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Purpose: The traditional method for measuring left atrial volume (LAV) involves manual tracing. Recently, semi-automated techniques for measuring LAV, based on 2D speckle tracking echocardiography (STE) and 3D echocardiography (3DE), have become commercially available. This study aimed to investigate the efficiency and feasibility of these semi-automated software methods for LAV measurement in pediatric patients., Methods: We analyzed 207 pediatric patients with 2D and 3D echocardiographic images of the left atrium. The maximum LAV was measured using three techniques: (1) manual tracing, (2) STE-based semi-automated measurement, and (3) 3DE-based semi-automated measurement. We compared both LAV and the time required for LAV measurement among these three techniques. Intra- and inter-observer reproducibility of the LAV measurements was assessed using the intraclass correlation (ICC)., Results: There was no difference in the LAV between the manual tracing and the STE-based method, but the LAV measured by 3DE-based method was slightly smaller than manual tracing. The measurement time was 32.6 ± 3.5, 53.8 ± 10.8, and 33.8 ± 13.0 s for manual tracing, STE-based, and 3DE-based techniques, respectively. There was no difference the time for LAV measurement between the manual tracing and the 3D-based technique. The agreement and ICC for intra-observer reproducibility was similar across all three techniques, but inter-observer reproducibility was superior with the 3DE-based technique., Conclusions: Although the maximum LAV obtained through the 3DE-based techniques was slightly smaller compared with the traditional manual tracing method, the 3DE-based technique is anticipated to be integrated into routine examinations owing to its short measurement time and superior reproducibility., (© 2024. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

Purpose: We investigated whether peripheral pulse synchronization (PPUS) can be an alternate method for electrocardiographic synchronization (ECGS) in measuring myocardial T1 values in cardiac magnetic resonance imaging (CMRI)., Methods: T1 map imaging was performed on 49 patients undergoing CMRI using the 5s (3s) 3s modified Look-Locker inversion recovery (MOLLI) method for both ECGS and PPUS. The short-axis images of basal, mid, and apical segments were obtained. The T1 map images were analyzed using an image processing system, and T1 values were obtained for each cardiac segment. To assess the degree of agreement between T1 values obtained from ECGS and PPUS, the Bland-Altman analysis and the estimating intraclass correlation coefficient (ICC) were performed for the average T1 value of the entire myocardium and T1 values of each cardiac segment. Also, to evaluate whether PPUS imaging is possible in the diastole phase, we measured the length of systole in the electrocardiogram and the length of transmission (R-R') from R in the electrocardiogram to R (R') in the pulse waveform., Results: From the comparison of T1 values, a good agreement of ICC was confirmed between the ECGS and PPUS (whole myocardium: 0.97, apical: 0.93, mid: 0.98, and basal: 0.97). The results of the Bland-Altman analysis also indicated good agreement. Moreover, it was shown that the heart was imaged in the diastole phase even with the default scan parameters of PPUS., Conclusion: Our results indicated that PPUS can be an alternate method for ECGS.

A 76-year-old woman was referred to our hospital for evaluation of markedly high intraocular pressures (IOPs): ≥40 mmHg in both eyes. No intraocular inflammation, pseudoexfoliation, or glaucomatous optic neuropathy was observed on ophthalmological examination. The 24-hour IOP fluctuations, measured with a Goldmann tonometer in the sitting position at 8, 12, 16, 20, and 24 o' clock, were 22/17, 33/28, 41/33, 30/22, and 30/24 mmHg, respectively, and showed a peak in the afternoon. The patient was diagnosed with ocular hypertension, and microhook trabeculotomy (μLOT) (right eye with lens reconstruction, left eye initially pseudophakic) was performed. After μLOT surgery in both eyes (4 days postoperatively in the right eye and 1 day postoperatively in the left eye), IOP decreased in both eyes to 12/15, 11/14, 12/15, 10/11, and 10/10 mmHg, and the fluctuation range was suppressed. At 3 months postoperatively (measured at 8, 12, 16, 20, 24, and 4 o' clock), the effect was maintained at 14/15, 15/19, 14/19, 11/12, 13/14, and 13/13 mmHg, respectively, but was slightly attenuated. In a patient with marked 24-hour IOP fluctuation, μLOT was effective in reducing IOP values and fluctuation.

Background and Objectives : Proximal junctional kyphosis (PJK) and failure (PJF), the most prevalent complications following long-segment thoracolumbar fusions for adult spinal deformity (ASD), remain lacking in defined preventive measures. We studied whether one of the previously reported strategies with successful results-a prophylactic augmentation of the uppermost instrumented vertebra (UIV) and supra-adjacent vertebra to the UIV (UIV + 1) with polymethylmethacrylate (PMMA)-could also serve as a preventive measure of PJK/PJF in minimally invasive surgery (MIS). Materials and Methods : The study included 29 ASD patients who underwent a combination of minimally invasive lateral lumbar interbody fusion (MIS-LLIF) at L1-2 through L4-5, all-pedicle-screw instrumentation from the lower thoracic spine to the sacrum, S2-alar-iliac fixation, and two-level balloon-assisted PMMA vertebroplasty at the UIV and UIV + 1. Results : With a minimum 3-year follow-up, non-PJK/PJF group accounted for fifteen patients (52%), PJK for eight patients (28%), and PJF requiring surgical revision for six patients (21%). We had a total of seven patients with proximal junctional fracture, even though no patients showed implant/bone interface failure with screw pullout, probably through the effect of PMMA. In contrast to the PJK cohort, six PJF patients all had varying degrees of neurologic deficits from modified Frankel grade C to D3, which recovered to grades D3 and to grade D2 in three patients each, after a revision operation of proximal extension of instrumented fusion with or without neural decompression. None of the possible demographic and radiologic risk factors showed statistical differences between the non-PJK/PJF, PJK, and PJF groups. Conclusions : Compared with the traditional open surgical approach used in the previous studies with a positive result for the prophylactic two-level cement augmentation, the MIS procedures with substantial benefits to patients in terms of less access-related morbidity and less blood loss also provide a greater segmental stability, which, however, may have a negative effect on the development of PJK/PJF.

BACKGROUND Fulminant type 1 diabetes is characterized by a low prevalence of autoantibodies, and was originally described as a nonautoimmune subtype of type 1 diabetes. Herein, we report a case in which we observed the process of extremely rapid onset of diabetes and early decline in anti-glutamic acid decarboxylase (GAD) antibody titers during the inpatient stay. CASE REPORT A 61-year-old man was brought to our hospital with marked hyperglycemia (1327 mg/dL), ketonemia (3-hydroxybutyrate: 14 012 µmol/L), and moderately elevated HbA1c (7.2%) and glycoalbumin (22.3%). C-peptide levels were undetectable. He had suffered from thirst, polyuria, and fatigue for 2 days. Abrupt onset was proven by the clinical data when he visited the hospital with respiratory symptoms 6 days before his admission; plasma glucose, glycoalbumin, C-peptide, and insulin levels were 117 mg/dL, 13.0%, 5.07 ng/mL, and 24.4 µIU/mL, respectively. The anti-GAD antibody titer measured by enzyme-linked immunosorbent assay was 111 U/mL at admission, 22.8 U/mL 2 weeks after admission, and negative 1 year later. He had a susceptible haplotype DRB1*09: 01-DQB1*03: 03, which is significantly more common in anti-GAD antibody-positive patients with fulminant type 1 diabetes. CONCLUSIONS The early decline of anti-GAD antibody titer likely reflected rapid and complete beta cell loss. The sequential metabolic and immunological observation in this case may provide insight into the pathogenesis of fulminant type 1 diabetes.

The morphologic features of the multiple atrial septal defects assessed by TTE-based 3D imaging were similar to those by 3D-TEE. TTE-based 3D model had excellent visibility, allowing observation of 3D structure of the rims of the defects. It may be useful method for assessment of the multiple atrial septal defects., (© 2024 Wiley Periodicals LLC.)

Background: The detailed hemodynamics after patent ductus arteriosus (PDA) ligation in preterm infants remain unknown. We aimed to clarify the effect of surgical ligation on left ventricular (LV) and right ventricular (RV) volume and function., Methods: Echocardiography was performed in 41 preterm infants (median gestational age: 25 weeks) before and after PDA ligation. Global longitudinal strain was determined using three-dimensional speckle-tracking echocardiography. These values were compared with those in 36 preterm infants without PDA (non-PDA)., Results: Preoperatively, the PDA group had greater end-diastolic volume (EDV) and cardiac output (CO) in both ventricles, a higher LV ejection fraction (LVEF) (53% vs 44%) and LV global longitudinal strain, and a lower RVEF (47% vs 52%) than the non-PDA group. At 4-8 h postoperatively, the two groups had a similar LVEDV and RVEDV. However, the PDA group had a lower EF and CO in both ventricles than the non-PDA group. At 24-48 h postoperatively, the RVEF was increased, but the LVEF remained decreased, and LVCO was increased., Conclusions: PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery. Three-dimensional echocardiography may be beneficial to understand the status of both ventricles., Impact: Preterm infants are at high risk of hemodynamic compromise following a sudden change in loading conditions after PDA ligation. Three-dimensional echocardiography enables quantitative and serial evaluation of ventricular function and volume in preterm infants with PDA. PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery., (© 2024. The Author(s).)

Ultrafine splittings are found in the optical absorption spectra of boron-doped diamond measured with high resolution. An analytical model of an exciton complex is developed, which permits assigning all absorption lines and sizing the interactions among the constituent charges and crystal field. We conclude that the entry of split-off holes in the acceptor-bound exciton fine structure yields two triplets separated by a spin-orbit splitting of 14.3 meV. Our findings thereby resolve a long-standing controversy [R. Sauer et al., Revised fine splitting of excitons in diamond, Phys. Rev. Lett. 84, 4172 (2000).PRLTAO0031-900710.1103/PhysRevLett.84.4172; M. Cardona et al., Comment on "Revised fine splitting of excitons in diamond,", Phys. Rev. Lett. 86, 3923 (2001).PRLTAO0031-900710.1103/PhysRevLett.86.3923; R. Sauer and K. Thonke, Sauer and Thonke reply, Phys. Rev. Lett. 86, 3924 (2001).PRLTAO0031-900710.1103/PhysRevLett.86.3924], revealing the underlying physics common in diverse semiconductors, including diamond.

A 7-year-old female sugar glider showed a well-defined, homogeneous, hypoechoic mass in the caudal abdominal cavity on ultrasound. Exploratory laparotomy revealed both uteri were severely dilated by viscous, purulent exudate. The patient underwent ovariohysterectomy with removal of bilateral vaginal canals. Antibiotic therapy was initiated postoperatively. Histopathological findings were consistent with suppurative metritis, leading to a clinical diagnosis of pyometra. Despite abscess formation in the uterine stump or central vaginal canal and bilateral renal pelvic/ureter dilations postoperatively, the sugar glider survived more than 270 days. Unfortunately, surgical and medical treatments performed in this case did not prove curative. The outcome of this case highlights the importance of further accumulation of cases to define more appropriate therapies for pyometra in sugar gliders.

Objective: The efficacy of anterior column realignment (ACR) remains relatively unclear, possibly because some safety concerns have limited its adoption and extensive evaluation. The authors aimed to study whether a minimally invasive surgery (MIS) triad consisting of ACR, lateral lumbar interbody fusion, and percutaneous pedicle screw fixation in a select group of adult spinal deformity (ASD) patients helps shorten fusion length without compromising clinical and radiographic outcomes over a minimum 2-year follow-up period., Methods: A series of 61 ASD patients (mean age 72.8 years) with pelvic incidence (PI) - lumbar lordosis (LL) (PI-LL) mismatch > 10° underwent the short-segment MIS triad (mean fusion length 3.0 levels) as a single-stage operation with a mean operative time and estimated blood loss of 157 minutes and 127 mL, respectively. Exclusion criteria were 1) thoracic scoliosis as the main deformity, 2) thoracolumbar junction kyphosis > 25°, 3) ankylosed facet joints, and 4) previous spinal fusion surgery. Seven patients, who needed fusion to be extended to S1, underwent mini-open transforaminal lumbar interbody fusion at L5-S1., Results: The segmental disc angle at the ACR level more than quintupled, averaging from 2.9° preoperatively to 18.9° at the latest follow-up (p < 0.0001). LL, in turn, nearly doubled from 17.0° to 32.8° (p < 0.0001) and PI-LL decreased by nearly half from 28.8° to 13.2° (p < 0.0001). At the same time, other spinopelvic deformity parameters as well as Oswestry Disability Index (ODI) scores significantly improved. Patients were divided into two groups at the latest postoperative evaluation: 36 patients whose PI-LL improved to < 10° and 25 patients who maintained a PI-LL mismatch > 10°. Binary logistic regression revealed preoperative PI-LL mismatch as the only factor that significantly influenced this dichotomous separation postoperatively. Receiver operating characteristic curve analysis identified the critical preoperative mismatch of 26.4° with 68% sensitivity and 84% specificity. Despite this different radiographic consequence, the two groups had an equally successful clinical outcome with no significant difference in ODI scores., Conclusions: As long as the ASD characteristics are consistent with the authors' exclusion criteria, the short-segment MIS triad served as an excellent surgical option in the patients with preoperative PI-LL mismatch < 26.4°, but the technique also worked well even in those with a mismatch > 26.4°, although ideal spinopelvic alignment targets were not necessarily achieved in these patients.

Background: Congenital diaphragmatic hernia (CDH) is associated with high neonatal mortality. We performed this study to test the hypothesis that left ventricular (LV) and right ventricular (RV) volumes assessed by three-dimensional echocardiography may be associated with mortality in CDH., Methods: This study was a single-center retrospective cohort study involving 35 infants with CDH. RV and LV end-diastolic volume (RVEDV and LVEDV, respectively) were measured by three-dimensional echocardiography and were corrected by birth body weight (BBW) on day 1. RVEDV/BBW, LVEDV/BBW, and LVEDV/RVEDV were compared between CDH survivors and non-survivors. Receiver-operating characteristic curve analysis was performed to assess the predictive ability for mortality of the echocardiographic parameters., Results: Comparing CDH non-survivors (n = 6) with survivors (n = 29), respectively, RVEDV/BBW was significantly larger (2.54 ± 0.33 vs 1.86 ± 0.35 ml/kg; P < 0.01), LVEDV/BBW was significantly smaller (0.86 ± 0.21 vs 1.22 ± 0.33 ml/kg; P < 0.001), and LVEDV/RVEDV was significantly lower (0.34 ± 0.06 vs 0.66 ± 0.18; P < 0.001). The area under the curve for LVEDV/RVEDV was the largest (0.98)., Conclusions: Three-dimensional echocardiographic volume imbalance between the RV and LV was remarkable in CDH non-survivors. The LVEDV/RVEDV ratio may be associated with mortality in CDH., Impact: Mortality with congenital diaphragmatic hernia (CDH) is high, and evaluating left and right ventricular structures and functions may be helpful in assessing the prognosis. Three-dimensional (3D) echocardiography indicated that the left ventricular end-diastolic volume/right ventricular end-diastolic volume ratio within 24 h after birth was associated with mortality in CDH infants. The usefulness of this ratio should be validated in prospective multicenter studies involving larger numbers of patients., (© 2023. The Author(s).)

Background: The purpose of this meta-analysis was to comprehensively investigate the diagnostic ability of 1.5 T and 3.0 T whole heart coronary angiography (WHCA) to detect significant coronary artery disease (CAD) on X-ray coronary angiography., Methods: A literature search of electronic databases, including PubMed, Web of Science Core Collection, Cochrane advanced search, and EMBASE, was performed to retrieve and integrate articles showing significant CAD detectability of 1.5 and 3.0 T WHCA., Results: Data from 1899 patients from 34 studies were included in the meta-analysis. 1.5 T WHCA had a summary area under ROC of 0.88 in the patient-based analysis, 0.90 in the vessel-based analysis, and 0.92 in the segment-based analysis. These values for 3.0 T WHCA were 0.94, 0.95, 0.96, respectively. Contrast-enhanced 3.0 T WHCA had significantly higher specificity than non-contrast-enhanced 1.5 T WHCA on a patient-based analysis (0.87, 95% CI 0.80-0.92 vs. 0.74, 95% CI 0.64-0.82, P = 0.02). There were no differences in diagnostic performance on a patient-based analysis by use of vasodilators, beta-blockers or between Asian and Western countries., Conclusions: The diagnostic performance of WHCA was deemed satisfactory, with contrast-enhanced 3.0 T WHCA exhibiting higher specificity compared to non-contrast-enhanced 1.5 T WHCA in a patient-based analysis. There were no significant differences in diagnostic performance on a patient-based analysis in terms of vasodilator or beta-blocker use, nor between Asian and Western countries. However, further large-scale multicentre studies are crucial for the widespread global adoption of WHCA., (© 2023. The Author(s).)

Background: We herein report the anesthetic management for extracardiac conduit-total cavopulmonary connection (EC-TCPC) for weaning from an extracorporeal right ventricular assist device (RVAD) in a patient with an implantable left ventricular assist device (LVAD) for fulminant cardiomyopathy., Case Presentation: A 24-year-old man developed fulminant cardiomyopathy and was placed on a biventricular assist device (BiVAD) comprising an implantable LVAD and an extracorporeal RVAD. The Fontan procedure was performed to wean the patient from the RVAD and allow him to be discharged home. Atrial septal defect creation, right ventricular suture, and tricuspid valve closure were then simultaneously performed to ensure sufficient left ventricular preload to drive the LVAD. Furthermore, to keep the central venous pressure lower, the inflow cannula of the LVAD was oriented in the correct direction., Conclusion: This is the first report of anesthetic management of the Fontan procedure in a patient with a BiVAD., (© 2023. The Author(s).)

The present study describes the clinical and pathological characteristics of skin lesions in two four-toed hedgehogs (Atelerix albiventris). We performed inverse PCR to identify the genome of papillomavirus (PV) in the skin lesions and subsequently sequenced the full genome of the virus, which was tentatively named Atelerix albiventris papillomavirus 1 (AalbPV1). The overall sequences of the viral genomes of both four-toed hedgehogs were identical. This study first identified the presence of a novel PV in Japanese four-toed hedgehogs and provided genetic information about this virus., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The morphology of 58 apocrine tumors from 31 domestic Richardson's ground squirrels ( Urocitellus richardsonii ) was examined, comparing tumor subtypes with clinical information and histological findings. The squirrels included 23 males (74%) and 8 females (26%). Of the 58 tumors, 36 (62%) were located from the cervical skin to the dorsal skin, 17 (29%) were from the perioral region to the cheek, and the location was unrecorded for the remaining 5 squirrels (9%). Histologically, 49 tumors (84%) were apocrine adenocarcinomas, 6 were apocrine adenomas (10%), and 3 were apocrine cysts (5%). The 49 apocrine adenocarcinomas were subclassified as 16 (33%) cystic papillary type, 16 (33%) tubulopapillary type, 12 (24%) solid type, and 5 (10%) micropapillary invasive type. Fisher's exact test revealed correlations between tumor subtypes and tumor diameters, nuclear atypia, tumor margins, vascular invasion, and periodic acid-Schiff (PAS) positivity. This study showed a predominance of apocrine adenocarcinomas with various histological subtypes. In addition, subclassification of these tumors was useful for predicting the biological behavior of apocrine gland tumors in Richardson's ground squirrels.

No standard treatment has been established for most rare cancers. Here, we report a clinical trial of a biweekly WT1 tri-peptide-based vaccine for recurrent or advanced rare cancers. Due to the insufficient number of patients available for a traditional clinical trial, the trial was designed for rare cancers expressing shared target molecule WT1. The recruitment criteria included WT1-expressing tumors as well as HLA-A*24:02 or 02:01. The primary endpoints were immunoglobulin G (IgG) antibody (Ab) production against the WT1-235 cytotoxic T lymphocyte (CTL) epitope and delayed-type hypersensitivity (DTH) skin reactions to targeted WT1 CTL epitopes. The secondary endpoints were safety and clinical efficacy. Forty-five patients received WT1 Trio, and 25 (55.6%) completed the 3-month protocol treatment. WT1-235 IgG Ab was positive in 88.0% of patients treated with WT1 Trio at 3 months, significantly higher than 62.5% of the weekly WT1-235 CTL peptide vaccine. The DTH positivity rate in WT1 Trio was 62.9%, which was not significantly different from 60.7% in the WT1-235 CTL peptide vaccine. The WT1 Trio safety was confirmed without severe treatment-related adverse events, except grade 3 myasthenia gravis-like symptoms observed in a patient with thymic cancer. Fifteen (33.3%) patients achieved stable disease after 3 months of treatment. In conclusion, the biweekly WT1 Trio vaccine containing the WT1-332 helper T lymphocyte peptide induced more robust immune responses targeting WT1 than the weekly WT1-235 CTL peptide vaccine. Therefore, WT1-targeted immunotherapy may be a potential therapeutic strategy for rare cancers.

Purpose: To objectively evaluate surgically induced astigmatism (SIA) after trabeculectomy with mitomycin C and investigate the relationships between SIA and various factors., Patients and Methods: This retrospective study included the right eyes of 66 consecutive patients who underwent standard trabeculectomy performed in the superior temporal quadrant for the first time by a single surgeon. Keratometry recordings made before surgery and 3 months after surgery were collected to calculate the SIA in each patient. The arithmetic mean of SIA (M-SIA) and the centroid of SIA (C-SIA) were determined using vector analysis. The relationships between the magnitude of SIA and the following possible related factors were assessed: age, sex, pre-operative corneal astigmatism, pre-operative intraocular pressure (IOP), 3-month postoperative IOP, pre-operative best-corrected visual acuity (BCVA), 3-month postoperative BCVA, the number of total scleral flap sutures (T-SFS), the number of leftover scleral flap sutures without laser suture lysis at 3 months postoperatively (L-SFS), shape of the scleral flap (triangle or trapezoid), and incision type of the conjunctival flap (fornix- or limbal-based)., Results: The mean (± standard deviation) M-SIA was 1.00 ± 0.85 D, and the mean C-SIA was 0.34 ± 1.28 D at 104°. The direction of C-SIA showed a trend of corneal steepening to the superior temporal location, in the direction of the scleral flap location. There were significant correlations of the magnitudes of SIA with the number of T-SFS (P = 0.001) and the number of L-SFS (P < 0.001)., Conclusion: Trabeculectomy induced SIA in the direction of the scleral flap location, and scleral sutures are significantly associated with the SIA. The scleral suture may play a key role in steepening the cornea toward the scleral flap direction in post-trabeculectomy patients., Competing Interests: The authors report no conflicts of interest in this work., (© 2022 Shiratori et al.)

Pigmentary mosaicism of the Ito type, also known as hypomelanosis of Ito, is a neurocutaneous syndrome considered to be predominantly caused by somatic chromosomal mosaicism. However, a few monogenic causes of pigmentary mosaicism have been recently reported. Eleven unrelated individuals with pigmentary mosaicism (mostly hypopigmented skin) were recruited for this study. Skin punch biopsies of the probands and trio-based blood samples (from probands and both biological parents) were collected, and genomic DNA was extracted and analyzed by exome sequencing. In all patients, plausible monogenic causes were detected with somatic and germline variants identified in five and six patients, respectively. Among the somatic variants, four patients had MTOR variant (36%) and another had an RHOA variant. De novo germline variants in USP9X, TFE3, and KCNQ5 were detected in two, one, and one patients, respectively. A maternally inherited PHF6 variant was detected in one patient with hyperpigmented skin. Compound heterozygous GTF3C5 variants were highlighted as strong candidates in the remaining patient. Exome sequencing, using patients' blood and skin samples is highly recommended as the first choice for detecting causative genetic variants of pigmentary mosaicism., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

A recent study has shown that the heterogeneity of native T1 mapping may be a new prognostic factor for patients with non-ischemic dilated cardiomyopathy (NIDCM). This study aimed to investigate the predictive value of native T1 heterogeneity of the left ventricular (LV) myocardium, as assessed by pixel-wise histogram analysis, for predicting left ventricular reverse remodeling (LVRR) by medical therapy in patients with NIDCM. A total of one hundred and thirteen NIDCM patients (mean age: 63 ± 12 years; 91 males and 22 females; mean LV ejection fraction (EF): 37 ± 10%) were retrospectively analyzed. T1 mapping images were acquired using a modified look-locker inversion recovery (MOLLI) sequence. We performed histogram analysis of native T1 mapping of LV myocardium, mean (T1-mean) and standard deviation (T1-STD) of native T1 time from each pixel were calculated. Extracellular volume fraction (ECV) was also evaluated. LVRR was defined as LVEF increased ≥ 10% points and decrease in LV end-diastolic volume ≥ 10% at 12 months from initiation of medical therapy. Cutoff value of T1-mean and T1-STD was set as median value of each parameter. Sixty (53%) NIDCM patients reached LVRR. Area under the receiver-operating characteristics curve for predicting LVRR was 0.763 (95% confidence interval (CI) 0.679-0.847) for %LGE, 0.757 (95% CI 0.663-0.850) for T1-mean, 0.724 (95% CI 0.625-0.823) for T1-STD, 0.800 (95% CI 0.717-0.882) for ECV, respectively. Proportion of LVRR was significantly lower in NIDCM patients with high T1-mean and high T1-STD (12%) compared to NIDCM with high T1-mean and low T1-STD (65%) (p < 0.001). Adding T1-STD to T1-mean improved AUC from 0.757 to 0.806, comparable to AUC of ECV. Combination of T1-mean and T1-STD, a parameter of heterogeneity of native T1 of the LV myocardium, may be a useful for prediction of LVRR by medical therapy without use of gadolinium contrast for patients with NIDCM., (© 2022. Springer Japan KK, part of Springer Nature.)

Background: Intimal smooth muscle cells (SMCs) play an important role in the vasculitis caused by Kawasaki disease (KD). Lipoprotein receptor 11 (LR11) is a member of the low-density lipoprotein receptor family, which is expressed markedly in intimal vascular SMCs and secreted in a soluble form (sLR11). sLR11 has been recently identified as a potential vascular lesion biomarker. sLR11 is reportedly elevated in patients with coronary artery lesions long after KD, but there is no description of sLR11 in acute KD. Our aim was to determine the sLR11 dynamics in acute KD and to assess its usefulness as a biomarker.Methods and Results: 106 acute KD patients and 18 age-matched afebrile controls were enrolled. KD patients were classified into the following subgroups: intravenous immunoglobulin (IVIG) responders (n=85) and non-responders (n=21). Serum sLR11 levels before IVIG therapy were higher in non-responders (median, 19.6 ng/mL; interquartile range [IQR], 13.0-24.9 ng/mL) than in controls (11.9 ng/mL, 10.4-14.9 ng/mL, P<0.01) or responders (14.3 ng/mL, 11.7-16.5 ng/mL, P<0.01). Using a cutoff of >17.5 ng/mL, non-responders to initial IVIG therapy were identified with 66.7% sensitivity and 78.8% specificity., Conclusions: sLR11 can reflect the state of acute KD and might be a biomarker for patient response to IVIG therapy.

Oxidation of polyunsaturated fatty acids produces various oxidized lipids whose absolute configuration (AC) and conformations are difficult to analyze due to their flexibility. Through studies on hydroxy fatty acids, lipid hydroperoxides, and lipid epoxides, this work demonstrates the effectiveness of VCD spectroscopy to elucidate their AC and conformational preferences.

Background: These clinical practice guidelines are intended to provide recommendations based on the best evidence obtained to date on key issues in clinical practice to improve the prognosis, diagnostic and therapeutic processes for patients with soft tissue tumors., Methods: The Guidelines Development Committee and Systematic Review Committee were composed of a multidisciplinary team of specialists who play an important role in soft tissue tumor care. Clinical questions (CQs) were determined by choosing key decision-making points based on Algorithms for the diagnosis and treatment of soft tissue tumors. The guidelines were developed according to the "Medical Information Network Distribution Service (Minds) Handbook for Clinical Practice Guideline Development 2014" and "Minds Manual for Clinical Practice Guideline Development 2017." Recommendation strength was rated on two levels and the strength of evidence was rated on four levels. The recommendations were decided based on agreement by 70% or more voters., Results: Twenty-two CQs were chosen by the Guidelines Development Committee. The Systematic Review Committee reviewed the evidence concerning each CQ, a clinical value judgment was added by experts, and the text of each recommendation was determined., Conclusion: We established 22 CQs and recommendations for key decision-making points in the diagnosis and treatment of soft tissue tumors according to the Minds Clinical Practice Guideline development methods. We hope that these guidelines will assist the decision-making of all medical staff engaged in the treatment and diagnosis of soft tissue tumors, and eventually lead to improved soft tissue tumor care in the country., Competing Interests: Declaration of competing Interest Akira Kawai reports research grants from Daiichi-Sankyo, Eli-Lilly, Taiho, Takara-Bio and Japanese Orthopaedic Association. Hirotaka Kawano reports honoraria from Daiichi Sankyo and Hisamitsu, and research grants from Japanese Orthopaedic Association, Nakatomi Foundation, Daiichi-Sankyo, Kyocera, Shionogi, Teijin, Zimmer Biomet, Johnson and Johnson, Smith and Nephew, Peter Blehm Japan and Stryker Japan. Toshiyuki Kunisada reports a research grant from Kuraray, and the Department of Medical Materials for Musculoskeletal Reconstruction is an endowment department, supported with an unrestricted grant from Kyocera. Shunji Takahashi reports honoraria from Eisai, Novartis, Taiho, Astrazeneca, Chugai, Bayer, Daiichi-Sankyo, and research grants from EISAI, Novartis, Taiho, Astrazeneca, Chugai, Bayer, Daiichi-Sankyo, Ono pharmaceutical, IQVIA and Bristol-Myers-Squib. Kazuhiro Tanaka belongs to an endowment department supported with grants from Teijin Nakashima Medical Co., Ltd. and Meditec Co., Ltd. Norio Yamamoto belongs to an endowed department supported with grants from Zimmer Biomet G.K. and Hisamitsu Pharmaceutical Co., Inc., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Takotsubo cardiomyopathy, a disease that causes transient contractile abnormalities mainly in the left ventricular apex, is rarely reported in children, especially in those with single-ventricle disease. A 4-year-old boy with a single right ventricle was transferred to our hospital following a severe seizure and was diagnosed with takotsubo cardiomyopathy by echocardiography. His cardiac function improved; however, he developed hypoxic-ischemic encephalopathy.

Objectives: Metastasectomy is often the local treatment for pulmonary metastases arising from osteosarcoma or soft tissue sarcoma. However, there have been few investigations on the outcomes of patients who undergo this procedure. In this study, we identified prognostic factors in patients with pulmonary metastases arising from osteosarcoma and soft tissue sarcoma to determine more appropriate eligibility criteria for metastasectomy., Methods: We retrospectively examined 37 patients who underwent metastasectomy of pulmonary nodules arising from osteosarcomas or soft tissue sarcomas at our institute between 2005 and 2020. Overall and recurrence-free survival intervals were determined using univariate and multivariate analyses., Results: A tumor doubling time > 1 month and a primary tumor histological type of osteosarcoma were independent predictors of longer overall survival on multivariate analysis (hazard ratios: 3.618 and 2.979, p = 0.00986 and 0.0373, respectively). Moreover, a > 1-month tumor doubling time and > 10-cm diameter of the primary tumor were independent predictors of longer recurrence-free survival (hazard ratios: 3.293 and 2.67, p = 0.0121 and 0.0134, respectively). Patients who underwent repeat pulmonary metastasectomy after complete resection of sarcoma-derived pulmonary metastases had significantly longer overall survival than those who did not (median: 5.91 years vs. 0.81 years, p < 0.0001)., Conclusions: Tumor doubling time is a significant predictor of clinical outcomes in patients who undergo resection of pulmonary metastases originating from sarcomas. The surgical indication for this procedure should be decided carefully, particularly for patients with metastatic lesion doubling times ≤ 1 month., (© 2021. The Japanese Association for Thoracic Surgery.)

The Wilms' tumor gene WT1 is highly expressed in various malignancies and may be a common target antigen for cancer immunotherapy. In our group, peptide-based cancer vaccines targeting WT1 CTL epitopes were developed as an immunotherapy for these malignancies. In the present study, WT1 epitope-specific immune responses were analyzed in 31 patients with advanced sarcoma with human leukocyte antigen-A*24:02- and WT1-expressing tumors who received the WT1-235 peptide vaccine as monotherapy. The serum levels of IgG and IgM antibodies against the target epitope WT1-235 and the non-target epitopes WT1-332 and WT1-271 were measured using ELISA. IgM antibodies against WT1-235, WT1-332 and WT1-271 were detected in three (9.6%), four (12.9%) and 20 patients (64.5%), respectively, prior to vaccine administration, indicating immune recognition of the WT1 antigen prior to administering the vaccine. Of 15 patients who had completed the 3-month treatment protocol, WT1-235 IgG was positive in five (33.3%) patients. An enzyme-linked immunospot assay revealed that WT1-235 epitope-specific IL-10 production/secretion in peripheral blood mononuclear cells declined in the first month of vaccine administration in all three patients with positivity for WT1-235 IgM at the start of the vaccine. Furthermore, positivity for both WT1-235 and WT1-271 IgM antibodies at the start of treatment was associated with unfavorable tumor control at 3 months after vaccine administration. These results suggested that WT1 epitope-specific IgG and IgM antibodies may be utilized as immune-monitoring markers for WT1 peptide cancer vaccine immunotherapy. The trials were entered in the University hospital Medical Information Network (UMIN) Clinical Trials Registry (https://www.umin.ac.jp/ctr; no. UMIN000002001 on May 24, 2009 and no. UMIN000015997 on December 20, 2014)., Competing Interests: The authors declare that they have no competing interests., (Copyright: © Alzaaqi et al.)

A 5-y-old, male degu ( Octodon degus ) was presented with a subcutaneous mass in the ventral aspect of the cervical area. The mass was removed surgically. Histologically, the mass was a densely cellular, expansile neoplasm, with compression of thymic tissue to the periphery. The neoplasm consisted of solid sheets of polygonal cells, mixed with fewer small lymphocytes. Rare Hassall bodies were scattered throughout the mass. Polygonal cells were positive for anti-keratin/cytokeratin AE1/AE3 antibody, and small lymphocytes were positive for anti-CD3 antibody. The histopathologic and immunohistochemical findings were consistent with a thymoma. In addition, an autopsy revealed myxosarcoma of the right thoracic wall with metastasis to the lung. To our knowledge, thymoma originating from the cervical component of the thymus has not been documented previously in a rodent species.

Background osteosarcoma is a rare, primary malignant bone tumour with limited available treatments for advanced or recurrent disease, resulting in a poor prognosis for patients. TAS-115 is a novel tyrosine kinase inhibitor under investigation in a phase I study in patients with solid tumours. We report data of osteosarcoma patients in the expansion cohort of this ongoing study. Patients and methods an analysis of this multicentre, open-label study was performed 6 months after the final patient was enrolled, and included patients aged ≥15 years, with unresectable or recurrent osteosarcoma, and who had refractory to standard therapy or for whom no standard therapy was available. TAS-115 650 mg/day was orally administered in a 5 days on/2 days off schedule. Results a total of 20 patients with osteosarcoma were enrolled. The most common adverse drug reactions (ADRs) were neutrophil count decreased (75%), aspartate aminotransferase increased (50%), and platelet count decreased (50%); 85% of patients had grade ≥ 3 ADRs. Long-term disease control (>1 year) with TAS-115 was achieved in three patients. The best overall response was stable disease (50%); no patient achieved a complete or partial response. Median progression-free survival was 3 months; 4-month and 12-month progression-free rates were 42% and 31%, respectively. Conclusion the safety and tolerability of TAS-115 and long-term disease stability for patients with unresectable or recurrent osteosarcoma were confirmed in this study, suggesting that TAS-115 is a promising novel therapy for advanced osteosarcoma patients. Trial registration number: JapicCTI-132333 (registered on November 8, 2013)., (© 2021. The Author(s).)

Decreased vision and cystoid macular edema (CME) developed in phakic eyes of a patient who underwent laser iridotomy after changing the glaucoma eye drops from carteolol 2% long-acting ophthalmic solution to omidenepag isopropyl 0.002%. CME completely disappeared at approximately 2 months after discontinuation of omidenepag isopropyl in conjunction with the use of bromfenac sodium 0.1%.

Skin lesions possibly caused by Papillomavirus infections in two four-toed hedgehogs are described. In case 1, there was a papillary mass on the right hind limb. Histologically, the mass was consistent with a viral papilloma. In the other case, multifocal papillary masses with erosions and ulcers were found throughout the body, mainly on the extremities. Histology showed continuative lesions composed of acanthosis, Bowenoid in situ carcinoma, and squamous cell carcinoma, with abrupt transitions between the lesions. In both cases, keratinocytes in the granular layer infrequently had features of koilocytes and intranuclear inclusion bodies, and immunohistochemical staining was positive for anti-human papillomavirus antibody. To the best of the authors' knowledge, this is the first pathological documentation of possibly papillomavirus-associated skin lesions in four-toed hedgehogs.

The relationship between hippocampal subfield volumetry and verbal list-learning test outcomes have mostly been studied in clinical and elderly populations, and remain controversial. For the first time, we characterized a relationship between verbal list-learning test outcomes and hippocampal subfield volumetry on two large separate datasets of 447 and 1,442 healthy young and middle-aged adults, and explored the processes that could explain this relationship. We observed a replicable positive linear correlation between verbal list-learning test free recall scores and CA1 volume, specific to verbal list learning as demonstrated by the hippocampal subfield volumetry independence from verbal intelligence. Learning meaningless items was also positively correlated with CA1 volume, pointing to the role of the test design rather than word meaning. Accordingly, we found that association-based mnemonics mediated the relationship between verbal list-learning test outcomes and CA1 volume. This mediation suggests that integrating items into associative representations during verbal list-learning tests explains CA1 volume variations: this new explanation is consistent with the associative functions of the human CA1., (© 2021 The Authors. Human Brain Mapping published by Wiley Periodicals LLC.)

Background: As percutaneous coronary intervention (PCI) for chronic total occlusion (CTO) gains wider acceptance as a therapeutic option for coronary artery disease, the importance of appropriate patient selection has increased. Although cardiovascular magnetic resonance imaging (MRI) allows segmental and quantitative analyses of myocardial ischemia and scar transmurality, it has limitations, including contraindications, cost, and accessibility. This study established a non-invasive method to evaluate patients undergoing CTO-PCI using two-dimensional speckle-tracking echocardiography (2D-STE)., Methods: Overall, we studied 55 patients who underwent successful CTO-PCI. Cardiovascular MRI and 2D-STE were performed before and 8 ± 2 months after CTO-PCI. Segmental findings of strain parameters were compared with those obtained with late gadolinium enhancement and stress-perfusion MRI., Results: With a cutoff of -10.7, pre-procedural circumferential strain (CS) showed reasonable sensitivity (71%) and specificity (73%) for detecting segments with transmural scar. The discriminatory ability of longitudinal strain (LS) for segments with transmural scar significantly improved during follow-up after successful CTO-PCI in the territory of the recanalized artery (area under the curve (AUC) 0.70 vs. 0.80, p < 0.001). LS accuracy was lower than that of CS at baseline (AUC 0.70 vs. 0.79, p = 0.048), and was increased at follow-up (AUC 0.80 vs. 0.82, p = 0.81). Changes in myocardial perfusion reserve from baseline to follow-up were significantly associated with those in LS but not in CS., Conclusions: Use of 2D-STE may allow the non-invasive evaluation of patients undergoing CTO-PCI to assess the indication before the procedure and treatment effects at follow-up.

Purpose: To clarify the intraocular pressure (IOP)-lowering effect of a selective prostanoid EP2 receptor agonist, omidenepag isopropyl (OMDI) during a 24-hour period., Patients and Methods: Subjects aged ≥20 years and with diagnosed, untreated primary open-angle glaucoma or ocular hypertension were enrolled. IOP measurements were performed every 4 hours over a 24-hour period using a Goldmann applanation tonometer (GAT) and Icare PRO tonometer (PRO). The baseline 24-hour IOP was measured in untreated subjects. After the baseline measurements, participants were given OMDI 1 drop once daily at night for 4 weeks. At week 4, the IOP measurement was repeated under the same conditions. Diurnal (9 am, 1 pm, 5 pm) and nocturnal (9 pm, 1 am, 5 am) IOP measurements were compared between baseline and treatment with OMDI. Safety measures included adverse events, slit-lamp biomicroscopy, visual acuity, heart rate and blood pressure., Results: Of 27 participants enrolled, 25 patients (20 males and 5 females, average age 52.2 ± 8.5 years) completed the study. In the sitting position, the baseline diurnal and nocturnal mean IOPs (GAT) were 19.1 ± 2.1 mmHg and 18.2 ± 2.6 mmHg, respectively, the diurnal and nocturnal mean IOP reduction from baseline were -2.8 ± 2.6 mmHg (p < 0.0001) and -3.3 ± 2.9 mmHg (p < 0.0001), respectively, mean 24-hour IOP (GAT) was significantly lower with the OMDI treatment (-3.1 ± 2.5 mmHg, p < 0.0001). In the supine position, the baseline nocturnal mean IOP (PRO) was 17.99 ± 2.22 mmHg, and the nocturnal mean IOP reduction from baseline was -1.78 ± 2.37 mmHg (p = 0.0009) after 4 weeks of the treatment. Nine adverse events were observed in 8 patients including mild conjunctival hyperemia (n = 8) and mild iritis (n=1). There were no significant effects on systemic safety., Conclusion: Once daily OMDI treatment was able to produce stable 24-hour IOP reduction., Competing Interests: The authors have made the following disclosure: NS, YN, AT, SS and KT received non-financial research support from Santen Pharmaceutical Co., Ltd. during the conduct of study; NO, NI, DS, and KH were employees of Santen Pharmaceutical Co., Ltd.; KN had a consulting relationship with Santen Pharmaceutical Co., Ltd and reports personal fees from Shinanozaka Clinic. The authors report no other conflicts of interest in this work., (© 2021 Shiratori et al.)

We report on MRi-Share, a multi-modal brain MRI database acquired in a unique sample of 1870 young healthy adults, aged 18-35 years, while undergoing university-level education. MRi-Share contains structural (T1 and FLAIR), diffusion (multispectral), susceptibility-weighted (SWI), and resting-state functional imaging modalities. Here, we described the contents of these different neuroimaging datasets and the processing pipelines used to derive brain phenotypes, as well as how quality control was assessed. In addition, we present preliminary results on associations of some of these brain image-derived phenotypes at the whole brain level with both age and sex, in the subsample of 1722 individuals aged less than 26 years. We demonstrate that the post-adolescence period is characterized by changes in both structural and microstructural brain phenotypes. Grey matter cortical thickness, surface area and volume were found to decrease with age, while white matter volume shows increase. Diffusivity, either radial or axial, was found to robustly decrease with age whereas fractional anisotropy only slightly increased. As for the neurite orientation dispersion and densities, both were found to increase with age. The isotropic volume fraction also showed a slight increase with age. These preliminary findings emphasize the complexity of changes in brain structure and function occurring in this critical period at the interface of late maturation and early ageing., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Human brain white matter undergoes a protracted maturation that continues well into adulthood. Recent advances in diffusion-weighted imaging (DWI) methods allow detailed characterizations of the microstructural architecture of white matter, and they are increasingly utilized to study white matter changes during development and aging. However, relatively little is known about the late maturational changes in the microstructural architecture of white matter during post-adolescence. Here we report on regional changes in white matter volume and microstructure in young adults undergoing university-level education. As part of the MRi-Share multi-modal brain MRI database, multi-shell, high angular resolution DWI data were acquired in a unique sample of 1,713 university students aged 18-26. We assessed the age and sex dependence of diffusion metrics derived from diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) in the white matter regions as defined in the John Hopkins University (JHU) white matter labels atlas. We demonstrate that while regional white matter volume is relatively stable over the age range of our sample, the white matter microstructural properties show clear age-related variations. Globally, it is characterized by a robust increase in neurite density index (NDI), and to a lesser extent, orientation dispersion index (ODI). These changes are accompanied by a decrease in diffusivity. In contrast, there is minimal age-related variation in fractional anisotropy. There are regional variations in these microstructural changes: some tracts, most notably cingulum bundles, show a strong age-related increase in NDI coupled with decreases in radial and mean diffusivity, while others, mainly cortico-spinal projection tracts, primarily show an ODI increase and axial diffusivity decrease. These age-related variations are not different between males and females, but males show higher NDI and ODI and lower diffusivity than females across many tracts. These findings emphasize the complexity of changes in white matter structure occurring in this critical period of late maturation in early adulthood., 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 © 2021 Tsuchida, Laurent, Crivello, Petit, Pepe, Beguedou, Debette, Tzourio and Mazoyer.)

We demonstrate that broadband sum frequency generation (SFG) spectroscopy based on a partially incoherent supercontinuum light source can elucidate dark p-series excitons in monolayer WSe 2 encapsulated between hexagonal boron nitride (hBN) slabs. The observed 2p exciton peak energy is a few meV higher than that predicted by the Rytova-Keldysh potential model, which is originated from the Berry phase effect. Interestingly, although the radiative relaxation of the 2p exciton is weaker, the 2p exciton peak is broader than the 1s and 2s peaks, which indicates its faster dephasing than the 1s and 2s excitons. Measuring the excitation intensity and temperature dependence, we clarified that this broader linewidth is not caused by excitation- or phonon-induced dephasing, but rather by exciton-electron scattering.

We elucidated clinicopathological characteristics of giant cell tumor of bone (GCTB) in Japan, and significant clinicopathological factors for predicting local recurrence. Clinicopathological profiles of 213 patients with GCTB (100 male, 113 female) involving extra-craniofacial bones were retrieved. Pathological slides obtained at the initial surgery were reviewed. Fourteen pathological and five clinical features were statistically analyzed to disclose prognostic significance. Patient age ranged from 12-80 years (Average 38.7). Long bones were most frequently affected (86.4%), especially around the knee (62.9%). Histological features are basically similar to those previously reported. Within a follow-up period (24-316 months, average 106.1 months), the local recurrence rate is 29.1%. Metastasis has occurred in 9 patients. Cox regression analysis of representative clinicopathological features shows that younger age, higher mitotic count, smaller zones of stromal hemorrhage, considerable vascular invasion and absence of ischemic necrosis are significant predictors for local recurrence. Initial operative method (curettage) is a significant risk factor in univariate analysis but not by multivariate analysis (P = 0.053). Denosumab administration increases risk but not significantly (P = 0.053). Histone 3.3 G34W immunopositivity is not significant for predicting local recurrence., (© 2021 The Authors. Pathology International published by Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.)

Purpose: The relationship between endometrial polyps (EPs), chronic endometritis (CE), hysteroscopic findings, and antimicrobial in infertility patients was determined., Methods: We retrospectively enrolled 115 infertility patients with suspected EPs who underwent office hysteroscopy. Patients were divided into 3 groups: 38 with increased plasma cells in EPs (group 1); 31 without increased plasma cells in EPs (group 2); and 46 without EPs (group 3). The 3 groups underwent hysteroscopy with or without polypectomies, and immediately thereafter, an endometrial aspiration biopsy (EAB) was performed. CE was diagnosed based on plasma cell infiltration in the non-polypoid endometrium obtained by EAB., Results: The percentage of CE was 68.4%, 32.2%, and 28.3% in groups 1, 2, and 3, respectively. CE was more frequent in group 1 than group 2 or 3 ( P  = .01 and P  = .002, respectively). The number of polyps was higher in group 1 than group 2. After adjustment for age and assisted reproductive technology, antibiotic therapy was not associated with pregnancy (adjusted odds ratio, 0.44; 95% confidence interval, 0.05-3.57) in patients with EPs and CE., Conclusions: Group 1 was associated with CE, and hysteroscopic findings were different from group 2. Antibiotic therapy after polypectomy for EPs with CE may not always be necessary., Competing Interests: Conflict of interest: The authors declare no conflicts of interest. Human rights statements and informed consent: All of the procedures were followed in accordance with the ethical standards of the responsible committees on human experimentation (institutional and national) and with the principles of the 1964 Helsinki Declaration and subsequent amendments. This study was approved by the Institutional Review Board of Takagi Hospital. This was a retrospective study involving patients who submitted informed consent for undergoing fertility treatment in our Department. Animal studies: This article did not involve animal experimentation by any of the authors., (© 2021 The Authors. Reproductive Medicine and Biology published by John Wiley & Sons Australia, Ltd on behalf of Japan Society for Reproductive Medicine.)

Background and Objectives: Young adults represent an increasingly large proportion of healthy volunteers in brain imaging research, but descriptions of incidental findings (IFs) in this age group are scarce. We aimed to assess the prevalence and severity of IFs on brain MRIs of healthy young research participants aged 18-35 years, and to describe the protocol implemented to handle them. Methods: The study population comprised 1,867 participants aged 22.1 ± 2.3 years (72% women) from MRi-Share, the cross-sectional brain MRI substudy of the i-Share student cohort. IFs were flagged during the MRI quality control. We estimated the proportion of participants with IFs [any, requiring medical referral, potentially serious (PSIFs) as defined in the UK biobank]: overall, by type and severity of the final diagnosis, as well as the number of IFs. Results: 78/1,867 participants had at least one IF [4.2%, 95% Confidence Interval (CI) 3.4-5.2%]. IFs requiring medical referral ( n = 38) were observed in 36/1,867 participants (1.9%, 1.4-2.7%), and represented 47.5% of the 80 IFs initially flagged. Referred IFs were retrospectively classified as PSIFs in 25/1,867 participants (1.3%, 0.9-2.0%), accounting for 68.4% of anomalies referred (26/38). The most common final diagnosis was cysts or ventricular abnormalities in all participants (9/1,867; 0.5%, 0.2-0.9%) and in those with referred IFs (9/36; 25.0%, 13.6-41.3%), while it was multiple sclerosis or radiologically isolated syndrome in participants with PSIFs (5/19; 26.3%, 11.5-49.1%) who represented 0.1% (0.0-0.4%) and 0.2% (0.03-0.5%) of all participants, respectively. Final diagnoses were considered serious in 11/1,867 participants (0.6%, 0.3-1.1%). Among participants with referred IFs, 13.9% (5/36) required active intervention, while 50.0% (18/36) were put on clinical surveillance. Conclusions: In a large brain imaging study of young healthy adults participating in research we observed a non-negligible frequency of IFs. The etiological pattern differed from what has been described in older adults., Competing Interests: SD is currently a guest editor for the Research Topic this work will be submitted to, and has collaborated/is collaborating on research projects or publications with the other two editors of this topic. Outside the submitted work, BB served on the scientific advisory board of Biogen, BMS, Novartis, Roche, Merck, and Jansen, received speaker honoraria from Biogen, BMS, and Roche, served as an editorial board member of Multiple Sclerosis International (2019–2020), received research support from Roche, Biogen, Bayer, Sanofi, and Caridian. The remaining 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 © 2021 Soumaré, Beguedou, Laurent, Brochet, Bordes, Mournet, Mellet, Pereira, Pollet, Lachaize, Mougin, Tsuchida, Loiseau, Tourdias, Tzourio, Mazoyer and Debette.)

A 45-year-old woman was referred to our hospital for the evaluation of proximal muscle weakness and serum creatine kinase elevation [corrected]. She had atrial fibrillation and left ventricular asynergy. She was diagnosed with myopathy, accompanied by cardiomyopathy of unknown etiology. She was treated with prednisolone. After long-term follow-up and a detailed examination, the patient was diagnosed with antimitochondrial antibody (AMA)-associated myopathy with cardiac involvement. Although the patient received medical treatment, including beta-blockers and prednisolone, her cardiac function deteriorated progressively. Physicians should consider AMA-associated myopathy when diagnosing myopathies of unknown etiology. The presence of cardiac involvement should be proactively investigated in AMA-associated myopathy.

Previous studies have not clearly identified a prognostic factor for desmoid tumours (DT). Whole-exome sequencing (WES) and/or RNA sequencing (RNA-seq) were performed in 64 cases of DT to investigate the molecular profiles in combination with the clinicopathological characteristics. CTNNB1 mutations with specific hotspots were identified in 56 cases (87.5%). A copy number loss in chromosome 6 (chr6) was identified in 14 cases (21.9%). Clustering based on the mRNA expression profiles was predictive of the patients' prognoses. The risk score generated by the expression of a three-gene set (IFI6, LGMN, and CKLF) was a strong prognostic marker for recurrence-free survival (RFS) in our cohort. In risk groups stratified by the expression of IFI6, the hazard ratio for recurrence-free survival in the high-risk group relative to the low-risk group was 12.12 (95% confidence interval: 1.56-94.2; p = 8.0 × 10 6 ). In conclusion, CTNNB1 mutations and a chr6 copy number loss are likely the causative mutations underlying the tumorigenesis of DT while the gene expression profiles may help to differentiate patients who would be good candidates for wait-and-see management and those who might benefit from additional systemic or radiation therapies., Competing Interests: Conflict of interest statement The authors certify that no actual or potential conflict of interest in relation to this article exists., (Copyright © 2020 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

We report an unusual case of acute myocardial infarction in a high school girl. The patient was 17 years of age and had multiple coronary risk factors, including marked obesity with a body mass index (BMI) of 42.7 kg/m 2 , dyslipidemia and glucose intolerance. She had been an on and off smoker since she was 13 years of age. Due to the recent Westernization of the lifestyle, the prevalence of metabolic syndrome in the young generation has been increasing in Japan. Cardiovascular disease based on lifestyle-related diseases may become more common in young people.

Malignant peripheral nerve sheath tumor (MPNST) often does not respond well to chemotherapy and develops against a background of NF1. The purpose of our study was to examine the efficacy of pazopanib against MPNST. Our study was designed as a physician-initiated phase II clinical trial in patients with advanced MPNST. Patients were registered from 11 large hospitals. The primary endpoint was set to clarify the clinical benefit rate (CBR) at 12 weeks according to response evaluation criteria in solid tumors (RECIST). Progression-free survival (PFS), overall survival (OS) and the CBR based on modified Choi evaluation at week 12 were set as secondary endpoints along with treatment-related safety. The study enrolled 12 patients. Median age was 49 years. Seven had Grade 2 and five Grade 3 according to the FNCLCC evaluation. Median follow-up period was 10.6 months. CBR at 12 weeks was both 50.0% (RECIST and Choi). The median PFS was 5.4 months for both RECIST and Choi, and the median OS was 10.6 months. Of special interest, the median PFS was 2.9 months for patients with FNCLCC Grade 2 and 10.2 months for Grade 3 (both RECIST and Choi). Grade 4 adverse events of neutropenia and lipase elevation were noted in one patient each. The results of this pazopanib therapy were generally better than those of any of the other single molecular targeted therapies reported previously. Although accumulation of more cases remains necessary, we conclude pazopanib treatment for MPNST to be a safe and promising treatment after doxorubicin-based chemotherapy., (© 2020 Union for International Cancer Control.)