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.

Unclassifiable short-rib thoracic dysplasia diagnosed using targeted gene panel sequencing.

We report a case of a fetus with short-rib thoracic dysplasia (SRTD) with polydactyly that also presented with atypical severe acro-mesomelic ossification defects. Genetic analysis using massively parallel sequencing of a skeletal dysplasia panel revealed compound heterozygous variants in DYNC2H1. This clinical report highlights the challenges associated with diagnosing the diverse phenotypes in the SRTD group and emphasizes the importance of genetic surveillance with a targeted gene panel for accurate diagnosis.

FLT3 inhibitors and hematopoietic cell transplantation prolong survival in patients with FLT3-ITD-positive AML.

Acute myeloid leukemia (AML) is an aggressive hematological malignancy with genetic alterations. The FMS-like tyrosine kinase 3 (FLT3) gene is frequently mutated in adult de novo AML, with two types of mutations: internal tandem duplication (ITD) and point mutations in the tyrosine kinase domain. This study aimed to investigate the impact of FLT3 inhibitors and hematopoietic cell transplantation (HCT) on survival outcomes in patients with FLT3-ITD AML in a real-world setting.

Clinical Characteristics of Steatotic Liver Disease Categories in a Large Cohort of Japanese Health Checkup Participants.

Background And Aims: The clinical characteristics and risk factors involved in the development of liver fibrosis in the subtypes of steatotic liver disease (SLD) remain unknown. We examined the clinical characteristics of SLD subtypes using a large Japanese cohort.

Methods: We performed a cross-sectional analysis (total n = 108,446).

Impact of visitation restrictions in neonatal intensive care units during the COVID-19 pandemic on parents in northern Hokkaido, Japan.

Aim: Medical institutions restricted visitation to neonatal intensive care units (NICUs) during the coronavirus disease (COVID-19) pandemic. Therefore, this study aimed to investigate the impact of COVID-19 NICU visitor restrictions on parents.

Methods: We conducted a questionnaire of 378 parents of infants who were hospitalised for more than 1 week at two NICUs in our area and discharged between 1 April 2020 and 31 March 2022.

Comparison of kidney and hepatic outcomes among sodium-glucose cotransporter-2 inhibitors: a retrospective study using multiple propensity scores.

Background: Sodium-glucose cotransporter-2 inhibitors (SGLT2i) have been reported to have effects beyond lowering blood glucose levels, with certain SGLT2i expanding their indications to chronic kidney disease and chronic heart failure. We focused on the hepatoprotective and renoprotective effects of six SGLT2i and assessed whether the effects were unique to each drug or common class effects, in addition to whether the renal and hepatoprotective effects vary based on renal and hepatic status.

Methods: Patients with diabetes (ipragliflozin: 837, empagliflozin: 850, canagliflozin: 922, dapagliflozin: 590, tofogliflozin: 288, and luseogliflozin: 193) who initiated SGLT2i treatment and were monitored for one year were included.

Oral Breathing Facilitates Endoscopic Operability Compared With Nasal Breathing in Peroral Endoscopy: A Randomized Controlled Trial.

Introduction: Unsedated peroral endoscopy, including ultrathin endoscopy (UE) and conventional endoscopy (CE), is feasible in clinical practice but requires improved endoscopic operability and patient tolerance. Currently, the impact of the breathing method on these factors remains unclear. We conducted the first randomized controlled trial comparing oral breathing (OB) and nasal breathing (NB) during both UE and CE to assess their influence.

Risk Factors for Post-Colorectal Endoscopic Submucosal Dissection Bleeding and Efficacy of Carbazochrome Sodium Sulfonate: A Multicenter Retrospective Cohort Study.

Introduction: Carbazochrome sodium sulfonate (CSS) is a hemostatic agent that reduces capillary permeability and enhances capillary resistance. However, its specific effects on colorectal endoscopic submucosal dissection (ESD) outcomes remain uncertain. This study aimed to assess the risk factors for post-ESD bleeding and the effect of CSS on colorectal ESD outcomes.

Evaluation of learning curves for contact laser vaporization of the prostate using the 980 nm diode laser for benign prostatic hyperplasia.

Objectives: We investigated the background of patients who underwent contact laser vaporization of the prostate (CVP) surgery and the learning curve of the operators.

Methods: A total of 207 patients who underwent CVP surgery for benign prostatic hyperplasia between August 2018 and March 2023 were included in this study. Patient background, perioperative results, pre- and postoperative urinary flow tests, and complications were collected retrospectively.

Robot-assisted distal gastrectomy and local resection for gastric cancer and gastrointestinal stromal tumor.

Gastrointestinal stromal tumors surrounding the esophagogastric junction are often challenging to resect, with no consensus regarding the optimal surgical technique. Here in, we present a case of concurrent gastric cancer in the antrum and gastrointestinal stromal tumors adjacent to the esophagogastric junction. The patient underwent simultaneous distal gastrectomy and local resection assisted by a surgical robot, avoiding the need for total gastrectomy.

Unguarded Tricuspid Valve and Pulmonary Atresia With Intact Ventricular Septum Complicated With Right Coronary Artery Fistula and Advanced Atrioventricular Block in a Fetus: A Case Report.

The unguarded tricuspid valve is a rare and severe condition. When found in the fetus, they mostly undergo abortion or intrauterine death. The details of the fetal course in such cases are poorly understood.

Background factors of idiopathic peptic ulcers and optimal treatment methods: a multicenter retrospective Japanese study.

This study investigated the trends in idiopathic peptic ulcers, examined the characteristics of refractory idiopathic peptic ulcer, and identified the optimal treatment. The characteristics of 309 patients with idiopathic peptic ulcer were examined. We allocated idiopathic peptic ulcers that did not heal after 8 weeks' treatment (6 weeks for duodenal ulcers) to the refractory group and those that healed within this period to the healed group.

Characteristics and prognosis of patients with COVID-19 and hematological diseases in Japan: a cross-sectional study.

The Japanese Society of Hematology performed an observational cross-sectional study to clarify the morbidity, prognosis, and prognostic factors in patients with COVID-19 with hematological diseases (HDs) in Japan. The study included patients with HDs who enrolled in our epidemiological survey and had a COVID-19 diagnosis and a verified outcome of up to 2 months. The primary endpoints were characteristics and short-term prognosis of COVID-19 in patients with HDs.

The clinical efficacy and safety of granulocyte and monocyte adsorptive apheresis in patients with Crohn's disease: A multicenter retrospective pilot study.

Introduction: A remission induction therapy of granulocyte and monocyte adsorptive apheresis (GMA) was given to patients with Crohn's disease (CD). However, establishing an appropriate treatment strategy for GMA in patients with CD remains unclear.

Methods: This study evaluated the clinical efficacy and subsequent clinical progression after GMA in patients with CD who underwent GMA in seven independent institutions in Japan from 2010 to 2023.

Standalone Percutaneous Vertebroplasty for Hyperextension Injuries of the Ankylosed Thoracolumbar Spinal Kyphosis.

Hyperextension injuries of the ankylosed thoracolumbar spine, particularly those with preexisting kyphotic deformity, present significant therapeutic challenges. The authors viewed that such injuries without displacement or fractures of the posterior elements are reasonable candidates for standalone percutaneous vertebroplasty (PVP). In such cases, the posterior tension band is spared; thus, fractures are unstable not in the lateral direction, which would lead to the translation of the fracture, but in the vertical direction.

Demonstration of equivocal anti-glomerular basement membrane antibody positivity as a non-specific reaction through multiple immunologic assays in a case of pediatric asymptomatic hematuria.

Background: Anti-glomerular basement membrane (anti-GBM) antibody is essential for the diagnosis of anti-GBM disease. The major epitope consists of the α3 subunits of type IV collagen non-collagenous domain (α 3(IV)NC1). There have been only a few reports of patients false-positive for anti-GBM antibody.

Prevalence and associated metabolic factors of nonalcoholic fatty liver disease in the general population from 2014 to 2018 in Japan: A large-scale multicenter retrospective study.

Aim: The prevalence of nonalcoholic fatty liver disease (NAFLD) is increasing worldwide. The aim of this study was to determine the recent prevalence and clinical characteristics of NAFLD in Japan.

Methods: This study initially included 410 061 retrospectively enrolled adults from the medical health checkup registry for metabolic syndrome, chronic kidney disease, and fatty liver in Japan (MIRACLE-J; UMIN-CTR no.

Phase III study of long-term prognosis of estrogen receptor-positive early breast cancer treated with neoadjuvant endocrine therapy with/without adjuvant chemotherapy.

Purpose: Neoadjuvant endocrine therapy (NET) is a treatment option for estrogen receptor-positive (ER+) postmenopausal early breast cancer (EBC). This phase III trial evaluated the prognosis of EBC patients treated with/without chemotherapy (CT) following NET.

Methods: ER+/HER2-, T1c-2, and clinically node-negative EBC patients were enrolled in 2008-2013 and treated with endocrine therapy (ET) in weeks 24-28.

Novel Calmodulin Variant p.E46K Associated With Severe Catecholaminergic Polymorphic Ventricular Tachycardia Produces Robust Arrhythmogenicity in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes.

Background: CaM (calmodulin) is a ubiquitously expressed, multifunctional Ca sensor protein that regulates numerous proteins. Recently, CaM missense variants have been identified in patients with malignant inherited arrhythmias, such as long QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT). However, the exact mechanism of CaM-related CPVT in human cardiomyocytes remains unclear.

Concomitant pharmacologic medications influence the clinical outcomes of granulocyte and monocyte adsorptive apheresis in patients with ulcerative colitis: A multicenter retrospective cohort study.

Background: Granulocyte and monocyte adsorptive apheresis (GMA) with Adacolumn has been used as a remission induction therapy for patients with active ulcerative colitis (UC). Herein, we investigated the influence of concomitant medications in the remission induction of GMA in patients with active UC.

Methods: This multicenter retrospective cohort study included patients with UC underwent GMA in five independent institutions in Japan from January 2011 to July 2021.

Perioperative Management of Robotic-Assisted Gynecological Surgery in a Super Morbidly Obese Patient.

We report the perioperative management of a 32-year-old woman with super-morbid obesity (body mass index (BMI) of 60.9 kilograms per meter squared (kg/m)) who underwent a robotic-assisted total laparoscopic hysterectomy in a hospital that was not specialized for obese patients. She successfully reduced her BMI by 10% using dietary restrictions in five weeks, and her surgery was performed two weeks later by consultation between gynecologists and anesthesiologists.