Four-Year Emicizumab Treatment in an Elderly Patient With Acquired Hemophilia A: A Case Report on Perioperative Management Along With a Literature Review.

Acquired hemophilia A (AHA) is a life-threatening bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). While immunosuppressive therapy (IST) can eradicate these autoantibodies, it may fail or cause adverse events, especially in elderly patients. Clinical trials involving AHA patients have confirmed the efficiency of emicizumab, a bispecific antibody mimicking FVIII widely used in congenital hemophilia A with or without inhibitors, but the long-term safety and effectiveness in perioperative hemostatic management remain unclear.

Comparison of genotypes and phenotypes for von Willebrand factor gene variants using Japanese genome database.

von Willebrand disease (VWD) is a common inherited bleeding disorder. The aim of this study was to determine the predicted disease states associated with various pathogenic von Willebrand factor (VWF) variants and their phenotypes using the largest Japanese whole-genome database. Of the 5857 gene variants registered in the Japanese Multi-Omics Reference Panel (jMorp), variants with the following criteria were extracted: (1) caused protein abnormalities due to genetic alterations; (2) have already been detected and included in a database, including known association with VWD; and (3) highly likely pathogenic by in silico analysis.

Perioperative cardiac arrest requiring admission to intensive care units in Japan: epidemiological differences between emergency and elective surgery.

Background: Perioperative cardiac arrest is more prevalent in emergency surgery and carries substantial mortality risks; however, large-scale studies examining its incidence, aetiology, and outcomes remain scarce. This study investigated the epidemiology of perioperative cardiac arrest in emergency and elective surgeries in Japan.

Methods: We conducted a multicentre retrospective cohort study using the Japanese Intensive care PAtient Database (JIPAD), a nationwide ICU registry.

Acquisition of Childcare Skills by a Patient With Transient Paraparesis Following Epidural Anesthesia for Cesarean Section.

For first-time mothers, every aspect of childcare is a new experience, and acquiring these skills can be physically and mentally challenging. It is reasonable to assume that patients with disabilities, including those experiencing lower-limb paraparesis, may face even greater difficulty mastering the physical movements required for childcare while simultaneously striving to regain independence in daily living. We report the case of a 29-year-old woman who developed lower limb paraparesis following an elective caesarean section and describe her recovery in limb function, activities of daily living (ADLs), and childcare skills.

Nutritional and functional profiles of aquaponic leaf lettuce across seasons.

Aquaponics is a sustainable food production system that combines aquaculture and hydroponics. However, limited studies have focused on the nutritional and functional properties of aquaponic produce. Thus, this study aimed to explore the nutritional and functional components of 4 leaf lettuce lines with different colors cultivated in a commercial aquaponic farm in 4 seasons from summer 2022 to spring 2023.

A Case of Varicella Zoster Virus Meningoencephalitis With Generalized Chickenpox-Like Rash.

We report the case of a 91-year-old man with varicella zoster virus (VZV) meningoencephalitis who presented with acute progressive delirium. Brain MRI revealed multiple small vessel lesions, and cerebrospinal fluid testing came back positive for VZV DNA by polymerase chain reaction. We assume that VZV had reactivated since he was elderly and had lung cancer.

Analgesic effect of Keishinieppiittokajutsubu on low barometric pressure-induced pain response in arthritic model rats.

Rheumatoid arthritis (RA) is a disease that causes inflammation of joints, resulting in pain and swelling. Reduced barometric pressure induces painful symptom of RA, but there is no appropriate animal model and pharmacological evaluation. Keishinieppiittokajutsubu (KNEIJB), a Kampo medicine used to treat RA; however, its mechanism of action is not clear.

Visualizing Intraoperative Transcranial Motor-Evoked Potentials During Glioma Surgery for Predicting Postoperative Paralysis Prognosis.

Objective: The primary goals of glioma surgery are maximal tumor resection and preservation of brain function. Intraoperative motor-evoked potential (MEP) monitoring is commonly used to predict and minimize postoperative paralysis. However, studies on intraoperative MEP trends and postoperative paralysis are scarce.

Effects of switching to dolutegravir/lamivudine from tenofovir alafenamide fumarate/emtricitabine/dolutegravir or abacavir/lamivudine/dolutegravir on body weight and lipid profile in Japanese people living with HIV.

Background: The two-drug regimen of dolutegravir/lamivudine (DTG/3TC) is currently an optional antiretroviral therapy (ART). Despite its reported advantages on body weight and lipid profile, the same effects have not yet been reported for Asian population.

Methods: We conducted a single-center retrospective study involving Japanese people living with HIV (PLWH).

The interplay between peptides and RNA is critical for protoribosome compartmentalization and stability.

The ribosome, owing to its exceptional conservation, harbours a remarkable molecular fossil known as the protoribosome. It surrounds the peptidyl transferase center (PTC), responsible for peptide bond formation. While previous studies have demonstrated the PTC activity in RNA alone, our investigation reveals the intricate roles of the ribosomal protein fragments (rPeptides) within the ribosomal core.

A facile access to aliphatic trifluoromethyl ketones photocatalyzed cross-coupling of bromotrifluoroacetone and alkenes.

Biological molecules incorporating trifluoromethyl ketones (TFMKs) have emerged as reversible covalent inhibitors, aiding in the management and treatment of inflammatory diseases, cancer, and respiratory conditions. TFMKs, renowned for their versatile binding properties and adaptability, are pivotal in the rational design of novel drugs for diverse diseases. The photocatalytic insertion of alkenes, abundant feedstocks, into the α-carbon of trifluoromethylacetone represents a highly effective and atom-economical method for synthesizing valuable TFMKs.

Predicting postpartum hemorrhage in women undergoing planned cesarean section: A multicenter retrospective cohort study in Japan.

Given Japan's unique social background, it is critical to understand the current risk factors for postpartum hemorrhage (PPH) to effectively manage the condition, especially among specific groups. Therefore, this study aimed to identify the current risk factors for PPH during planned cesarean section (CS) in Japan. This multicenter retrospective cohort study was conducted in two tertiary maternal-fetal medicine units in Fukushima, Japan and included 1,069 women who underwent planned CS between January 1, 2013, and December 31, 2022.

Mechanisms Associated with Superoxide Radical Scavenging Reactions Involving Phenolic Compounds Deduced Based on the Correlation between Oxidation Peak Potentials and Second-Order Rate Constants Determined Using Flow-Injection Spin-Trapping EPR Methods.

Flow-injection spin-trapping electron paramagnetic resonance (FI-EPR) methods that involve the use of 5,5-dimethyl-pyrroline--oxide (DMPO) as a spin-trapping reagent have been developed for the kinetic study of the O radical scavenging reactions occurring in the presence of various plant-derived and synthetic phenolic antioxidants (Aox), such as flavonoid, pyrogallol, catechol, hydroquinone, resorcinol, and phenol derivatives in aqueous media (pH 7.4 at 25 °C). The systematically estimated second-order rate constants () of these phenolic compounds span a wide range (from 4.

Nucleophilic fluorine substitution reaction of α-carbonyl benzyl bromide, phenylthiofluoroalkyl bromide, and 2-bromo-2-phenoxyacetonitrile.

We herein describe a new method for nucleophilic fluorine substitution of alkylbromides using EtN·3HF. The process is characterized by a broad substrate scope, good functional-group compatibility, and mild conditions and provides a variety of alkylfluorides including tertiary alkylfluorides that are versatile and structurally attractive.

Pioneering cord blood transplantation in relapsed/refractory HIV-related lymphoma: a case study with concurrent intramuscular antiretroviral therapy.

A 44-year-old HIV-positive man diagnosed with diffuse large B-cell lymphoma in 2021 achieved complete remission with six cycles of R-CHOP therapy but had a relapse in November 2022. ESHAP therapy failed to induce remission, leading to complete remission with four cycles of Pola-BR therapy. Post-failure of autologous stem cell harvest, cord blood transplantation (CBT) was performed in June 2023.

Hydropic leiomyoma-like ovarian tumor: a case report.

Uterine leiomyomas, benign tumors common in reproductive-aged women, can display rare variants such as hydropic leiomyoma (HL), which exhibit unique histological features like zonal edema and increased vascularity. However, due to its rarity, comprehensive clinical knowledge about HL is limited. We report a case of a 49-year-old Japanese woman who was premenopausal and nulliparous, presenting with a two-year history of abdominal distension.

Plasma extracellular vesicle microRNAs reflecting the therapeutic effect of the CBP/β-catenin inhibitor PRI-724 in patients with liver cirrhosis.

There is an unmet need for antifibrotic therapies to prevent the progression of liver cirrhosis. Previously, we conducted an exploratory trial to assess the safety and antifibrotic efficacy of PRI-724, a selective CBP/β-catenin inhibitor, in patients with liver cirrhosis. PRI-724 was well tolerated and exerted a potential antifibrotic effect.

Trisomy 12 compromises the mesendodermal differentiation propensity of human pluripotent stem cells.

Trisomy 12 is one of the most frequent chromosomal abnormalities in cultured human pluripotent stem cells (hPSCs). Although potential oncogenic properties and augmented cell cycle caused by trisomy 12 have been reported, the consequences of trisomy 12 in terms of cell differentiation, which is the basis for regenerative medicine, drug development, and developmental biology studies, have not yet been investigated. Here, we report that trisomy 12 compromises the mesendodermal differentiation of hPSCs.

A 6-Month Follow-up Study on Adherence and Satisfaction with Lower Limb Prostheses and Orthoses Delivered to Community-Dwelling People in Japan.

Introduction: We aimed to evaluate adherence and satisfaction with prostheses and orthoses (POs) of the lower extremities delivered to community-dwelling patients and to assess the relationship of adherence and satisfaction with patient background factors, including medical status, physical findings, and level of participation.

Methods: : We conducted a descriptive cohort study. Consecutive patients with disability who applied for lower extremity POs were invited to enroll.

Comprehensive comparison of global coagulation assays to differentiate lupus anticoagulant from acquired hemophilia A in patients with prolonged APTT.

There is no established method for differentiating acquired hemophilia A (AHA) from lupus anticoagulant (LA) positivity because both present with prolonged activated partial thromboplastin time. We compared various parameters of rotational thromboelastometry (ROTEM), thrombin generation assay (TGA), and clot waveform analysis (CWA) in patients with AHA (n = 10) and LA (n = 44). Compared with AHA, possible (n = 12) and definite (n = 32) LA showed significantly shorter clotting time (CT) in NATEM mode of ROTEM (> 3600 vs.

Development and Functional Evaluation of MDR1-expressing Microvascular Endothelial-like Cells Derived from Human iPS Cells as an In vitro Blood-brain Barrier Model.

In order to establish an in vitro model of the human blood-brain barrier (BBB), MDR1-overexpressing human induced pluripotent stem cells (hiPSCs) were generated, and they were differentiated to MDR1-expressing brain microvascular endothelial-like cells (MDR1-expressing hiPS-BMECs). MDR1-expressing hiPS-BMECs monolayers showed good barrier function in terms of tight junction protein expression and trans-epithelial electrical resistance (TEER). In sequential window acquisition of all theoretical fragment ion spectra mass spectrometry (SWATH-MS), MDR1 protein expression was markedly increased in MDR1-expressing hiPS-BMECs, whereas other ABC and SLC transporters showed almost identical expression between MDR1-expressing hiPS-BMECs and mock hiPS-BMECs, suggesting that MDR1 overexpression had little or no knock-on effect on other proteins.

Trophoblast cell surface antigen-2 phosphorylation triggered by binding of galectin-3 drives metastasis through down-regulation of E-cadherin.

The expression of trophoblast cell surface antigen-2 (Trop-2) is enhanced in many tumor tissues and is correlated with increased malignancy and poor survival of patients with cancer. Previously, we demonstrated that the Ser-322 residue of Trop-2 is phosphorylated by protein kinase Cα (PKCα) and PKCδ. Here, we demonstrate that phosphomimetic Trop-2 expressing cells have markedly decreased E-cadherin mRNA and protein levels.

Purification and CryoEM Image Analysis of the Bacterial Flagellar Filament.

The bacterial flagellum is a large assembly of about 30 different proteins and is divided into three parts: the filament that acts as a screw propeller, the hook as a universal joint, and the basal body as a rotary motor. In the case of Salmonella, the filament length is 10-15 μm, which is more than ten times longer than the size of the cell. The filament is composed of only one component protein, flagellin, and is made of 11 protofilaments.

Real-World Use of Albutrepenonacog Alfa, A Recombinant Coagulation Factor IX Albumin Fusion Protein, for Personalized Prophylaxis in Japanese Individuals With Hemophilia B: A Case Series.

Currently, the mainstay of disease management for hemophilia B, a hemorrhagic disease caused by a congenital deficiency or molecular abnormalities of blood coagulation factor IX (FIX), is prophylaxis using FIX concentrate. On-demand injections of FIX concentrate may also be required, even during prophylaxis, when a patient with hemophilia B is bleeding. Albutrepenonacog alfa (rFIX-FP) is a human albumin fusion gene recombinant FIX, which is administered once every seven, 14, or 21 days, depending on patient preferences and symptoms.