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Tomoki Nakamura,1 Jun Sugaya,2 Norifumi Naka,3 Hiroshi Kobayashi,4 Tomotake Okuma,5 Toshiyuki Kunisada,6 Kunihiro Asanuma,1 Hedetatsu Outani,7 Shunji Nishimura,8 Hiroyuki Kawashima,9 Toru Akiyama,10 Taketoshi Yasuda,11 Shinji Miwa,12 Akihiro Sudo,1 Takafumi Ueda13 1Department of Orthopaedic Surgery, Mie University Graduate School of Medicine, Tsu 514-8507, Japan; 2Department of Musculoskeletal Oncology, National Cancer Center Hospital, Chuo-ku, Tokyo 104-0045, Japan; 3Musculoskeletal Oncology Service, Osaka International Cancer Institute, Chuo-ku, Osaka 541-8567, Japan; 4Department of Orthopaedic Surgery, The University of Tokyo Hospital, Bunkyo-ku, Tokyo 113-8655, Japan; 5Department of Musculoskeletal Oncology, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Bunkyo-ku, Tokyo 113-8677, Japan; 6Department of Orthopaedic Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan; 7Department of Orthopaedic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka 565-0871, Japan; 8Department of Orthopaedic Surgery, Kindai University Hospital, Osaka-Sayama, Osaka 589-8511, Japan; 9Division of Orthopaedic Surgery, Niigata University Graduate School of Medical and Dental Sciences, Chuo-ku, Niigata 951-8510, Japan; 10Department of Orthopaedic Surgery, Saitama Medical Center, Jichi Medical University, Saitama, Saitama 330-8503, Japan; 11Department of Orthopaedic Surgery, University of Toyama, Toyama 930-0194, Japan; 12Department of Orthopaedic Surgery, Kanazawa University Graduate School of Medical Sciences, Kanazawa 920-8641, Japan; 13Department of Orthopaedic Surgery, Osaka National Hospital, Chuo-ku, Osaka 540-0006, JapanCorrespondence: Tomoki NakamuraDepartment of Orthopedic Surgery, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, JapanTel +81-592315022Fax +81-592315211Email tomoki66@clin.medic.mie-u.ac.jpBackground: The outcomes of unplanned surgery for bone sarcomas have not been frequently discussed. However, it is important to recognize patterns, treatment, and clinical outcomes of unplanned surgeries for patients with bone sarcomas. This multicenter study aimed to characterize the clinical outcomes of patients with bone sarcomas who underwent unplanned surgeries.Patients and Methods: Data of 43 patients with bone sarcomas who underwent unplanned surgery between 2006 and 2017 were obtained from 23 hospitals in Japan. These included 18 cases of osteosarcoma, 9 of Ewing sarcoma, 8 of chondrosarcoma, and 6 of undifferentiated pleomorphic sarcoma. The study included 28 men and 15 women, with a mean age of 46 years. The mean follow-up duration was 59 months.Results: The main primary tumor sites were the femur (n = 19), spine (n = 6), pelvis (n = 5), tibia (n = 3), and humerus (n = 3). The primary diagnoses were benign bone tumor (n = 24), trauma (n = 7), bone metastasis (n = 5), osteomyelitis (n = 4), degeneration (n=2), and unknown (n = 1). As unplanned surgeries, curettage, with or without bone graft, was performed in 26 patients; internal fixation was performed in 7; spinal surgery in 5; arthroplasty in 4; and arthroscopy in one. Thirty-eight patients received additional standard treatments. Thirty-four of these patients underwent surgical tumor resections, including amputation (n = 10), and the remaining 4 received radiotherapy or carbon ion radiotherapy as additional standard treatments. The 5-year disease-specific survival (DSS) rates in patients with osteosarcoma, Ewing sarcoma, and chondrosarcoma were 65.5%, 58.3%, and 72.9%, respectively. Twelve (27.9%) patients developed local recurrences (LR); among the total 43 patients studied, the 5-year DSS rates were significantly worse for those who developed LR compared to those who did not (p = 0.03). The 5-year DSS rates in patients with and without LR were 44% and 73.8%, respectively.Conclusion: We recommend that patients who have undergone unplanned surgery be administered standard treatment, including the option of amputation because herein, LR was shown to be a risk factor for decreased DSS.Keywords: bone sarcoma, unplanned surgery, local recurrence, osteosarcoma, Ewing sarcoma, chondrosarcoma

Background. In children, hypernatraemia occurs most commonly in infants (younger than 1 year). Although hypernatraemia is associated with high mortality and morbidity rates, it is variably defined in the paediatric literature as either serum sodium ≥150 mmol/L or serum sodium >145 mmol/L. In hospitalised adults, a serum sodium level >145 mmol/L but <150 mmol/L (called borderline hypernatraemia) has recently been identified as an independent risk factor for mortality. There are limited data about a potential association between borderline hypernatraemia and mortality in infants. Objectives. To determine whether borderline hypernatraemia is associated with increased mortality in hospitalised infants. Methods. We conducted a single-centre, retrospective observational study of 8 343 infants admitted to a tertiary-level academic hospital in Johannesburg, South Africa, of whom 254 had borderline hypernatraemia, 376 had hypernatraemia (serum sodium ≥150 mmol/L), and 7 713 did not have hypernatraemia. Mortality rates were reported as odds ratios (ORs) with 95% confidence intervals (CIs). Results. In 254 infants with borderline hypernatraemia, 115 (45.3%) were neonates (≤28 days old) and 140 (55.1%) were male. In 139 infants >28 days old with borderline hypernatraemia, the mortality rate (n=9/139; 6.5%) was significantly higher than the mortality rate observed in infants without hypernatraemia (n=194/5 857; 3.3%) (OR 2.02; 95% CI 1.03 - 3.98). Conclusion. Borderline hypernatraemia may be a risk factor associated with higher mortality in hospitalised infants. Prospective studies are required to determine whether borderline hypernatraemia contributes independently to mortality risk in hospitalised infants. [ABSTRACT FROM AUTHOR]

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).)