Publications by authors named "Hiroki Maruyama"

Background: Artificial intelligence and large language models (LLMs)-particularly GPT-4 and GPT-4o-have demonstrated high correct-answer rates in medical examinations. GPT-4o has enhanced diagnostic capabilities, advanced image processing, and updated knowledge. Japanese surgeons face critical challenges, including a declining workforce, regional health care disparities, and work-hour-related challenges.

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Background: Endoscopic submucosal dissection (ESD) is a minimally invasive treatment for early gastric cancer (GC); however, post-ESD bleeding remains a serious and unpredictable complication. This study aimed to develop machine-learning (ML) models to predict post-ESD bleeding and identify associated risk factors, ensuring accurate and interpretable predictions.

Methods: A retrospective, multicenter clinical database was constructed for patients who underwent ESD for early GC.

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Polystyrene (PS) is widely used because of its transparency, mechanical strength, and ease of production. With rising health concerns, antibacterial PS is increasingly sought after, but few polymer-based antibacterial agents have been prepared to date. In this study, polystyrene was synthesized using a cationic radical initiator, 2,2'-azobis-[2-(1,3-dimethyl-4,5-dihydro-1-imidazol-3-ium-2-yl)] propane triflate (ADIP), and evaluated as an antibacterial additive.

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Article Synopsis
  • A 75-year-old woman was diagnosed with early-stage gastric cancer during an endoscopic examination, specifically a type 0-I carcinoma located on the middle part of her stomach.
  • During the procedure, small whitish structures called white globe appearances (WGAs) were observed near the cancer area, indicating potential subepithelial cancer spread despite the surface appearing flat and noncancerous.
  • Histopathology results confirmed the presence of cancer with significant lymphatic invasion, showing that the cancer had spread along the lymphatic vessels into surrounding tissues, which was likely responsible for the WGA characteristics seen during the endoscopy.
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Fabry disease (FD) is an X-linked disorder resulting in a deficiency of α-galactosidase A (GLA) activity. The R112H mutation of GLA is relatively common in Japanese FD patients, characterized by a late-onset phenotype, almost normal to mild lyso-Gb3 elevation, and mild clinical symptoms, despite low GLA activity. This is due to the structural features of the R112H GLA protein.

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Contact-killing antibacterial materials are attracting attention owing to their ability for sustained antibacterial activity. However, contact-killing antibacterial polystyrene (PS) has not been extensively studied because its chemically stable structure impedes chemical modification. In this study, we developed an antibacterial PS sheet with a contact-killing surface using PS synthesized from 2,2'-azobis-[2-(1,3-dimethyl-4,5-dihydro-1-imidazol-3-ium-2-yl)]propane triflate (ADIP) as a radical initiator with cationic moieties.

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A 71-year-old man presented to our hospital with abdominal pain. He was diagnosed with acute pancreatitis and pancreatic cancer. Peritoneal washing cytology(CY)was positive, and laparotomy findings revealed severe inflammatory changes of pancreatitis, suggesting a high likelihood of the need for combined resection of other organs.

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Fabry disease (FD) is an inherited disease caused by deficient α-galactosidase A activity that is characterized by the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3). Although plasma lyso-Gb3 is a sensitive biomarker of FD, the correlation between its concentration and clinical symptoms remains unclear. To clarify the influence of plasma Gb3 and lyso-Gb3 in a symptomatic FD mouse model, the total contents of Gb3, lyso-Gb3 and their analogs in various organs and plasma were determined in mice with early- (5-week-old) and late-stage (20-week-old) renal dysfunction.

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The application of periodontal tissue in regenerative medicine has gained increasing interest since it has a high potential to induce hard-tissue regeneration, and is easy to handle and graft to other areas of the oral cavity or tissues. Additionally, bone morphogenetic protein-2 (BMP-2) has a high potential to induce the differentiation of mesenchymal stem cells into osteogenic cells. We previously developed a system for a gene transfer to the periodontal tissues in animal models.

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Fabry disease is an X-linked disorder of α-galactosidase A (GLA) deficiency. Our previous interim analysis (1 July 2014 to 31 December 2015) revealed plasma globotriaosylsphingosine as a promising primary screening biomarker for Fabry disease probands. Herein, we report the final results, including patients enrolled from 1 January to 31 December 2016 for evaluating the potential of plasma globotriaosylsphingosine and GLA activity as a combined screening marker.

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Dialysis patients are at increased risk of ischemic colitis and are likely to develop irreversible ischemic colitis. We report a rare case of ischemic colitis after the closure of a temporary ileostomy for low anterior resection(LAR)of rectal cancer in a dialysis patient. A 77-year-old man undergoing maintenance dialysis was diagnosed as having colorectal cancer with a type 2 tumor at the anastomosis site of high anterior resection performed for sigmoid colon cancer 14 years ago.

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Purpose: To assess the utility of globotriaosylsphingosine (lyso-Gb) for clinical monitoring of treatment response in patients with Fabry disease receiving migalastat.

Methods: A post hoc analysis evaluated data from 97 treatment-naive and enzyme replacement therapy (ERT)-experienced patients with migalastat-amenable GLA variants from FACETS (NCT00925301) and ATTRACT (NCT01218659) and subsequent open-label extension studies. The relationship between plasma lyso-Gb and measures of Fabry disease progression (left ventricular mass index [LVMi], estimated glomerular filtration rate [eGFR], and pain) and the relationship between lyso-Gb and incidence of Fabry-associated clinical events (FACEs) were assessed in both groups.

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Low bone mineral density (BMD)-diagnosed as osteoporosis or osteopenia-has been reported as a new characteristic feature of Fabry disease; however, the mechanism underlying the development of low BMD is unknown. We previously revealed that a mouse model of Fabry disease [] exhibits impaired functioning of medullary thick ascending limb (mTAL), leading to insufficient Ca reabsorption and hypercalciuria. Here, we investigated bone metabolism in mice without marked glomerular or proximal tubular damage.

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Fabry disease is a heritable lipid disorder caused by the low activity of α-galactosidase A and characterized by the systemic accumulation of globotriaosylceramide (Gb3). Recent studies have reported a structural heterogeneity of Gb3 in Fabry disease, including Gb3 isoforms with different fatty acids and Gb3 analogs with modifications on the sphingosine moiety. However, Gb3 assays are often performed only on the selected Gb3 isoforms.

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Peliosis hepatis (PH) is a rare disease characterized by the presence of sinusoidal dilation and blood-filled cysts throughout the hepatic parenchyma. We report a case of PH in a 49-year-old woman with chronic renal failure (CRF) on hemodialysis and with renal cell carcinoma (RCC). Dynamic contrast-enhanced computed tomography (CT) showed a 35-mm-diameter, hypervascular tumor in the liver and RCC in the right renal cyst.

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Background: Although previous studies have suggested a certain prevalence of Fabry disease (FD) in left ventricular hypertrophy (LVH) patients, the screening of FD is difficult because of its wide-ranging clinical phenotypes. We aimed to clarify the utility of combined measurement of plasma globotriaosylsphingosine (lyso-Gb3) concentration and α-galactosidase A activity (α-GAL) as a primary screening of FD in unexplained LVH patients.

Methods and results: Between 2014 and 2016, both lyso-Gb3 and α-GAL were measured in 277 consecutive patients (male 215, female 62, age 25-79 years) with left ventricular wall thickness >12 mm on echocardiogram: 5 patients (1.

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The HTML version of this Article contained errors in Supplementary Figure S2 "Flowchart of the lyso-Gb3 screening and gene analysis in female patients", which have been detailed in the associated Correction.

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Article Synopsis
  • A study was conducted to investigate the prevalence of coronary spastic angina (CSA) in patients with Anderson-Fabry disease (AFD), as this connection was previously reported but not quantified.
  • The researchers conducted acetylcholine-induced provocation tests on 9 patients from 5 different AFD families, finding that 8 out of 9 patients (89%) experienced coronary spasms along with related symptoms and ECG changes.
  • The results indicate a significantly high prevalence of CSA among AFD patients, highlighting a potential link that needs further exploration.
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Background: Fabry disease is an X-linked lysosomal storage disorder caused by mutations in GLA, which encodes the enzyme α-galactosidase A (α-Gal A). Although the prevalence of Fabry disease in patients with stroke has been reported to range from 0% to 4%, few cohort studies have examined Japanese stroke patients. We aimed to clarify the prevalence of Fabry disease and the frequency of GLA mutations among patients with young-onset stroke in Japan.

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Article Synopsis
  • - The article discusses the exclusion of a previously diagnosed Fabry disease patient from a study on a positive plasma lyso-Gb3 test due to her already confirmed condition.
  • - The decision to exclude this patient is to ensure the integrity of the study by not including individuals with established Fabry disease or familial connections to it.
  • - The information regarding this exclusion has been accurately revised in the study documentation.
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Alveolar bone is not spontaneously regenerated following trauma or periodontitis. We previously proposed an animal model for new alveolar bone regeneration therapy based on the non-viral BMP-2/7 gene expression vector and in vivo electroporation, which induced the formation of new alveolar bone over the course of a week. Here, we analysed alveolar bone during a period of three weeks following gene transfer to periodontal tissue.

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An enantioselective synthesis of cis-dihydropyrans as the formal HDA reaction products was achieved through the catalyst-controlled anti-selective conjugate addition of aldehydes to β,γ-unsaturated α-keto esters. The observed unusual cis-selectivity could be attributed to the stabilization of the less favorable transition state for anti-conjugate adducts by the hydrogen bonding between the hydroxy group of the amino diol catalyst and β,γ-unsaturated α-keto esters.

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A main feature of Fabry disease is nephropathy, with polyuria an early manifestation; however, the mechanism that underlies polyuria and affected tubules is unknown. To increase globotriaosylceramide (Gb3) levels, we previously crossbred asymptomatic Gla mice with transgenic mice that expressed human Gb3 synthase (A4GALT) and generated the GlaTg(CAG-A4GALT) symptomatic Fabry model mice. Additional analyses revealed that these mice exhibit polyuria and renal dysfunction without remarkable glomerular damage.

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