174 results match your criteria: "National Hospital Organization Osaka Toneyama Medical Center.[Affiliation]"

Aberrant immune responses to viral pathogens contribute to pathogenesis, but our understanding of pathological immune responses caused by viruses within the human virome, especially at a population scale, remains limited. We analyzed whole-genome sequencing datasets of 6,321 Japanese individuals, including patients with autoimmune diseases (psoriasis vulgaris, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), pulmonary alveolar proteinosis (PAP) or multiple sclerosis) and coronavirus disease 2019 (COVID-19), or healthy controls. We systematically quantified two constituents of the blood DNA virome, endogenous HHV-6 (eHHV-6) and anellovirus.

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Identification of α-galactosylceramide as an endogenous mammalian antigen for iNKT cells.

J Exp Med

February 2025

Department of Molecular Immunology, Research Institute for Microbial Diseases, Osaka University, Suita, Japan.

Article Synopsis
  • Invariant natural killer T (iNKT) cells are unique T cells that recognize lipid antigens through a molecule called CD1d, with α-galactosylceramide (α-GalCer) being the strongest known antigen.
  • Researchers created a specialized system using supercritical fluid chromatography tandem mass spectrometry (SFC/MS/MS) to separate and identify different forms of hexosylceramide.
  • Their findings revealed that α-GalCer, previously not found in mammals, is present in various biological fluids, representing the first identification of this potent antigen in mammalian systems.
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Background: Spinal muscular atrophy (SMA) is a genetic disease characterized by loss of motor neurons in the spinal cord and lower brainstem. The term "SMA" usually refers to the most common form, 5q-SMA, which is caused by biallelic mutations in (located on chromosome 5q13). However, long before the discovery of , it was known that other forms of SMA existed.

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  • The study investigates the combined efficacy of afatinib, an EGFR-tyrosine kinase inhibitor, and bevacizumab, a VEGF inhibitor, in patients with EGFR-mutant non-small cell lung cancer who have become resistant to osimertinib.
  • Out of 28 enrolled patients, the combination treatment resulted in a 17.9% response rate and an overall disease control rate of 78.6%, with a median duration of response of 9 months.
  • The analysis of rebiopsied samples identified various resistance mechanisms, revealing that certain mutations (like C797S and uncommon EGFR mutations) were associated with better responses, while patients with T790M
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Article Synopsis
  • A 23-year-old woman had a long history of fungal infections and started having headaches, leading doctors to analyze her cerebrospinal fluid (CSF), which showed a lot of immune cells.
  • They found a fungus called Candida albicans in her CSF, which caused a type of brain infection called Candida meningitis, and gave her antifungal medication.
  • Unfortunately, she didn't get better and suffered complications, leading to her death, and doctors later found out she had a rare genetic issue known as CARD9 deficiency, which can cause immune problems.
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Background: The optimal subsequent treatment strategy for locally advanced non-small cell lung cancer (LA-NSCLC) after chemoradiotherapy (CRT) and consolidative durvalumab therapy remains unknown. We aimed to determine the optimal subsequent treatment strategy for this clinical population.

Materials And Methods: We retrospectively enrolled 523 consecutive patients with LA-NSCLC treated with CRT and analyzed the treatment outcomes of subsequent therapy after progression following CRT and consolidative durvalumab therapy.

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Background: Several patients treated with osimertinib experience progressive disease. The aim was to clarify the mechanisms underlying resistance to osimertinib.

Methods: ELUCIDATOR: A multi-centre, prospective, observational study involved chemotherapy-naive patients with advanced non-small cell lung cancer receiving osimertinib.

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CD98 heavy chain protein is overexpressed in non-small cell lung cancer and is a potential target for CAR T-cell therapy.

Sci Rep

August 2024

Laboratory of Cellular Immunotherapy, World Premier International Research Center Initiative (WPI), Immunology Frontier Research Center (IFReC), Osaka University, Suita, Osaka, Japan.

Article Synopsis
  • CAR T cell therapy has shown success in treating blood cancers but struggles with solid tumors like non-small cell lung cancer (NSCLC) due to a lack of specific cell surface targets.
  • Researchers identified that CD98 heavy chain protein is overexpressed in NSCLC cells and could serve as a target for CAR T cells.
  • A specific monoclonal antibody called R8H283, which reacts selectively with NSCLC cells without impacting normal tissues, led to the development of CAR T cells that demonstrated significant anti-tumor effects in model studies.
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Article Synopsis
  • * Researchers screened 1555 Japanese IPN patients for CGG repeat expansions using advanced techniques, finding 44 cases with this genetic marker, making it a common cause of the condition.
  • * The findings underscore the importance of tailored screening strategies in clinical settings, particularly for identifying Charcot-Marie-Tooth disease (CMT) cases linked to CGG repeat expansions.
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X-Linked Myotubular Myopathy and Mitochondrial Function in Muscle and Liver Samples.

Neuropediatrics

February 2025

Department of Metabolism, Center for Medical Genetics, Chiba Children's Hospital, Midori-ku, Chiba, Japan.

X-linked myotubular myopathy (XLMTM) is a rare congenital myopathy that commonly manifests with liver involvement. In most XLMTM cases, disease-causing variants have been identified in the myotubularin gene () on chromosome Xq28, which encodes myotubularin protein (MTM1). The impairment of mitochondrial respiratory chain (MRC) enzyme activity in muscle has been observed in the XLMTM mouse model.

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Background: Chemoimmunotherapy is a standard treatment for advanced non-small-cell lung cancer (NSCLC). However, data on clinical predictive factors remain scarce.

Objective: We aim to identify clinical biomarkers in patients undergoing chemoimmunotherapy.

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A workshop of the Special Committee on Measures for Transition from Pediatric to Adult Health Care, the Japanese Society of Neurology was held to discuss various issues and practices involved in healthcare transition. The following points were addressed: (1) the history of, and issues involved in, promoting support for patients requiring medical care, (2) cooperation between pediatric medical centers and university hospitals, (3) collaboration between pediatrics and neurology in medical and rehabilitation facilities, and (4) a questionnaire survey of members of the Japanese Society of Neurology. The reasons for extreme difficulties in pediatric-adult healthcare transition for patients with neurological diseases, especially those who require continuous intensive medical care over a long period of time, include the difference in the operating systems of pediatric and adult departments, in addition to the difference in the diseases treated during childhood and adulthood.

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Background: Measurement of exhaled nitric oxide (FeNO) is a potentially useful diagnostic test for asthma. However, no study has explored the relationship between FeNO and respiratory symptoms of nontuberculous mycobacterial pulmonary disease (NTM-PD) complicated with asthma. The objective of this study was to assess the utility of measuring FeNO levels in patients with NTM-PD complicated by asthma.

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Based on a recent review by Krohn et al, the respiratory center and its regulatory mechanisms are described. Although the respiratory control centers in the medulla and pons ensure rhythmic respiration, maintaining and regulating respiration involves a complex network of peripheral chemoreceptors, vagal nerves, and central chemoreceptors. This review discusses the pathophysiology of respiratory disorders in neuromuscular diseases and evaluation and treatment methods based on the anatomy of the respiratory network.

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The leading cause of death for patients with Duchenne muscular dystrophy (DMD), a progressive muscle disease, is heart failure. Prostaglandin (PG) D, a physiologically active fatty acid, is synthesized from the precursor PGH by hematopoietic prostaglandin D synthase (HPGDS). Using a DMD animal model ( mice), we previously found that HPGDS expression is increased not only in injured muscle but also in the heart.

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Introduction: Pulmonary sarcomatoid carcinoma (PSC) is a rare subtype of lung cancer associated with poor prognosis and resistance to conventional chemotherapy. Immune checkpoint inhibitors (ICIs), alone or in combination with chemotherapy, were found to have clinical benefits in PSC in recent studies. Nevertheless, because these studies included a small number of patients owing to disease rarity, larger studies are needed to evaluate the effectiveness and safety of ICI-based therapy for PSC.

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Introduction: Nivolumab plus ipilimumab with chemotherapy (NICT) and pembrolizumab with chemotherapy (PCT) are commonly used in patients with advanced non-small cell lung cancer (NSCLC). Compared with immune checkpoint inhibitor (ICI) monotherapy, ICI combination therapy can increase immune-related toxicity instead of prolonging survival. This study aimed to compare the efficacy and safety of NICT and PCT to decide on the favorable treatment.

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Article Synopsis
  • - The study investigates the effectiveness and safety of combining bevacizumab with chemotherapy and atezolizumab in advanced non-small cell lung cancer (NSCLC) patients, building on the existing standard treatment protocols.
  • - Conducted as a phase 3 randomized clinical trial at 37 hospitals in Japan, it enrolled patients with advanced nonsquamous NSCLC between January 2019 and August 2020, focusing on those without genetic driver alterations or who had prior treatment with tyrosine kinase inhibitors.
  • - The primary outcome measured was progression-free survival (PFS), assessed by independent reviewers, with a total of 412 patients enrolled in two treatment groups: one receiving the standard regimen and the other receiving the combination with bev
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  • Biomarker testing for driver mutations in non-small cell lung cancer (NSCLC) is crucial for treatment selection, yet current practices in Japan reveal limitations.
  • The REVEAL cohort study involved data collection from 29 institutions, examining 1479 patients diagnosed with advanced or recurrent NSCLC to assess biomarker testing and treatment issues.
  • The study found that while 86.1% of patients had confirmed biomarker status, there were varying positivity rates among different gene tests, indicating challenges in effective treatment decisions based on biomarker testing.
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We report the case of a 55-year-old man presented with a nodule in the right middle lung on computed tomography. The size of the nodule had increased at 12 years after the first visit. Consequently, the patient was diagnosed with a Mixed squamous cell and glandular papilloma (MSGP) by surgical resection.

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Introduction: Necitumumab plus gemcitabine and cisplatin (GCN) is a standard therapy for patients with advanced lung squamous cell carcinoma (LSqCC). However, the efficacy and tolerability of GCN in second-line or later treatment for patients previously treated with immune checkpoint inhibitors (ICIs) remain unknown.

Methods: This multicenter, retrospective, cohort study assessed the efficacy and tolerability of GCN initiated between November 1, 2019 and March 31, 2022 as second-line to fourth-line treatment in patients with advanced LSqCC who had been pretreated with ICIs.

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Introduction: Durvalumab consolidation therapy is the standard of care after concurrent chemoradiotherapy (CRT) for stage III NSCLC. Immune-related pneumonitis during durvalumab treatment is potentially fatal; however, information is lacking regarding the impact of pneumonitis on patient survival. This study investigates the effect of pulmonary and nonpulmonary immune-related adverse events (irAEs) on the efficacy of durvalumab treatment in patients with stage III NSCLC.

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Article Synopsis
  • Osimertinib is the main treatment for advanced lung cancer with EGFR mutations, but its effectiveness in patients over 75 years is not well-studied.
  • A study analyzed 538 patients, showing no significant difference in progression-free survival between elderly (median 16.9 months) and non-elderly (median 22.1 months) patients, though older patients had shorter treatment durations and higher discontinuation rates due to side effects.
  • Despite these challenges, elderly patients had a median overall survival of 30 months, while non-elderly patients' survival data was still ongoing.
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NF-κB is a transcription factor that is activated with aging. It plays a key role in the development of osteoporosis by promoting osteoclast differentiation and inhibiting osteoblast differentiation. In this study, we developed a small anti-NF-κB peptide called 6A-8R from a nuclear acidic protein (also known as macromolecular translocation inhibitor II, Zn2+-binding protein, or parathymosin) that inhibits transcriptional activity of NF-κB without altering its nuclear translocation and binding to DNA.

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