Granular cell tumors: a report of six cases

Abstract Granular cell tumor is a soft tissue neoplasm that originates in the nervous system and arises at virtually any body site, but is mainly found in the skin, oral cavity or digestive tract. Most are benign and reportedly malignant cases are rare, occurring in only 1% or 2% of cases. We report on our findings in six patients who developed granular cell tumor in the mammary gland, esophagus, subcutaneous tissue and muscle. Of six patients, two had granular cell tumor located in the breast, two in the submucosa of the esophagus, and the other two under the skin of the left axillary cavity and in the right latissimus dorsi muscle, respectively. One of the two patients with tumor in the submucosa of the esophagus also had esophageal cancer. Patients’ age ranged from 41 to 70 years (average, 59.1 years). Two patients with tumor in the submucosa of the esophagus were men, and the others were women. All of them were given a diagnosis of granular cell tumor by tissue biopsy and examination of excised specimens, but no evidence of malignancy was found. No recurrence has been noted in the patients who underwent surgical tumor removal.</p

Introduction of Low-Carbon Community Energy Systems by Combining Information Networks and Cogeneration-Type District Heating and Cooling Systems

Achievement of a low-carbon society is becoming extremely important. In this report, we introduce an example of carbon dioxide (CO2) emission reductions and energy savings, using a local energy-control system. Our research is focused on the town of Shinchi in the Fukushima Prefecture, Japan. Shinchi is pursuing initiatives to create a low-carbon, energy-efficient society and a disaster-resilient community. The National Institute for Environmental Studies provides academic support for the design and planning of low-carbon community energy systems by the local government of Shinchi, based on the Basic Cooperation Agreement. For the redevelopment of the Japan Railway (JR) Shinchi Station district that is being carried out, construction of a cogeneration-type district heating and cooling system is currently in progress. CO2 reductions of about 20% can be expected by introducing this community energy system. To support these initiatives, we have developed an information and communication technology (ICT) system that shares a wide range of local information to support energy conservation. By analyzing electricity consumption data from the ICT, we evaluated the pattern of residential power consumption and confirmed that the project supports energy-saving behavior within the community. Additionally, the community energy project in the JR Shinchi Station district enables adjustment of the supply and demand balance

Spontaneous Bilateral Pneumothorax in a Patient with Anorexia Nervosa: The Management of Prolonged Postoperative Air Leakage

A 24-year-old Japanese female with anorexia nervosa presented to our hospital for bilateral pneumothorax, and 12-Fr thoracostomy catheters were inserted into the bilateral pleural cavities. On hospital day 9, a thoracoscopic bullectomy was performed. However, air leakage relapsed on both sides on postoperative day 1. The air leakage on the right side was particularly persistent, and we switched the drainage to a Heimlich valve. Both lungs expanded gradually and the chest tube was removed on postoperative day 19. Passive pleural drainage might be an option for prolonged air leakage after a bullectomy in patients with anorexia nervosa

Prenatal Diagnosis of Interrupted Aortic Arch: Usefulness of Three-Vessel and Four-Chamber Views

Interrupted aortic arch (IAA) is fatal if not diagnosed. Prenatal diagnosis is helpful, but it is difficult to detect IAA and even more so to differentiate types A and B prenatally. Our objectives were to find a way to detect IAA using 2 views—three-vessel view (3VV) and four-chamber view (4CV)—and to differentiate between types A and B. We retrospectively analyzed fetal echocardiographic images and medical records of eight IAA patients. All eight patients had a ventricular septal defect (VSD) on 4CV. The aorta/main pulmonary artery (Ao/MPA) diameter ratio on 3VV was significantly low, which is characteristic of type B IAA. The left/right ventricular diameter (LV/RV) ratio on 4CV was 0.61± 0.17 for type A and almost 1.0 for type B. The thymus was not observed on 3VV in some type B IAA patients. These findings suggest that we could increase the number of prenatal diagnoses of IAA using the Ao/MPA ratio on 3VV and the presence of VSD on 4CV. Additionally, we could differentiate types A and B with the LV/RV ratio on 4CV, the Ao/MPA ratio, and the presence of a thymus on 3VV, which results in better management of IAA after birth

A Bicyclic 1-Deoxygalactonojirimycin Derivative as a Novel Pharmacological Chaperone for GM1 Gangliosidosis

Lysosomal β-galactosidase (β-Gal) deficiency causes a group of disorders that include neuronopathic GM1 gangliosidosis and non-neuronopathic Morquio B disease. We have previously proposed the use of small molecule ligands of β-Gal as pharmacological chaperones (PCs) for the treatment of GM1 gangliosidosis brain pathology. Although it is still under development, PC therapy has yielded promising preclinical results in several lysosomal diseases. In this study, we evaluated the effect of bicyclic 1-deoxygalactonojirimycin (DGJ) derivative of the sp2-iminosugar type, namely 5N,6S-(N′-butyliminomethylidene)-6-thio-1- deoxygalactonojirimycin (6S-NBI-DGJ), as a novel PC for human mutant β-Gal. In vitro, 6S-NBI-DGJ had the ability to inhibit the activity of human β-Gal in a competitive manner and was able to protect this enzyme from heat-induced degradation. Computational analysis supported that the rigid glycone bicyclic core of 6S-NBI-DGJ binds to the active site of the enzyme, with the aglycone N′-butyl substituent, in a precise E-orientation, located at a hydrophobic region nearby. Chaperone potential profiling indicated significant increases of enzyme activity in 24 of 88 β-Gal mutants, including four common mutations. Finally, oral administration of 6S-NBI-DGJ ameliorated the brain pathology of GM1 gangliosidosis model mice. These results suggest that 6S-NBI-DGJ is a novel PC that may be effective on a broad range of β-Gal mutants.Ministerio de Ciencia e Innovación de España. SAF2010-15670 y CTQ2010-15848Junta de Andalucía. P08-FQM-0371

PIM kinases facilitate lentiviral evasion from SAMHD1 restriction via Vpx phosphorylation

Lentiviruses have evolved to acquire an auxiliary protein Vpx to counteract the intrinsic host restriction factor SAMHD1. Although Vpx is phosphorylated, it remains unclear whether such phosphorylation indeed regulates its activity toward SAMHD1. Here we identify the PIM family of serine/threonine protein kinases as the factors responsible for the phosphorylation of Vpx and the promotion of Vpx-mediated SAMHD1 counteraction. Integrated proteomics and subsequent functional analysis reveal that PIM family kinases, PIM1 and PIM3, phosphorylate HIV-2 Vpx at Ser13 and stabilize the interaction of Vpx with SAMHD1 thereby promoting ubiquitin-mediated proteolysis of SAMHD1. Inhibition of the PIM kinases promotes the antiviral activity of SAMHD1, ultimately reducing viral replication. Our results highlight a new mode of virus–host cell interaction in which host PIM kinases facilitate promotion of viral infectivity by counteracting the host antiviral system, and suggest a novel therapeutic strategy involving restoration of SAMHD1-mediated antiviral response

Identification of novel proteins differentially expressed in pluripotent embryonic stem cells and differentiated cells

Mammalian pluripotent stem cells possess properties of self-renewal and pluripotency. These abilities are maintained by the strict regulation of pluripotent stem cell-specific transcription factor network and unique properties of chromatin in the stem cells. Although these major signaling pathways robustly control the characteristics of stem cells, other regulatory factors, such as metabolic pathways, are also known to modulate stem cell proliferation and differentiation. In this study, we fractionated protein samples from mouse embryonic stem (ES) cells cultured with or without the leukemia inhibitory factor (LIF). Protein expression was quantified by 2-dimensional differential gel electrophoresis (2D-DIGE). In total, 44 proteins were identified as being differentially expressed in the pluripotent stem cells and the differentiated cells. Surprisingly, half of the identified proteins were the proteins localized in mitochondria, which supply cellular energy and regulate cell cycle, development, and cell death. Some of these identified proteins are involved in the metabolic function and the regulation of pluripotency. Further analysis of the identified proteins could provide new information for the manipulation of pluripotency in ES cells

Background Factors Affecting Visual Acuity at Initial Visit in Eyes with Central Retinal Vein Occlusion : Multicenter Study in Japan

Purpose: To determine the baseline characteristics of patients with central retinal vein occlusion (CRVO) that were significantly associated with the best-corrected visual acuity (BCVA) at the initial examination. Methods: This was a retrospective multicenter study using the medical records registered in 17 ophthalmological institutions in Japan. Patients with untreated CRVO (≥20-years-of-age) who were initially examined between January 2013 and December 2017 were studied. The patients’ baseline factors that were significantly associated with the BCVA at the initial examination were determined by univariate and multivariate linear regression analyses. Results: Data from 517 eyes of 517 patients were analyzed. Univariate analyses showed that an older age (r = 0.194, p < 0.001) and the right eye (r = −0.103, p < 0.019) were significantly associated with poorer BCVA at the initial visit. Multivariate analyses also showed that an older age (β = 0.191, p < 0.001) and the right eye (β = −0.089, p = 0.041) were significantly associated with poorer BCVA at the initial visit. Conclusions: The results indicate that an older age, a known strong factor, and the right eye were significantly associated with poorer BCVA at the initial visit to the hospital. These results suggest that functional and/or anatomical differences between the right and left eyes may be involved in these results