecular pattern receptor.

The agreement between measured HbA1c and optimized target HbA1c based on the Dementia Assessment Sheet for Community-based Integrated Care System 8-items (DASC-8): A cross-sectional study of elderly patients with diabetes.

Aim: To investigate the achievement of individualized target HbA1c based on the Japanese guideline after geriatric assessment with the Dementia Assessment Sheet for Community-based Integrated Care System 8-items (DASC-8) and to evaluate patient characteristics acting as barriers to achieving the target HbA1c in elderly outpatients with diabetes.

Methods: This cross-sectional study enrolled 303 Japanese outpatients aged ≥65 years with diabetes. Their health status was measured using the DASC-8.

HOPS-R01 phase II trial evaluating neoadjuvant S-1 therapy for resectable pancreatic adenocarcinoma.

Although neoadjuvant therapy (Nac) is recommended for high-risk resectable pancreatic cancer (R-PDAC), evidence regarding specific regimes is scarce. This report aimed to investigate the efficacy of S-1 Nac for R-PDAC. In a multicenter phase II trial, we investigated the efficacy of Nac S-1 (an oral fluoropyrimidine agent containing tegafur, gimeracil, and oteracil potassium) in R-PDAC patients.

Associations Between Regional Cerebral Blood Flow and Psychiatric Symptoms in Dementia With Lewy Bodies Without Parkinsonism.

Because dementia with Lewy bodies (DLB) has various psychiatric symptoms, early diagnosis in patients without parkinsonism is difficult. To reveal associations between regional brain perfusion and psychiatric symptoms in DLB patients without parkinsonism, we quantified brain perfusion using an automated brain perfusion single-photon emission computed tomography analysis program, FineSRT. We statistically analyzed the differences in brain perfusion between groups, divided by the presence or absence of psychiatric symptoms.

Liver cirrhosis with encapsulating peritoneal sclerosis after 4 years of peritoneal dialysis: A case report.

Rationale: Encapsulating peritoneal sclerosis (EPS), or abdominal cocoon, is a rare but fatal syndrome characterized by intestinal obstruction owing to adhesions in a diffusely thickened peritoneum. Long-term peritoneal dialysis (PD) for more than 5 years is commonly associated with EPS, while liver cirrhosis also carries a risk of EPS. However, there have been only a few reports that describe a case of EPS complicated with both cirrhosis and PD.

Laparoscopic approach for surgical treatment of pleuroperitoneal communication interfering with peritoneal dialysis: a case report.

Background: Pleuroperitoneal communication is a rare disorder that interferes with peritoneal dialysis. Although favorable results of thoracoscopic fistula closure have been reported, there are some cases in which the fistulas cannot be identified by thoracoscopy and the patients are forced to switch to hemodialysis.

Case Presentation: We present two cases of pleuroperitoneal communication in which diaphragmatic fistulas could not be identified thoracoscopically, but could be identified laparoscopically.

gene of isolated from 2016 to 2019 and relationship between genotyping and macrolide resistance in Hokkaido, Japan.

We characterized 515 specimens in Hokkaido. In 2013 and 2014, the gene type 1 strain, mostly macrolide-resistant, was dominant and the prevalence of macrolide resistance was over 50 %. After 2017, the gene type 2 lineage, mostly macrolide-sensitive, increased and the prevalence of macrolide resistance became 31.

A case report: Twin sisters with restrictive cardiomyopathy associated with rare mutations in the cardiac troponin I gene.

Restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children, and its prognosis until now, has been poor. Recently some sarcomeric mutations have been reported as disease-causing genes of RCM. However, the genotype-phenotype correlation is not fully understood.

The effect of rituximab on the quality of life of children with refractory nephrotic syndrome.

Background: Rituximab (RTX) is an effective treatment for maintaining remission in patients with nephrotic syndrome (NS), but there are few reports on the effect of RTX treatment on quality of life (QOL). The purpose of this study was to examine the effect of periodically repeated RTX treatment from the perspective of QOL.

Methods: We systematically assessed the QOL of pediatric patients with refractory NS and parents' perceptions of their children's QOL through a 2 year RTX treatment protocol.

Does cognitive behavioral therapy using a self-check sheet improve night-time frequency in patients with nocturia? Results of a multicenter randomized controlled trial.

Objective: To determine whether cognitive behavioral therapy using a self-check sheet is effective in improving night-time frequency of patients with nocturia.

Methods: We carried out a multicenter, open-labeled, randomized controlled trial in eight institutions. Patients having two or more episodes of nocturia were randomly assigned to either cognitive behavioral therapy with completion of frequency volume charts regularly (cognitive behavioral therapy group) or frequency volume charts regularly alone (frequency volume charts group).

Efficacy and safety of sodium RISedronate for glucocorticoid-induced OsTeoporosis with rheumaTOid arthritis (RISOTTO study): A multicentre, double-blind, randomized, placebo-controlled trial.

Objective: No evidence has shown the efficacy of Sodium Risedronate (Risedronate) for glucocorticoid-induced osteoporosis (GIO) in patients with Rheumatoid arthritis (RA). The aim of this study was to explore the effectiveness and safety of Risedronate for GIO complicated with RA.

Methods: This was a six-month randomized, double-blind, placebo-controlled trial of 95 patients with GIO complicated with RA from 19 centers.

Calcineurin inhibitors for adult-onset Still's disease: a multicentre retrospective cohort study.

Objectives: To clarify the efficacy and safety of calcineurin inhibitors (CNI) for treating adult-onset Still's disease (AOSD).

Methods: This multicentre historical cohort study enrolled the consecutive patients with AOSD according to Yamaguchi classification criteria. The endpoints were set as the time from the initiation of treatment to events, the persistency rate of CNI and safety.