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A π-extended cyclobutenofullerene containing an N,N-dimethylanilinoethynyl group was synthesized via a one-pot cascade reaction of C60 with the corresponding propargylic phosphate. The cyclobutenofullerene was further modified using either one-pot or sequential post-functionalization methods, yielding derivatives containing altered addend structures. During one-pot post-functionalization, hydration reaction of the alkyne moiety continued after the formation of cyclobutenofullerenes. The sequential post-functionalization approach involved introducing the tetracyanobutadiene structure through formal [2 + 2] cycloaddition and a subsequent retroelectrocyclization reaction with tetracyanoethylene. The electronic and optical properties of the derivatives in solution, as well as their field-effect transistor behavior in thin films, were thoroughly assessed to elucidate the optoelectronic differences arising from various addend structures. The properties of the three characteristic cyclobutenofullerene derivatives in the solution and thin films significantly varied depending on the addends. Among the three derivatives studied, only cyclobutenofullerene, featuring a folded structure induced by the hydration of the alkyne moiety, exhibited n-type semiconductor behavior in the thin films. The findings of this study present a novel methodology for synthesizing and functionalizing fullerene derivatives, together with a conceptual framework for tailoring molecular properties., (© 2024 Wiley‐VCH GmbH.)

Mature cystic teratomas (MCTs) of the ovary comprise tissues from all three germ layers. The coexistence of the complete intestinal tract, prostatic tissue, and bladder component within the same ovarian MCT is unprecedented. Here, we report the diagnosis and management of such a rare case. A 26-year-old woman presented with a right ovarian mass, which was later confirmed as an MCT by histopathological examination. The patient underwent a successful laparoscopic cystectomy with no evidence of malignancy or postoperative complications. Histological examination revealed that this MCT contained the complete organ structures including a lower intestinal tract and male genital tract with prostate, urethra, and bladder components, which is unusual. This case underscores the importance of understanding the pathogenesis of extensive organogenesis in MCTs and raises questions about the differentiation processes leading to such unique presentations., Competing Interests: Declarations Conflict of Interest None. Informed consent The patient provided written consent for the publication of this case report. All personal identifiers have been removed to ensure privacy.Coexistence of Complete Intestinal Tract, Prostatic Tissue, Prostatic Urethra and Bladder., (© 2024. The Author(s).)

Canine histiocytic sarcoma (CHS) is characterized by aggressive biological behavior. In our previous study, ERK and Akt pathways were found to be activated in CHS tissues. Thus, the objective of this study was set to investigate the relationships between the activation status of these pathways and the proliferation of CHS cell lines by examining the effects of single and co-administrations of drugs targeting these pathways. First, we evaluated the changes in cell proliferations and the activations of ERK and Akt pathways after treatments with ERK and Akt-specific inhibitors in CHS cells. Then, these changes after treatments with dasatinib and trametinib were also examined in CHS cells. Inhibitors specific to ERK and Akt pathways successfully inhibited the respective pathways in CHS cell lines. It was also indicated that these pathways were associated with the regulations of proliferations of CHS cells, although the anti-proliferative effect was not necessarily observed by inhibition of Akt pathway alone. Dasatinib and trametinib also showed the inhibitions of Akt and ERK pathway activations, respectively, in CHS cells. However, the anti-proliferative effects of these drugs varied among CHS cell lines, and co-administration showed enhanced anti-proliferative effects in only a part of CHS cell lines. Further studies are needed to investigate the molecular mechanisms associated with the sensitivities to these molecular-targeted drugs in CHS cells., Competing Interests: Conflict of interest statement None of the authors has any financial or personal relationships that could inappropriately influence or bias the content of the paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Purpose: Risk stratification for incidence of major adverse cardiovascular events (MACE) in patients with dialysis-dependent end-stage renal disease (dd-ESRD) is challenging. Moreover, the usefulness of coronary CT angiography (CCTA) is often limited because of high calcification. This study aimed to investigate the prognostic value of comprehensive cardiac CT in patients with dd-ESRD for predicting MACE., Materials and Methods: This retrospective analysis included 92 patients with dd-ESRD who underwent comprehensive cardiac CT. Obstructive coronary artery disease (CAD) was defined by CCTA with > 50% stenosis. Global myocardial blood flow (MBF) and summed stress score (SSS) were obtained through dynamic CTP. Cox regression analysis was used to assess correlation with MACE. Kaplan-Meier curves were used to estimate cumulative event rates, and the global Chi-square test was used to assess the incremental value of dynamic CTP over CCTA., Results: During a median follow-up of 2.3 years, 43 patients experienced MACE. Univariate analysis revealed that presence of obstructive CAD, higher SSS, and lower global MBF were significantly associated with increased risk of MACE. In multivariable analysis, lower global MBF and presence of obstructive CAD were independently associated with MACE (p = 0.02, and p = 0.04, respectively). CCTA and dynamic CTP combination had incremental value over CCTA alone for predicting MACE, respectively (global Chi-square score, 19.3 and 11.7, respectively)., Conclusion: Presence of obstructive CAD on CCTA and lower global MBF on dynamic CTP are independently associated with increased risk of MACE in patients with dd-ESRD. The addition of dynamic CTP to CCTA may improve risk stratification in this population., (© 2024. The Author(s).)

Background: Seizures are commonly reported in patients with myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis (MOG-CCE). However, seizure management during the acute phase has not been established., Case Report: A 9-year-old previously healthy boy presented with fever persisting for approximately 6 days, along with headache and altered consciousness. Plain T2-weighted and fluid-attenuated inversion recovery imaging showed swelling and abnormal hyperintense lesions in the bilateral frontal, parietal, temporal, and insular cortices with left hemisphere predominance. Consciousness disturbance persisted, and focal myoclonic seizures clustered hourly. Seizures were arrested by titrating the thiopental dose but recurred with dose reduction, and the patient exhibited super refractory status epilepticus. Adverse effects due to long-term use of thiopental became apparent. Hence, continuous infusion of ketamine and intrathecal dexamethasone therapy (IT-DEX) was started. After administration of ketamine and IT-DEX, his seizure was arrested promptly. The cerebrospinal fluid and serum at the time of transfer were clear positive for ani-MOG antibody; therefore, the patient was diagnosed with MOG-CCE. The patient received three courses of intravenous methylprednisolone pulse therapy, followed by oral prednisolone gradually tapered over 6 months. He did not experience any relapse for 6 months., Conclusion: In MOG-CCE, some cases may present with super-refractory status epilepticus (SRSE) in the acute phase and be refractory to anti-seizure medication, analogous to febrile infection-related epilepsy syndrome. IT-DEX and continuous infusion ketamine are useful for seizure control in MOG-CCE., Competing Interests: Declaration of competing interest The authors declare no financial relationships relevant to this article. There was no honorarium, grant, or other form of payment to produce the manuscript., (Copyright © 2024. Published by Elsevier B.V.)

Soluble B-cell maturation antigen (sBCMA) is overexpressed on multiple myeloma (MM) cells. We investigated whether sBCMA levels correlated with other myeloma tumor volume indicators and its utility in monitoring oligo-secretory/non-secretory (O-S/Non-S) MM. In 115 patients with newly diagnosed MM, sBCMA was compared with M-protein levels, bone marrow plasma cells (BMPCs), circulating tumor cells (CTCs), and total diffusion volume (tDV; estimated by whole-body diffusion-weighted magnetic resonance imaging) at diagnosis. sBCMA levels increased significantly with International Staging System stage, chromosome 1q21 gain/amplification and CTC levels. sBCMA also correlated strongly with %BMPC (r = 0.65), moderately with tDV (r = 0.55) and paraprotein levels (involved immunoglobulin in IgG and IgA subtypes, r = 0.44 and 0.4; involved free light-chain levels in light-chain-only MM, r = 0.61, all P < 0.05). Longitudinal changes in sBCMA were consistent with disease status in both 17 O-S/Non-S and other secretory MM cases. Furthermore, sBCMA levels increased as early as 6 months pre-relapse in almost all O-S/Non-S relapsed patients. Thus, sBCMA correlates strongly with total tumor volume in MM, as assessed using different modalities. We suggest that sBCMA is useful, not only for monitoring responses in patients with O-S/Non-S MM but also for early relapse detection and prediction., (Copyright © 2024 American Society of Hematology.)

Purpose: To investigate the effectiveness and safety of thoracic endovascular aortic repair (TEVAR) for infectious aortic diseases., Materials and Methods: Patients who underwent TEVAR for infectious aortic diseases including infected thoracic aortic aneurysm, aortobronchial fistula (ABF), and aortoenteric fistula from December 2011 to October 2022 at 4 institutions were retrospectively studied. The primary outcome of the study was overall survival, whereas the secondary outcome was comprehensive adverse events. Comprehensive adverse events were defined as a combination of deaths, aortic events, and infectious adverse events., Results: A total of 28 patients were included in the analysis, with 13 patients having infected thoracic aortic aneurysms, 12 ABFs, and 3 aortoenteric fistulae. Seven patients (25%) underwent additional procedures (abscess drainage, 6 cases; total esophagectomy, 1 case). The mean follow-up period was 30.0 months (SD ± 33.9). The 1-year and 5-year survival rates were 85.7% and 67.9%, respectively. The 1-year and 5-year aorta-related complication-free survival rates were 64.3% and 42.9%, respectively. On univariate analysis, the presence of an ABF was associated with a higher risk of comprehensive adverse events (odds ratio = 11; P = .04)., Conclusions: TEVAR might be a promising treatment for infectious thoracic aortic diseases. Among the infectious pathologies, ABF was considered ominous in terms of late outcomes., (Copyright © 2024. Published by Elsevier Inc.)

Objectives: This study aimed to investigate the relationship between microvascular status in the non-ischemic myocardium and the diagnostic performance of stress dynamic CT perfusion imaging (CTP) in detecting hemodynamically significant stenosis., Materials and Methods: This study included 157 patients who underwent coronary computed tomography angiography (CTA), CTP, and invasive coronary angiography (ICA), including fractional flow reserve (FFR). Hemodynamically significant stenosis was defined by FFR and ICA. A relative myocardial blood flow (MBF) for each myocardial segment was normalized to the highest MBF (remote MBF) among 16 segments., Results: The receiver operating characteristic curve analysis for detecting hemodynamically significant stenosis at the vessel level indicated that patients with lower, intermediate, and higher remote MBF had areas under the curve (AUC) of 0.66, 0.70, and 0.80, respectively, for absolute MBF and AUCs of 0.63, 0.70, and 0.83, respectively, for relative MBF. The optimal cut-off values for absolute MBF were proportional to the levels of remote MBFs, while the ones for relative MBF were more consistent across lower to higher remote MBFs. For the patients with high remote MBF, the relative MBF demonstrated a sensitivity of 69%, specificity of 88%, and accuracy of 85% in detecting hemodynamically significant stenosis., Conclusion: The microvascular status in the non-ischemic myocardium influenced the diagnostic performance of dynamic CTP and threshold values of absolute MBFs, suggesting the potential preference for relative MBF over absolute MBF in clinical settings. Dynamic CTP's quantification of MBF offers the benefit of indicating reliability in ischemia detection relative to microvascular status., Key Points: Question The relationship between microvascular status and diagnostic performance of dynamic CTP imaging has not been fully investigated. Findings The diagnostic performance of dynamic CTP and threshold values of absolute MBF were impacted by microvascular status. Clinical relevance The differences in diagnostic accuracy of dynamic CTP related to varying remote MBF values necessitate a personalized evaluation of myocardial perfusion in dynamic CTP images., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

Purpose: To evaluate the value of Tc-99m-diethylenetriamine-penta-acetic acid-galactosyl human serum albumin ( 99m Tc-GSA) single photon emission computed tomography (SPECT) for assessing liver fibrosis, and to assess its complementary value to other liver function indices such as fibrosis-4 (FIB-4) index and indocyanine green (ICG) clearance test parameters (ICG-R15 and ICG-K)., Procedures: Seventy-eight patients with chronic liver disease and hepatocellular carcinoma who underwent 99m Tc-GSA scintigraphy and other liver function tests including ICG test and FIB-4 index prior to hepatectomy were studied. 99m Tc-GSA imaging was performed with SPECT/CT scanner (Discovery NM/CT 670). Immediately after injection of 99m Tc-GSA, dynamic imaging was performed for 20 min, followed by SPECT data acquisition for 6 min. LHL15 which is a conventional index by 99m Tc-GSA planar images, and liver uptake ration (LUR) was measured from 99m Tc-GSA SPECT images. From the liver resection specimens, the degree of liver fibrosis was graded according to the Ludwig scale (F0-4)., Results: Significant differences in LUR, LHL15, ICG-R15, ICG-K, platelet count and FIB-4 index were found between the F0-3 and F4 liver fibrosis patient groups (P < 0.05). Multivariate logistic regression analysis revealed that LUR and ICG-K were independent factors for identifying severe liver fibrosis (F4). Area under the curve of receiver operating curve analysis for the logistic regression model using LUR and ICG-K was 0.83. In the patient group with higher FIB-4 (≥ 3.16), the diagnostic performance of LUR for detecting severe liver fibrosis was significantly better than LHL15 (AUC: 0.83 vs. 0.75, P = 0.048). In the high FIB-4 index group, the sensitivity and specificity for identifying F4 was 88% and 85%, respectively, with LUR cutoff value of 41.2%., Conclusions: LUR, measured by 99m Tc-GSA SPECT, is a useful indicator for identifying sever liver fibrosis. Particularly in patients with high FIB-4 index (≥ 3.16), LUR can be a valuable indicator to identify severe liver fibrosis with high diagnostic accuracy., (© 2024. The Author(s), under exclusive licence to World Molecular Imaging Society.)

Purpose: To compare two blind source separation (BSS) techniques to principal component analysis and the electrocardiogram for the identification of cardiac triggers in self-gated free-running 5D whole-heart MRI. To ascertain the precision and robustness of the techniques, they were compared in three different noise and contrast regimes., Methods: The repeated superior-inferior (SI) projections of a 3D radial trajectory were used to extract the physiological signals in three cardiac MRI cohorts: (1) 9 healthy volunteers without contrast agent injection at 1.5T, (2) 30 ferumoxytol-injected congenital heart disease patients at 1.5T, and (3) 12 gadobutrol-injected patients with suspected coronary artery disease at 3T. Self-gated cardiac triggers were extracted with the three algorithms (principal component analysis [PCA], second-order blind identification [SOBI], and independent component analysis [ICA]) and the difference with the electrocardiogram triggers was calculated. PCA and SOBI triggers were retained for image reconstruction. The image sharpness was ascertained on whole-heart 5D images obtained with PCA and SOBI and compared among the three cohorts., Results: SOBI resulted in smaller trigger differences in Cohorts 1 and 3 compared to PCA (p < 0.01) and in all cohorts compared to ICA (p < 0.04). In Cohorts 1 and 3, the sharpness increased significantly in the reconstructed images when using SOBI instead of PCA (p < 0.03), but not in Cohort 2 (p = 0.4)., Conclusion: We have shown that SOBI results in more precisely extracted self-gated triggers than PCA and ICA. The validation across three diverse cohorts demonstrates the robustness of the method against acquisition variability., (© 2024 The Author(s). Magnetic Resonance in Medicine published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

Canine histiocytic sarcoma (CHS) is a malignant tumor derived from macrophages and dendritic cells. Since effective chemotherapy is needed for CHS cases, we conducted this prospective study to evaluate the efficacy and adverse events of vincristine treatment as a rescue therapy for this disease. We administered vincristine to nine CHS cases that acquired resistance to lomustine or nimustine. Complete remission was achieved in one dog, partial remission in two dogs, stable disease in five dogs, and progressive disease in one dog. The median progression-free survival was 21 days (range: 7-71 days). Severe adverse effect was observed in one dog (Grade 3 thrombocytopenia). It is essential to establish novel effective treatments for CHS.

Aim: To modify the antibody prevalence in epilepsy (APE) score of children with suspected autoimmune central nervous system disease with seizures., Methods: We retrospectively analysed the cerebrospinal fluid of 157 children (aged 0-18 years) with suspected autoimmune central nervous system disease for antineuronal antibodies in our laboratory from 2016 to 2023. Participants were randomly divided into the development cohort (n = 79, 35 females; median 7 years, SD 4 years 7 months, range 4-11 years) and validation cohort (n = 78, 28 females; median 7 years, SD 4 years 5 months, range 4-12 years). A paediatric antibody prevalence in seizure (PAPS) score was created for one cohort and evaluated in the other. Seven variables were selected through univariate and multivariate analysis to create a PAPS score., Results: One hundred and fifty-seven children who fulfilled the inclusion criteria were enrolled; 49 tested positive for antineuronal antibodies. The sensitivity and specificity of an APE score of 4 and greater were 92% and 22.2% respectively; the sensitivity and specificity of a PAPS score of 2.5 and greater were 83.3% and 77.8% respectively. The area under the curve was 0.832 (95% confidence interval = 0.743-0.921), which was significantly better than that for the APE score (p < 0.001)., Interpretation: The APE score had high sensitivity but low specificity in children. The PAPS score may be useful for determining the need for antineuronal antibody testing., What This Paper Adds: The antibody prevalence in epilepsy (APE) score had high sensitivity but low specificity in children. The sensitivity and specificity of the paediatric antibody prevalence in seizure (PAPS) score were 83.3% and 77.8% respectively. There were significant differences in the area under the curve for the APE and PAPS scores., (© 2024 Mac Keith Press.)

Objectives: This study aims to investigate the efficacy of thoracic endovascular aortic repair (TEVAR) for type B aortic dissection (TBAD) complicated by malperfusion. Methods: This retrospective study included patients who underwent TEVAR for the treatment of TBAD complicated by malperfusion from June 1998 to June 2022 in four institutions. In addition to the common outcomes, including short- and medium-term mortality and morbidity, the preservation of each organ was investigated. Results: A total of 23 patients were included in this analysis. The 30-day mortality was 4% (1/23) of the patients. The overall survival rate was 87% at 1 year. The preservation rate of each organ was 33% (4/12) for the visceral organs, 85% (17/20) for the kidneys, and 100% (18/18) for the legs. Fisher's exact test showed a significant difference in the preservation rate between the viscera and the other organs ( P = 0.018 vs. kidneys, P = 0.0025 vs. legs). It was shown that the survival rate of patients with visceral malperfusion was significantly lower than that of patients with non-visceral malperfusion ( P = 0.006). Conclusion: In terms of mortality, TEVAR showed satisfactory results. The preservation of visceral organs was still challenging even with TEVAR and adjunctive measures., (@ 2024 The Editorial Committee of Annals of Vascular Diseases.)

Background: Coronary artery disease (CAD) and coronary microvascular disease (CMD) are significant contributors to angina pectoris, necessitating reliable diagnostic techniques for effective management. While positron emission tomography has been the non-invasive gold standard for myocardial blood flow (MBF) quantification, stress dynamic CT myocardial perfusion imaging (CTMPI) has emerged as a promising alternative. This study aimed to evaluate the test-retest reproducibility of MBF measurements obtained using dynamic CTMPI., Methods: The study retrospectively analyzed MBF values from two dynamic CTMPI examinations conducted in the same patient cohort (n = 30) to examine the consistency of MBF quantification and the ability to visually detect and grade abnormal perfusion suggesting ischemia between the tests. Global and remote MBF were defined as the mean MBF and the maximum MBF of all segments, respectively., Results: MBF quantification revealed strong linear correlations between the tests (r = 0.89 for global MBF, r = 0.88 for remote MBF, and r = 0.82 for all segments), and intraclass correlation coefficients reflected high agreement between the tests (0.94 for global MBF, 0.93 for remote MBF, and 0.90 for all segments). Bland-Altman plots indicated a negligible mean difference with acceptable limits of agreements between the tests for global MBF, remote MBF, and all segments. Visual assessment of the CTMPI maps for abnormal perfusion suggesting ischemia yielded a good inter-test agreement with a weighted kappa value of 0.80., Conclusion: Dynamic CTMPI can consistently reproduce absolute MBF values and reliably detect myocardial perfusion abnormalities, potentially making it a robust diagnostic tool for evaluating the presence and severity of CAD and CMD., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Author(s).)

Background and Objectives: Lymphaticovenular anastomosis (LVA) is a surgical technique used to alleviate lymphedema by bypassing the lymphatic and venous vessels and facilitating lymphatic fluid drainage. Accurate evaluation of anastomotic patency is crucial for assessing LVA outcomes. Traditional near-infrared fluorescence lymphography has limitations, including fluorescence diffusion in subcutaneous fat and difficulty evaluating areas beneath the dermal backflow. Photoacoustic imaging (PAI) is a potential alternative for high-resolution visualization of lymphatic and blood vessels. We aimed to evaluate the utility of PAI for assessing LVA patency., Methods: Using the LUB0 PAI system, we examined patients who underwent LVA. Imaging was conducted using subcutaneously injected indocyanine green (ICG) to visualize lymphatic vessels., Results: Results showed clear patency in some cases, inability to evaluate it in others, and confirmed occlusion in certain instances., Conclusions: While PAI provides valuable insights, challenges remain, including the potential for ambiguous results from the intermittent nature of lymphatic flow and difficulty visualizing low-ICG-concentration lymphatic vessels. Nonetheless, PAI offers a promising method for detailed 3D evaluation of anastomoses. It may improve surgical outcomes and contribute to future evidence in the field. Further advancements, including real-time video assessment, may enhance the accuracy and reliability of LVA patency evaluation., (© 2024 The Author(s). Journal of Surgical Oncology published by Wiley Periodicals LLC.)

Objectives: Physician's evaluation of interstitial lung disease (ILD) extension with high-resolution computed tomography (HRCT) has limitations such as lack of objectivity and reproducibility. This study aimed to investigate the utility of computer-based deep-learning analysis using QZIP-ILD® software (DL-QZIP) compared with conventional approaches in connective tissue disease (CTD) -related ILD., Methods: Patients with CTD-ILD visiting our Rheumatology Centre between December 2020 and April 2024 were recruited. Quantitative scores, including the percentage of lung involvement in ground-glass opacity (QGG), total fibrotic lesion (QFIB), and overall ILD extension encompassing both QGG and QFIB (QILD), calculated by DL-QZIP, were compared with semiquantitative visual method, employing intraclass correlation coefficients (ICC). We compared the capability of QILD scores to distinguish patients with forced vital capacity (FVC) % <70 in both methods determined by the area under the curve (AUC) by the receiver-operating characteristic curve analysis and DeLong's test., Results: Eighty patients (median age, 66 years; 14 men) were included. Median QGG, QFIB, and QILD scores were 3.45%, 2.19%, and 5.35% using DL-QZIP, and 3.25%, 4.06%, and 8.48% using visual method, respectively. Correlations between DL-QZIP and visual method were 0.75 for QGG, 0.61 for QFIB, and 0.75 for QILD. The AUC of QILD scores for FVC% <70 was significantly higher with DL-QZIP (0.833) compared with visual method (0.660) (p < 0.01)., Conclusion: QZIP-ILD® demonstrates superior capability in distinguishing patients with a radiological scenario correlated to severe physiological impairment, while showing relatively good correlations in quantifying the extent on HRCT compared with conventional method in CTD-ILD., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

The lung is a major dose-limiting organ for radiation therapy (RT) for cancer in the thoracic region, and the clarification of radiation-induced lung damage (RILD) is important. However, there have been few reports containing a detailed comparison of radiographic images with the pathological findings of radiation pneumonitis (RP)/radiation fibrosis (RF). We recently reported the upregulated expression of tenascin-C (TNC), an inflammation-associated extracellular matrix molecule, in surgically resected lung tissue, and elevated serum levels were elevated in a RILD patient. Therefore, we have developed a novel mouse model of partial lung irradiation and studied it with special attention paid to the computed tomography (CT) images and immunohistological findings. The right lungs of mice (BALB/c) were irradiated locally at 30 Gy/1fr, and the following two groups were created. In Group 1, sequential CT was performed to confirm the time-dependent changes in RILD. In Group 2, the CT images and histopathological findings of the lung were compared. RP findings were detected histologically at 16 weeks after irradiation; they were also observed on the CT images from 20 weeks. The immunostaining of TNC was observed before the appearance of RP on the CT images. The findings suggest that TNC could be an inflammatory marker preceding lung fibrosis.

Objective: To develop and validate a scoring system to predict mid-term adverse events after elective thoracic endovascular aortic repair (TEVAR)., Methods: In this multicenter retrospective observational cohort study, 350 patients who underwent elective TEVAR for thoracic aortic disease between January 2008 and December 2021 were analyzed. The primary outcome was the first adverse event occurring within 5 years of the initial TEVAR, which included death during the initial hospitalization, perioperative neurologic complication, stent graft-induced new entry, dissection, rupture, graft infection, and reintervention related to the initial TEVAR. The scoring system was developed using the regression coefficients of the Fine-Gray subdistribution hazard model. Its performance was evaluated using the area under the receiver operating characteristic curve (AUC) for competing risk analysis and internally validated by cross-validation., Results: Eighty-two patients experienced at least 1 adverse event within 5 years of the initial TEVAR. The 5-year cumulative incidence of adverse events was 26% (95% confidence interval, 21%-31%). Female sex, comorbidity score, use of anticoagulants, preoperative aortic diameter at the diaphragm level, proximal oversizing ratio, aortic coverage length, and hybrid procedure were included in the scoring system. The AUC of the scoring system in the internal validation was 0.748 (standard error, 0.009). When the scoring system was validated in patients with intact thoracic aortic aneurysm only, the AUC was 0.780 (standard error, 0.012)., Conclusions: Although external validation is needed, our scoring system may be useful for decision making, especially in patients with an intact thoracic aortic aneurysm., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Aim: To develop standardized diagnostic criteria for 'infection-triggered encephalopathy syndrome (ITES)' and five specific clinical syndromes of ITES., Method: The draft definitions were based on existing criteria, standardized, and discussed by a panel of international experts using nominal group technique over 18 months to achieve consensus. All criteria use the same format: (1) presence of infection/fever; (2) clinical features including encephalopathy; (3) neuroradiological features on magnetic resonance imaging; (4) exclusion of other causes., Results: We first highlighted differences between ITES and infectious and autoimmune encephalitis, which is the most important differential diagnosis. Consensus was achieved to define five specific ITESs: acute encephalopathy with biphasic seizures and late reduced diffusion; acute necrotizing encephalopathy; mild encephalopathy with a reversible splenial lesion; acute fulminant cerebral oedema; and acute shock with encephalopathy and multiorgan failure. Two further conditions that are currently classified as epilepsy syndromes but have similar features to ITES, namely febrile infection-related epilepsy syndrome and hemiconvulsion-hemiplegia-epilepsy syndrome, are also discussed., Interpretation: The consensus definition is expected to improve awareness of this disease concept, provide diagnostic framework, and facilitate future international research and clinical trials., (© 2024 The Author(s). Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

To evaluate the usefulness of low-keV multiphasic computed tomography (CT) with deep learning image reconstruction (DLIR) in improving the delineation of pancreatic ductal adenocarcinoma (PDAC) compared to conventional hybrid iterative reconstruction (HIR). Thirty-five patients with PDAC who underwent multiphasic CT were retrospectively evaluated. Raw data were reconstructed with two energy levels (40 keV and 70 keV) of virtual monochromatic imaging (VMI) using HIR (ASiR-V50%) and DLIR (TrueFidelity-H). Contrast-to-noise ratio (CNR tumor ) was calculated from the CT values within regions of interest in tumor and normal pancreas in the pancreatic parenchymal phase images. Lesion conspicuity of PDAC in pancreatic parenchymal phase on 40-keV HIR, 40-keV DLIR, and 70-keV DLIR images was qualitatively rated on a 5-point scale, using 70-keV HIR images as reference (score 1 = poor; score 3 = equivalent to reference; score 5 = excellent) by two radiologists. CNR tumor of 40-keV DLIR images (median 10.4, interquartile range (IQR) 7.8-14.9) was significantly higher than that of the other VMIs (40 keV HIR, median 6.2, IQR 4.4-8.5, P < 0.0001; 70-keV DLIR, median 6.3, IQR 5.1-9.9, P = 0.0002; 70-keV HIR, median 4.2, IQR 3.1-6.1, P < 0.0001). CNR tumor of 40-keV DLIR images were significantly better than those of the 40-keV HIR and 70-keV HIR images by 72 ± 22% and 211 ± 340%, respectively. Lesion conspicuity scores on 40-keV DLIR images (observer 1, 4.5 ± 0.7; observer 2, 3.4 ± 0.5) were significantly higher than on 40-keV HIR (observer 1, 3.3 ± 0.9, P < 0.0001; observer 2, 3.1 ± 0.4, P = 0.013). DLIR is a promising reconstruction method to improve PDAC delineation in 40-keV VMI at the pancreatic parenchymal phase compared to conventional HIR., (© 2024. The Author(s) under exclusive licence to Society for Imaging Informatics in Medicine.)

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children often requires early immunosuppressive therapy before antibody detection. While various electroencephalogram (EEG) patterns, including extreme delta brushes (EDBs), have been reported in adults, pediatric EEG characteristics remain understudied. This study aims to assist clinicians in identifying severe cases early, potentially improving treatment outcomes through prompt intervention. This retrospective case series examined EEG features influenced by disease severity in children with anti-NMDAR encephalitis. We evaluated six children (1-13 years old; four females, two males) treated at Tokyo Metropolitan Neurological Hospital from January 2007 to January 2023. The severity of autoimmune encephalitis in our patients was assessed using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The literature proposes a severity classification for the CASE score, wherein scores of 0-8 points are categorized as mild, 9-18 points as moderate, and 19-27 points as severe. In our patients, CASE scores ranged from 4 to 25 (median:19). We reviewed acute-phase EEG recordings, including 13 long-term videos and 58 conventional recordings. None of the patients maintained a normal posterior-dominant rhythm, and only one exhibited EDBs. Notably, three patients with higher CASE scores (≥15) displayed abnormal theta-band rhythm during non-REM sleep and prolonged EEG recovery times. Our findings suggest that abnormal theta-band rhythms may serve as a potential acute-phase EEG biomarker for severe anti-NMDAR encephalitis in children., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Purpose: Predicting nonalcoholic fatty liver disease (NAFLD) following pancreaticoduodenectomy (PD) is challenging, which delays therapeutic intervention and makes its prevention difficult. We conducted this study to assess the potential application of preoperative computed tomography (CT) radiomics for predicting NAFLD., Methods: The subjects of this retrospective study were 186 patients with PD from a single institution. We extracted the predictors of NAFLD after PD statistically from conventional clinical and radiomic features of the estimated remnant pancreas and whole liver region on preoperative nonenhanced CT images. Based on these predictors, we developed a machine-learning predictive model, which integrated clinical and radiomic features. A comparative model used only clinical features as predictors., Results: The incidence of NAFLD after PD was 43.5%. The variables of the clinicoradiomic model included one shape feature of the pancreas, two texture features of the liver, and sex; the variables of the clinical model were age, sex, and chemoradiotherapy. The accuracy%, precision%, recall%, F1 score, and area under the curve of the two models were 75.0, 72.7, 66.7, 69.6, and 0.80; and 69.6, 68.4, 54.2, 60.5, and 0.69, respectively., Conclusions: Preoperative CT-derived radiomic features from the pancreatic and liver regions are promising for the prediction of NAFLD post-PD. Using these features enhances the predictive model, enabling earlier intervention for high-risk patients., (© 2024. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

Purpose: To delineate the characteristics of probable antibody-negative pediatric autoimmune encephalitis (probable Ab-negative AE), we compared the clinical features of probable Ab-negative AE to those of major antibody-positive AE., Methods: We retrospectively reviewed the clinical features of 18 patients with probable Ab-negative AE, 13 with anti-N-methyl-D-aspartate receptor encephalitis (NMDARE), and 13 with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Clinical characteristics, neuroimaging findings, treatments, and outcomes were analyzed., Results: The age of onset and length of hospital stay were significantly higher in the NMDARE group than in the other groups ( p  = 0.02 and p  < 0.01). Regarding initial neurological symptoms, acute symptomatic seizures in the probable Ab-negative AE group (67%) were significantly more frequent than in the NMDARE (15%) and MOGAD (23%) groups ( p  < 0.01). Paraclinical evidence of neuroinflammation within 1 month of disease onset revealed that single-photon emission computed tomography (SPECT) detected abnormal alterations in 14/14 (100%), cerebrospinal fluid (CSF) analysis in 15/18 (83%), and magnetic resonance imaging (MRI) in 11/18 (61%) in patients with probable Ab-negative AE. In the probable Ab-negative AE group, seven patients (39%) developed autoimmune-associated epilepsy, whereas one patient (8%) had both NMDARE and MOGAD (not statistically significant, p  = 0.07)., Conclusion: Patients with probable Ab-negative AE exhibited acute symptomatic seizures as initial neurological symptoms significantly more frequently. They developed autoimmune-associated epilepsy more frequently than those with NMDARE and MOGAD, which was not statistically significant. SPECT within 1 month of disease onset might be a valuable surrogate marker of ongoing neuroinflammation and neuronal dysfunction, even in patients with negative MRI findings., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Yamada, Inoue, Kuki, Yamamoto, Fukuoka, Nukui, Okuno, Ishikawa, Amo, Togawa, Sakuma and Okazaki.)

The effect of irradiation during computed tomography (CT) imaging on implantable cardioverter-defibrillators (ICDs) has not been fully evaluated in various settings. The purposes of this study were to evaluate the occurrence of electromagnetic interference (EMI) during CT irradiation in various clinically available ICDs with phantom experiments and to determine the potential risks related to irradiation during CT imaging. Five types of clinically available ICDs from five manufacturers were tested. An ICD was combined with an electrocardiogram (ECG) simulator, mounted in a chest phantom, and subjected to CT imaging. Each ICD was irradiated at the maximal power level (tube voltage, 135 kVp; tube current, 510 mA; rotation time, 1.5 s). EMI was defined as oversensing, ventricular tachycardia/ventricular fibrillation (VT/VF) detection, noise, or shock delivery during CT imaging. For ICDs in which EMI was observed, EMI was then evaluated under 144 different irradiation conditions (tube voltage [four patterns from 80-135 kVp], tube current [six patterns from 50-550 mA], and rotation time [six patterns from 0.35-1.5 s]). Testing was also performed during irradiation at the typical doses in three clinical settings and in two settings with inappropriate irradiation of ICDs due to incorrect setup. Among the five ICDs, a shock was delivered by one ICD manufactured by Medtronic (Minneapolis, MN, USA) due to oversensing during irradiation, which occurred at the maximal power level. No oversensing was observed in other ICDs. In the malfunctioned ICD, oversensing was observed in 134 of 144 irradiation patterns, even at a low power in the ICD. The VF-detection criterion was fulfilled in 20 of 134 tests and was significantly associated with tube voltage, tube current, ration time, and tube voltage × rotation time interaction. Although oversensing was observed in three clinical settings (typical chest CT, CT coronary angiography after coronary artery bypass graft, and dynamic assessment for pleural tumors) and one situation during an incorrect scan range on the chest for head perfusion CT, they were not recognized as tachycardia beats. Oversensing was observed when scans were incorrectly set over the ICD during bolus tracking of contrast-enhanced CT. Maximal power CT imaging induced VT/VF detection and shock delivery in one model of ICD placed in a chest phantom. VT/VF detection was observed when tube voltages were high and irradiation times were longer. Oversensing can occur during inappropriate CT imaging, particularly when slices are positioned over the ICD., Competing Interests: Dr. Dohi has received departmental research grant support from Otsuka Pharmaceutical Co., Ltd., Abbott Medical Japan LLC, Novartis Pharma K.K., Daiichi Sankyo Chemical Pharma Co., Ltd., Takeda Pharmaceutical Co., Ltd., Sumitomo Dainippon Pharma Co., Ltd., Kowa Co., Ltd., Ono Pharmaceutical Co., Ltd., and Shionogi Pharma Co., Ltd., as well as lecture fees from Otsuka Pharmaceutical Co., Ltd., Novartis Pharma K.K., Kowa Co., Ltd., AstraZeneca K.K., Daiichi Sankyo Chemical Pharma Co., Ltd., Nippon Boehringer Ingelheim Co., Ltd., and Bayer Yakuhin, Ltd. Dr. Sakuma has received departmental research grant support from Guerbet Japan Co., Ltd., FUJIFILM Toyama Chemical Co., Ltd., and GE HealthCare Pharma Co., Ltd. Dr. Ichikawa has received research grants from GE Healthcare Japan Co., Ltd. and Nihon Medi-Physics Co., Ltd. These funders played no roles in the design or conduct of the study; in the collection, analysis, or interpretation of the data; or in the preparation, review, or approval of the manuscript. The remaining authors report no conflicts of interest for the published content. No funding information was provided., (Copyright: © 2024 Innovations in Cardiac Rhythm Management.)

Progranulin (PGRN) may have two opposing effects-inflammation and anti-inflammation-in different diseases. Although previous studies have reported that PGRN is involved in liver fibrosis, its involvement in tubulointerstitial fibrosis remains to be fully elucidated. Herein, we investigated these issues using PGRN-knockout (KO) mice treated with unilateral ureteral obstruction (UUO). Eight-week-old male PGRN-KO and wild-type (WT) mice were euthanized 3 and 7 days following UUO, and their kidneys were harvested for histopathological analysis. The renal expression of PGRN was evaluated by immunohistochemical and/or western blot analyses. The renal mRNA levels of markers related to inflammation (Il1b, Tnf, Il6, Ccl2, and Adgre1) and fibrosis (Tgfb1, Acta2, Fn1, and Col1a2) were evaluated using quantitative PCR. Histological changes such as renal tubular atrophy, urinary casts, and tubulointerstitial fibrosis were significantly improved in UUO-KO mice compared with UUO-WT mice. Quantitative PCR revealed that the mRNA expression levels of all inflammation- and fibrosis-related markers were lower in UUO-KO mice than in UUO-WT mice at 3 and/or 7 days after UUO. Moreover, PGRN and GRN protein levels were higher in the kidneys of UUO-WT mice than in mice that did not undergo UUO. Elevated GRN levels associated with excess PGRN levels may be involved in the occurrence of renal inflammation and fibrosis in UUO mice.

Background: Current cardiovascular magnetic resonance sequences cannot discriminate between different myocardial extracellular space (ECSs), including collagen, noncollagen, and inflammation. We sought to investigate whether cardiovascular magnetic resonance radiomics analysis can distinguish between noncollagen and inflammation from collagen in dilated cardiomyopathy., Methods: We identified data from 132 patients with dilated cardiomyopathy scheduled for an invasive septal biopsy who underwent cardiovascular magnetic resonance at 3 T. Cardiovascular magnetic resonance imaging protocol included native and postcontrast T 1 mapping and late gadolinium enhancement (LGE). Radiomic features were computed from the midseptal myocardium, near the biopsy region, on native T 1 , extracellular volume (ECV) map, and LGE images. Principal component analysis was used to reduce the number of radiomic features to 5 principal radiomics. Moreover, a correlation analysis was conducted to identify radiomic features exhibiting a strong correlation (r>0.9) with the 5 principal radiomics. Biopsy samples were used to quantify ECS, myocardial fibrosis, and inflammation., Results: Four histopathological phenotypes were identified: low collagen (n=20), noncollagenous ECS expansion (n=49), mild to moderate collagenous ECS expansion (n=42), and severe collagenous ECS expansion (n=21). Noncollagenous expansion was associated with the highest risk of myocardial inflammation (65%). Although native T 1 and ECV provided high diagnostic performance in differentiating severe fibrosis (C statistic, 0.90 and 0.90, respectively), their performance in differentiating between noncollagen and mild to moderate collagenous expansion decreased (C statistic: 0.59 and 0.55, respectively). Integration of ECV principal radiomics provided better discrimination and reclassification between noncollagen and mild to moderate collagen (C statistic, 0.79; net reclassification index, 0.83 [95% CI, 0.45-1.22]; P <0.001). There was a similar trend in the addition of native T 1 principal radiomics (C statistic, 0.75; net reclassification index, 0.93 [95% CI, 0.56-1.29]; P <0.001) and LGE principal radiomics (C statistic, 0.74; net reclassification index, 0.59 [95% CI, 0.19-0.98]; P =0.004). Five radiomic features per sequence were identified with correlation analysis. They showed a similar improvement in performance for differentiating between noncollagen and mild to moderate collagen (native T 1 , ECV, LGE C statistic, 0.75, 0.77, and 0.71, respectively). These improvements remained significant when confined to a single radiomic feature (native T 1 , ECV, LGE C statistic, 0.71, 0.70, and 0.64, respectively)., Conclusions: Radiomic features extracted from native T 1 , ECV, and LGE provide incremental information that improves our capability to discriminate noncollagenous expansion from mild to moderate collagen and could be useful for detecting subtle chronic inflammation in patients with dilated cardiomyopathy., Competing Interests: Disclosures None.

L-Asparaginase (L-Asp) is often used to induce remission in feline large-cell gastrointestinal lymphoma (LCGIL). However, no study has evaluated the efficacy and adverse events following the initial use of this drug as a first-line treatment in feline LCGIL. We retrospectively reviewed medical records of cats with LCGIL treated with L-Asp to induce remission. This study included 43 cats. The response rate (RR) after the first administration of L-Asp was 37.2% (Complete remission: 7.0%, partial remission: 30.2%). RR was significantly higher in cases with primary gastric lesions (64.3%) than in those with primary intestinal lesions (24.1%) (P=0.018), and it was also higher in cases without anemia (57.1%) than those with anemia (15.0%) (P=0.009). The most common adverse event was hyperammonemia, which occurred in 10 of 12 cases where we could compare plasma ammonia concentrations before and after the first dose of L-Asp. Plasma phosphate concentrations were also significantly increased (P<0.001) within 24 hr after the first dose. Decreased appetite, vomiting, and diarrhea were also observed in five, three, and seven cases, respectively, and Grade 3 or higher gastrointestinal signs were observed as adverse events in three cases. The median overall survival of all cats was 150 days (range, 5-1,065 days), and the median progression-free survival was 104 days (range, 2-978 days). In conclusion, L-Asp was effective to induce remission, and severe adverse events were uncommon in feline LCGIL.

Purpose: To evaluate the amide proton transfer (APT), tumor blood flow (TBF), and apparent diffusion coefficient (ADC) combined diagnostic value for differentiating intracranial malignant tumors (MTs) from benign tumors (BTs) in young patients, as defined by the 2021 World Health Organization classification of central nervous system tumors., Methods: Fifteen patients with intracranial MTs and 10 patients with BTs aged 0-30 years underwent MRI with APT, pseudocontinuous arterial spin labeling (pCASL), and diffusion-weighted imaging. All tumors were evaluated through the use of histogram analysis and the Mann-Whitney U test to compare 10 parameters for each sequence between the groups. The diagnostic performance was evaluated using receiver operating characteristic (ROC) curve analysis., Results: The APT maximum, mean, 10th, 25th, 50th, 75th, and 90th percentiles were significantly higher in MTs than in BTs; the TBF minimum (min) was significantly lower in MTs than in BTs; TBF kurtosis was significantly higher in MTs than in BTs; the ADC min, 10th, and 25th percentiles were significantly lower in MTs than in BTs (all p < 0.05). The APT 50th percentile (0.900), TBF min (0.813), and ADC min (0.900) had the highest area under the curve (AUC) values of the parameters in each sequence. The AUC for the combination of these three parameters was 0.933., Conclusions: The combination of APT, TBF, and ADC evaluated through histogram analysis may be useful for differentiating intracranial MTs from BTs in young patients.

Objective: This study aimed to verify whether tooth extraction before the administration of bone-modifying agents (BMA) was effective in preventing the onset of medication-related osteonecrosis of the jaw (MRONJ)., Materials and Methods: This retrospective study included patients with a history of receiving BMA for cancer treatment. The patients were classified into three groups based on the timing of tooth extraction: no tooth extraction before the onset of MRONJ, tooth extraction before the administration of BMA, and tooth extraction after the administration of BMA. The incidence of MRONJ was compared between the groups. Fisher's exact test and Bonferroni correction were used to test for differences in proportions between the three groups., Results: The total number of subjects was 123. Twenty-four patients (19.5%) developed MRONJ. The incidence rates were 12.3% (10/81), 17.9% (5/28), and 64.3% (9/14) in the non-extraction group, the extraction before BMA administration group, and the extraction after BMA administration group, respectively, showing statistically significant differences between the extraction after BMA administration group and the non-extraction groups and between the extraction after BMA administration group and the extraction before BMA administration group (p < 0.001, p = 0.0049). On the other hand, there was no statistically significant difference in incidence between the non-extraction and the extraction before BMA administration group (p = 0.5274)., Conclusions: Tooth extraction before the administration of BMA is effective in preventing the onset of MRONJ in patients receiving BMA for cancer treatment. Prevention of MRONJ development in patients receiving BMA for cancer treatment contributes to the maintenance of patients' quality of life., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Human metapneumovirus (hMPV) is genetically classified into two major subgroups, A and B, based on attachment glycoprotein (G protein) gene sequences. The A2 subgroup is further separated into three subdivisions, A2a, A2b (A2b1), and A2c (A2b2). Subgroup A2c viruses carrying 180- or 111-nucleotide duplications in the G gene (A2c 180nt-dup or A2c 111nt-dup ) have been reported in Japan and Spain. The coronavirus disease 2019 (COVID-19) pandemic disrupted the epidemiological kinetics of other respiratory viruses, including hMPV. In this study, we analyzed the sequences of hMPV isolates in Tokyo and Fukushima obtained from 2017 to 2022, i.e., before and after the COVID-19 pandemic. Subgroup A hMPV strains were detected from 2017 to 2019, and most cases were A2c 111nt-dup , suggesting ongoing transmission of this clade, consistent with global transmission dynamics. Subgroup B viruses, but not subgroup A viruses, were detected in 2022 after the COVID-19 peak. Phylogenetic analysis showed that the subgroup B viruses were closely related to strains detected in Yokohama from 2013 to 2016, and strains detected in Fukushima in 2019, suggesting the reappearance of local endemic viruses in East Japan.

MicroRNAs (miRNAs) are sequence-specific inhibitors of post-transcriptional gene expression. However, the physiological functions of these non-coding RNAs in renal interstitial mesenchymal cells remain unclear. To conclusively evaluate the role of miRNAs, we generated conditional knockout (cKO) mice with platelet-derived growth factor receptor-β (PDGFR-β)-specific inactivation of the key miRNA pathway gene Dicer. The cKO mice were subjected to unilateral ureteral ligation, and renal interstitial fibrosis was quantitatively evaluated using real-time polymerase chain reaction and immunofluorescence staining. Compared with control mice, cKO mice had exacerbated interstitial fibrosis exhibited by immunofluorescence staining and mRNA expression of PDGFR-β. A microarray analysis showed decreased expressions of miR-9-5p, miR-344g-3p, and miR-7074-3p in cKO mice compared with those in control mice, suggesting an association with the increased expression of PDGFR-β. An analysis of the signaling pathways showed that the major transcriptional changes in cKO mice were related to smooth muscle cell differentiation, regulation of DNA metabolic processes and the actin cytoskeleton, positive regulation of fibroblast proliferation and Ras protein signal transduction, and focal adhesion-PI3K/Akt/mTOR signaling pathways. Depletion of Dicer in mesenchymal cells may downregulate the signaling pathway related to miR-9-5p, miR-344g-3p, and miR-7074-3p, which can lead to the progression of chronic kidney disease. These findings highlight the possibility for future diagnostic or therapeutic developments for renal fibrosis using miR-9-5p, miR-344g-3p, and miR-7074-3p., (© 2024. The Author(s).)

Objectives: Rheumatoid arthritis (RA) related interstitial lung disease (ILD) impacts on the treatment strategy and its prognosis in patients with RA. However, the relationship between RA disease activity and the severity of comorbid ILD has not been fully investigated. This study aimed to investigate the impact of RA disease activity on the severity of comorbid ILD in detail based on currently established visual scoring method along with physiological severity., Methods: Consecutive patients with RA visiting to our Rheumatology Centre between December 2020 and December 2023 were analysed. The radiological severity of ILD was evaluated by averaging the extent of the combined lesion of ground glass opacity, reticulation and honeycombing in 5% increments in six representative high-resolution computed tomography slices ranging from 0% (no involvement) to 100% (all lung fields affected) according to Goh and Walsh's method. Associations between the radiological and physiological severity of ILD and patients' features were investigated using linear regression analysis., Results: Among 124 patients (32 men, 92 women), the median age was 70 years, and the median disease duration was 2.92 years. Radiological severity of ILD was 0% (without ILD) in 107 (86.2%), ILD with extent < 10% in nine (7.2%), ILD with extent ≥10% and < 20% in three (2.4%), ILD with extent ≥20% in five (4.0%). Both disease activity score (DAS)28-erythrocyte sedimentation rate (ESR) (standardized coefficient = 0.199, P = 0.03) and rheumatoid factor titre (standardized coefficient = 0.247, P = 0.01) were significantly associated with the radiological quantitative severity of ILD in multivariate analysis adjusted for age, sex, disease duration, smoking status and anti-citrullinated peptide antibody titre. DAS28-ESR was significantly associated with forced vital capacity% predicted (standardized coefficient = -0.230, P = 0.047)., Conclusions: Disease activity of RA was significantly associated with the severity of RA-ILD both radiologically and physiologically., (© 2024. The Author(s).)

Background: The purpose of this study was to investigate the usefulness of second-generation intra-cycle motion correction algorithm (SnapShot Freeze 2, GE Healthcare, MC2) in improving the delineation and interpretability of coronary arteries in coronary computed tomography angiography (CCTA) compared to first-generation intra-cycle motion correction algorithm (SnapShot Freeze, GE Healthcare, MC1)., Methods: Fifty consecutive patients with known or suspected coronary artery disease who underwent CCTA on a 256-slice CT scanner were retrospectively studied. CCTA were reconstructed with three different algorithms: no motion correction (NMC), MC1, and MC2. The delineation of coronary arteries on CCTA was qualitatively rated on a 5-point scale from 1 (nondiagnostic) to 5 (excellent) by two radiologists blinded to the reconstruction method and the patient information., Results: On a per-vessel basis, the delineation scores of coronary arteries were significantly higher on MC2 images compared to MC1 images (median [interquartile range], right coronary artery, 5.0 [4.5-5.0] vs 4.5 [4.0-5.0]; left anterior descending artery, 5.0 [4.5-5.0] vs 4.5 [3.5-5.0]; left circumflex artery, 5.0 [4.5-5.0] vs 4.5 [3.9-5.0]; all p ​< ​0.05). On a per-segment basis, for both 2 observers, the delineation scores on segment 1, 2, 8, 9, 10, 12 and 13 on MC2 images were significantly better than those on MC1 images (p ​< ​0.05). The percentage of interpretable segments (rated score 3 or greater) on NMC, MC1, and MC2 images was 90.5-91.9%, 97.4-97.9%, and 100.0%, respectively., Conclusion: Second-generation intra-cycle motion correction algorithm improves the delineation and interpretability of coronary arteries in CCTA compared to first-generation algorithm., Competing Interests: Declaration of competing interest There is no industry support for this research project. The authors declare that they have no financial/personal interests or beliefs that would affect the results of this study., (Copyright © 2024 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Membranous nephropathy (MN) is a common cause of nephrotic syndrome in middle-aged and older adults. MN etiology is mainly primary or idiopathic; however, it may also be secondary to infections, drugs, neoplasms, and autoimmune diseases. We present the case of a 52-year-old Japanese man with coexisting nephrotic MN and immune thrombocytopenic purpura (ITP). Renal biopsy revealed glomerular basement membrane thickening with immunoglobulin (Ig) G and complement component 3 deposition. Glomerular IgG subclass analysis revealed predominant IgG4 deposition with weak IgG1 and IgG2 deposition. IgG3 and phospholipase A2 receptor deposits were negative. Upper endoscopy revealed no ulcers, but histological examination demonstrated Helicobacter pylori infection in the gastric mucosa with elevated IgG antibodies. After gastric Helicobacter pylori eradication, the nephrotic-range proteinuria and thrombocytopenia of the patient were markedly improved without initiation of immunosuppressive treatment. Therefore, clinicians should consider the possibility of Helicobacter pylori infection in patients with coexisting MN and ITP. Further studies are required to demonstrate the associated pathophysiological aspects., (© 2023. The Author(s).)

We observed a new SWI finding, "cortical brush sign," that represents prominent venous structures in the cortex of patients with acute cerebral infarct with or without moyamoya disease and cerebral venous thrombosis. The cortical brush sign disappeared on follow-up SWI in all cases. Cortical brush sign may help to understand the pathophysiology of venous structures in the cortex at acute phase.

A 66-year-old man presented with hemolytic anemia, which required frequent blood transfusion, 6 months after surgical repair of an ascending aortic pseudoaneurysm. Hemolysis was attributed to luminal stenosis caused by graft kinking by laboratory test, CT and four-dimensional magnetic resonance imaging. First, an Excluder cuff was placed at the stenotic site under rapid pacing, but it migrated distally. Thereafter a Palmaz XL stent was placed at the stenotic site, which led to resolution of anemia. In this case, a Palmaz XL stent was successfully used to treat hemolytic anemia caused by graft kinking following ascending aortic surgery., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Free-running 5D whole-heart coronary MR angiography (MRA) is gaining in popularity because it reduces scanning complexity by removing the need for specific slice orientations, respiratory gating, or cardiac triggering. At 3T, a gradient echo (GRE) sequence is preferred in combination with contrast injection. However, neither the injection scheme of the gadolinium (Gd) contrast medium, the choice of the RF excitation angle, nor the dedicated image reconstruction parameters have been established for 3T GRE free-running 5D whole-heart coronary MRA. In this study, a Gd injection scheme, RF excitation angles of lipid-insensitive binominal off-resonance RF excitation (LIBRE) pulse for valid fat suppression and continuous data acquisition, and compressed-sensing reconstruction regularization parameters were optimized for contrast-enhanced free-running 5D whole-heart coronary MRA using a GRE sequence at 3T. Using this optimized protocol, contrast-enhanced free-running 5D whole-heart coronary MRA using a GRE sequence is feasible with good image quality at 3T.

Resonance frequency breathing is a technique that involves breathing that maximizes heart rate variability. It is specific to individuals and is determined through a procedure taking approximately 30 min, using a procedure that is often best carried out at specialized medical institutions. This is a physical and time-consuming burden because of hospital visits and measurements, particularly for patients with cancer. Therefore it would be beneficial if a procedure can be found to determine resonance frequency from the patient's physical characteristics, without the need for special assessment procedures. This exploratory cross-sectional study examined the correlation between individual characteristics and resonance frequency in healthy volunteers. Multiple regression analysis was performed with the measured resonance frequency as the target variable and individual characteristic parameters as explanatory variables. The study aims to build an estimation formula for resonance frequency with some of these parameters and assess its validity. In addition, the validity of the formula's applicability to patients with incurable cancers is assessed. A total of 122 healthy volunteers and 32 patients with incurable cancers were recruited as participants. The median resonance frequency of 154 participants was six breaths per min. Sex and height were selected as explanatory variables associated with the measured resonance frequency in the volunteers. The estimation formula for resonance frequency using individual characteristics was 17.90-0.07 × height for men and 15.88-0.06 × height for women. Adjusted R-squared values were 0.55 for men and 0.47 for women. When the measured resonance frequency in patients with incurable cancers was six breaths per minute or less, the resonance frequency estimated by this formula was slightly larger than the measured ones. Information on individual characteristics, such as sex and height, which can be easily obtained, was useful to construct an estimation formula for resonance frequency., (© 2023. The Author(s).)

Hypoxic-ischemic brain injury induces metabolic dysfunction that ultimately leads to neuronal cell death. Astrocytes, a type of glial cell, play a key role in brain metabolism; however, their response to hypoxic-ischemic brain injury is not fully understood. Microglia were removed from murine primary mixed glial cultures to enrich astrocytes. Next, we explored genes whose expression is altered following oxygen-glucose deprivation using a microarray. Microarray analysis revealed that the expression of Nr4a1 and Nr4a3 is markedly increased in astrocyte-enriched cultures after 15 h of oxygen-glucose deprivation. The expression of both Nr4a1 and Nr4a3 was regulated by HIF-1α. At the protein level, NR4A1 was translocated from the nucleus to the cytoplasm following oxygen-glucose deprivation and co-localized with mitochondria in apoptotic cells; however, its localization was restored to the nucleus after reoxygenation. Oxygen-glucose deprivation causes an increase in NR4A1 mRNA in astrocytes as well as its nuclear to cytoplasmic transfer. Furthermore, reoxygenation enhances NR4A1 transcription and promotes its nuclear translocation.

Objective: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) sometimes triggers acute encephalopathy as a serious neurological complication in children. We previously reported the clinico-radiological findings of SARS-CoV-2-associated encephalopathy. The advent of the SARS-CoV-2 omicron variant led to a marked increase in pediatric patients with coronavirus disease 2019 (COVID-19); however, epidemiological changes with acute encephalopathy according to the emergence of SARS-CoV-2 have not yet been documented. Therefore, the present study investigated epidemiological differences in SARS-CoV-2-associated encephalopathy during the BA.1/BA.2 and BA.5 predominant periods and also between SARS-CoV-2-associated and non-SARS-CoV-2-associated encephalopathy., Methods: We conducted a nationwide survey of SARS-CoV-2-associated encephalopathy in Japanese children between June and November 2022. We compared the present results during the BA.5 predominant period and previous findings during the BA.1/BA.2 predominant period. We also compared the clinico-radiological syndromes of encephalopathy between SARS-CoV-2-associated and non-SARS-CoV-2-associated encephalopathy., Results: Although many patients with SARS-CoV-2-associated encephalopathy in the BA.5 predominant period had seizures as their initial symptoms, no significant differences were observed in the clinical features. Patients with SARS-CoV-2-associated encephalopathy had worse outcomes than those with non-SARS-CoV-2-associated encephalopathy (p-value = 0.003). Among 103 patients with SARS-CoV-2-associated encephalopathy, 14 (13.6%) had severe types of acute encephalopathy, namely, encephalopathy with acute fulminant cerebral edema (AFCE) and hemorrhagic shock and encephalopathy syndrome (HSES). Also, 28 (27.2%) patients with SARS-CoV-2-associated encephalopathy had poor outcome: severe neurological sequelae or death. Ninety-five patients (92.2%) were not vaccinated against SARS-CoV-2., Conclusions: In SARS-CoV-2-associated encephalopathy, high percentages of AFCE and HSES can result in poor outcomes., Competing Interests: Declaration of competing interest The authors declare no competing interests., (Copyright © 2023. Published by Elsevier B.V.)