2024 Update of the Japan College of Rheumatology Clinical Practice Guidelines for the Management of Rheumatoid Arthritis - secondary publication.

Objectives: To update the Japan College of Rheumatology Clinical Practice Guidelines for the Management of Rheumatoid Arthritis (CPG for RA).

Methods: The recommendations were developed based on the evidence published until the end of June 2022 using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE). The steering committee, CPG panel, systematic review (SR) group, and SR support team were organised.

Estimated Glucose Disposal Rate predicts the risk of incident Metabolic dysfunction-associated steatotic liver disease.

Objectives: There is a relationship between insulin resistance and metabolic dysfunction-associated steatotic liver disease (MASLD) and the estimated glucose disposal rate (eGDR) has been reported as a surrogate marker of insulin resistance. This study aimed to investigate the association between eGDR and the incident MASLD, and compare the ability to predict incident MASLD with other insulin resistance markers.

Methods: Retrospective cohort data from a health check-up program were analyzed.

Congenital Hypogonadotropic Hypogonadism With Novel Pathogenic Variants in FGFR1 and GNRHR.

Congenital hypogonadotropic hypogonadism (CHH) can cause delayed secondary sexual characteristics and contribute to juvenile osteoporosis, with multiple causative genes having been reported. We treated a 27-year-old man diagnosed with central hypogonadism, presenting with delayed secondary sexual characteristics and juvenile osteoporosis, using bone resorption inhibitors and testosterone therapy. Genetic testing revealed missense variants both in the fibroblast growth factor receptor 1 () and gonadotropin-releasing hormone receptor () genes, a combination that has not been previously reported.

Screening for Predictive Factors of Efficacy of Second-Generation Androgen Receptor Axis-Targeted Agents in Patients With High-Risk Metastatic Hormone-Sensitive Prostate Cancer.

Background: Differences in the effectiveness of second-generation androgen receptor axis-targeted agents (ARATs) in high-risk metastatic hormone-sensitive prostate cancer (mHSPC) remain unclear. This study aimed to identify the factors influencing the efficacy of ARATs in patients with high-risk mHSPC and compare their long-term effectiveness.

Methods: Four hundred and sixty-six patients with mHSPC treated with ARATs were retrospectively recruited from our hospital and affiliated hospitals of the Kindai Oncology Study Group and Kyoto Prefectural University of Medicine Oncology Study Group between December 2013 and March 2024.

Dynamic change of polarity in spread through air spaces of pulmonary malignancies.

Spread through air spaces (STAS) is a histological finding of lung tumours where tumour cells exist within the air space of the lung parenchyma beyond the margin of the main tumour. Although STAS is an important prognostic factor, the pathobiology of STAS remains unclear. Here, we investigated the mechanism of STAS by analysing the relationship between STAS and polarity switching in vivo and in vitro.

Anti-inflammatory effect of Angiotensin 1-7 in white adipose tissue.

Obesity is a global health concern that promotes chronic low-grade inflammation, leading to insulin resistance, a key factor in many metabolic diseases. Angiotensin 1-7 (Ang 1-7), a component of the renin-angiotensin system (RAS), exhibits anti-inflammatory effects in obesity and related disorders, though its mechanisms remain unclear. In this study, we examined the effect of Ang 1-7 on inflammation of white adipose tissue (WAT) in dietary-induced obese mice.

A novel semi-quantitative scoring method for CD8+ tumor-infiltrating lymphocytes based on infiltration sites in gastric cancer.

No established method currently exists for evaluating tumor-infiltrating lymphocytes (TILs) in gastric cancer (GC), and their clinical significance based on infiltration site in GC remains unclear. In this study, we developed a method to evaluate TILs according to their infiltration site as a prognostic marker for GC. We retrospectively analyzed 103 patients with advanced GC who underwent curative resection.

Clinical significance of cachexia index determined by bioelectrical impedance analysis in patients with gastrointestinal cancer.

Cancer cachexia is a complex disorder characterized by skeletal muscle loss, which may influence the prognosis of patients with cancer. The cachexia index (CXI) is a new index for cachexia. The present study aimed to assess whether the CXI determined by bioelectrical impedance analysis (BIA) is valuable for predicting survival in patients with gastrointestinal cancer.

Effectiveness for remission maintenance rate and safety of different rituximab regimens for treating anti-neutrophil cytoplasmic antibody-associated vasculitis in Japan: a J-CANVAS study.

Rituximab (RTX) has been reported to effectively maintain remission in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). In this multicenter study involving 57 patients who achieved remission after 24 weeks, we evaluated the effectiveness of RTX in maintaining remission in patients with AAV. Patients were divided into three groups based on RTX administration: continuous, induction phase-only, and maintenance phase-only groups.

Predictors of cytomegalovirus corneal endotheliitis postcorneal transplantation.

Purpose: This study was designed to investigate risk factors for the development of cytomegalovirus (CMV) corneal endotheliitis following corneal transplantation.

Methods: We retrospectively analysed 1225 corneal transplants for bullous keratopathy between 2011 and 2021. 31 cases who were administered the treatment of CMV corneal endotheliitis preoperatively were excluded, and 1194 cases were analysed for risk factors for the development of CMV corneal endotheliitis following corneal transplantation.

An autopsy case of TFE3-rearranged PEComa-like neoplasm with systematic embolization involving the heart.

A rare autopsy case of malignant transcription factor E3 (TFE3)-rearranged perivascular epithelioid cell tumor (PEComa)-like neoplasm is presented. An 84-year-old woman manifested multiple cerebral infarctions and repetitive embolic events in the supra mesenchymal artery (SMA), and the presence of a mobile mass in the heart's left ventricle was also revealed. Tumoral lesions were also found in a pelvic space and a right pleural cavity, and a biopsy was performed from one of the disseminated tumor masses in the right pleura.

Sex-Dependent Variations in Gene Expression in Corneal Endothelial Cells Among Healthy Individuals and Patients With Fuchs Endothelial Corneal Dystrophy.

Purpose: Fuchs endothelial corneal dystrophy (FECD) displays a higher incidence in females than in males, yet the underlying mechanism remains unclear. This study aimed to elucidate sex-dependent differential gene expressions in corneal endothelial cells (CECs) from healthy non-FECD individuals and from patients with FECD.

Methods: RNA-Seq data from CECs of non-FECD subjects (3 males, 4 females) and FECD subjects (5 males, 5 females) were analyzed to identify differentially expressed genes (DEGs) between the sexes.

Diagnostic Insighhts Into JC Virus-Infected Glial Cells: Intraoperative Cytopathological Analysis of Dot-Shaped Inclusions Associated With PML-NBs.

This is the first case report describing the diagnostic value of dot-shaped inclusions associated with promyelocytic leukaemia nuclear bodies (PML-NBs) to define JC virus-infected glial cells in an intraoperative cytopathological diagnosis for progressive multifocal leukoencephalopathy (PML).

Intrapericardial Pulmonary Vein Ligation to Prevent Stump Thrombosis During Left Upper Lobectomy.

Background: Postoperative cerebral infarction, a serious complication of surgery, is occasionally experienced with pulmonary vein stump thrombosis (PVST), which is frequently observed after left upper lobectomy (LUL). Herein, we prospectively investigated whether PVST could be safely prevented by intrapericardial ligation of the superior pulmonary vein (SPV) to shorten the SPV stump during LUL.

Methods: In a consecutive 21 patients who underwent LUL, we ligated the proximal intrapericardial SPV with 1-0 silk suture and divided the distal hilar SPV by an automatic stapling device.

Bioengineering strategies for regeneration of skin integrity: A literature review.

Objective: The skin is a complex organ that includes various stem cell populations. Current approaches for non-healing skin defects are sometimes inadequate and many attempts have been made to regenerate skin integrity. The aim of this review is to bridge the gap between basic research and clinical application of skin integrity regeneration.

Modified Damus-Kaye-Stansel Anastomosis to Prevent Coronary Obstruction Between the Great Arteries.

The conventional Damus-Kaye-Stansel procedure may cause coronary artery compression when the coronary arteries are situated between the great arteries. We have performed a modified Damus-Kaye-Stansel procedure utilizing a "flap-bridging technique," in which an inverted U-shaped flap incised from the aorta is bridged to the main pulmonary trunk, creating sufficient space between the great arteries, in an 8-month-old boy who was a Fontan candidate with congenitally corrected transposition of the great arteries. This modified approach yielded favorable outcomes without coronary events and can effectively prevent coronary obstruction in cases where the coronary arteries run between the great arteries.

Differences in Phenotypes, Treatments, and Outcomes of ANCA-associated Vasculitis across Europe, Japan, and the United States in 2020.

Objectives: To clarify the differences in clinical phenotypes, therapeutic patterns, and outcomes of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) across geographic regions using a multinational cohort.

Methods: Data were collected from patients with newly diagnosed or relapsing GPA or MPA in Europe, Japan, and the United States (US) from January to July 2020. The composite outcome of kidney failure and/or death within 52 weeks after treatment was evaluated, and the hazard ratios across the regions were estimated using the Cox proportional hazard model.

Sonic Hedgehog signaling regulates the optimal differentiation pace from early-stage mesoderm to cardiogenic mesoderm in mice.

Sonic Hedgehog (Shh), encoding an extracellular signaling molecule, is vital for heart development. Shh null mutants show congenital heart disease due to left-right asymmetry defects stemming from functional anomaly in the midline structure in mice. Shh signaling is also known to affect cardiomyocyte differentiation, endocardium development, and heart morphogenesis, particularly in second heart field (SHF) cardiac progenitor cells that contribute to the right ventricle, outflow tract, and parts of the atrium.

Pre-distance assessment from radial artery to lower extremity arterial lesion.

Endovascular treatment (EVT) for patients with lower extremity artery disease is widely used as a less invasive alternative to surgical bypass. Recently, transradial artery intervention has gained popularity owing to its minimally invasive nature. The distance from the radial artery to the target vessel is critical for success; however, effective pre-assessment methods have not yet been established.

Refeeding syndrome and vitamin B1 deficiency in a young man with normal body mass index following starvation in the COVID-19 era.

COVID-19 has spread worldwide and significantly influenced economies. Refeeding syndrome (RFS) is a potentially fatal abnormalities of electrolytes and fluid that can occur in malnourished patients undergoing mechanical refeeding. Herein, we report the case of a man in his 20s with a normal body mass index who presented with RFS and vitamin B1 deficiency.

The real-world efficacy and safety of frontline therapy of obinutuzumab plus bendamustine for untreated high-tumor-burden follicular lymphoma.

Background: While R-CHOP has been one of the standard therapies for untreated high-tumor-burden (HTB) follicular lymphoma (FL) for over 2 decades, obinutuzumab plus bendamustine (OB) is also currently regarded as the standard of care since its approval in 2018 in Japan; however, the long-term efficacy and safety of OB in the daily clinical practice has not been thoroughly evaluated.

Methods: We conducted a multicenter retrospective study for the clinical outcome of 53 patients with HTB FL treated by OB as the frontline therapy between 2018 and 2021 in the Kyoto Hematology Clinical Study Group (KOTOSG). All patients had at least 2-year follow-up period.

Plantar cutaneous sensation is independently associated with postural balance and lower limb motor function in older adults: the Shizuoka study.

Purpose: A relationship between decreased plantar cutaneous sensation and impaired balance function has been reported in patients with peripheral neuropathy and diabetes. This cross-sectional study aimed to investigate the relationship between plantar sensation and postural balance, as well as the association between plantar sensation and sarcopenia-related motor function in community-dwelling older adults.

Methods: The participants included 1659 community-dwelling older adults with a mean age of 74 ± 5 years, of which 43% were male patients.

Raman Spectroscopic Analysis of Molecular Structure and Mechanical Properties of Hypophosphatasia Primary Tooth.

Mild hypophosphatasia (HPP) can be difficult to distinguish from other bone disorders in the absence of typical symptoms such as the premature loss of primary teeth. Therefore, this study aimed to analyze the crystallinity of hydroxyapatite (HAp) and the three-dimensional structure of collagen in HPP teeth at the molecular level and to search for new biomarkers of HPP. Raman spectroscopy was used to investigate the molecular structure, composition, and mechanical properties of primary teeth from healthy individuals and patients with HPP.