Transitions in vascular ultrasonography findings of temporal arteritis in a GCA case with progressive temporal headache and visual impairment.

The European League Against Rheumatism and the American College of Rheumatology have stated that the halo sign on vascular ultrasonography (v-US) is relevant in diagnosing giant cell arteritis (GCA) and is equivalent to temporal artery biopsy. However, there are only a few reports about transitions in v-US findings after glucocorticoid (GC) therapy. We report the transitions in the v-US findings in a case of GCA after GC therapy. The patient had rapidly progressive symptoms, and there were concerns about blindness. After GC therapy, we first observed improvement in headache and visual impairment symptoms within 1 week, followed by rapid improvement in laboratory findings within 2 weeks. Subsequently, there were improvements in v-US findings after more than 2 months. In conclusion, these findings showed a dissociation between improvements in clinical symptoms and v-US findings of the temporal artery. Additionally, this case suggests that regular examination of v-US findings is useful in evaluating GCA with evident vascular wall thickness before GC therapy.

A redox-active inorganic crown ether based on a polyoxometalate capsule.

Cation-uptake has been long researched as an important topic in materials science. Herein we focus on a molecular crystal composed of a charge-neutral polyoxometalate (POM) capsule [MoVI72FeIII30O252(H2O)102(CH3CO2)15]3+ encapsulating a Keggin-type phosphododecamolybdate anion [α-PMoVI12O40]3-. Cation-coupled electron-transfer reaction occurs by treating the molecular crystal in an aqueous solution containing CsCl and ascorbic acid as a reducing reagent. Specifically, multiple Cs+ ions and electrons are captured in crown-ether-like pores {MoVI3FeIII3O6}, which exist on the surface of the POM capsule, and Mo atoms, respectively. The locations of Cs+ ions and electrons are revealed by single-crystal X-ray diffraction and density functional theory studies. Highly selective Cs+ ion uptake is observed from an aqueous solution containing various alkali metal ions. Cs+ ions can be released from the crown-ether-like pores by the addition of aqueous chlorine as an oxidizing reagent. These results show that the POM capsule functions as an unprecedented "redox-active inorganic crown ether", clearly distinguished from the non-redox-active organic counterpart.

A multicentre study of clinical features and HLA typing in Japanese patients with ankylosing spondylitis.

OBJECTIVES: Due to the low prevalence of HLA-B27 and ankylosing spondylitis (AS) in Japan, rheumatologists have little experience with AS. We conducted a multicentre study to identify the characteristics and frequency of HLA-B types. METHODS: We analysed epidemiological and clinical data, blood tests, spine radiographs, and HLA-B types in Japanese AS patients. RESULTS: We evaluated 111 AS patients, predominantly men (82.9%). The mean age, disease onset, diagnosis, and time from onset to diagnosis were 43.7, 24.2, 36.0, and 11.6 years, respectively. Inflammatory low back pain was found in 96 cases (86.5%); peripheral arthritis in 59 (53.2%), enthesitis in 35 (31.5%), and dactylitis in 6 (5.4%). Extra-articular symptoms included uveitis, psoriasis, and inflammatory bowel disease in 41 (36.9%), 1 (0.9%), and 5 (4.5%) cases, respectively. HLA-B27 was positive in 83 cases (74.8%; odds ratio, 1146.0); and HLA-B48 in 9 (8.1%; odds ratio, 3.0). HLA-B27-positive patients were younger at onset and had a shorter diagnostic delay. CONCLUSIONS: AS clinical symptoms were almost the same as other countries except for the low coexistence of psoriasis. HLA-B27 positivity in Japanese patients was 78%. HLA-B27-positive patients were younger and diagnosed earlier. In addition to HLA-B27, a relationship with HLA-B48 was suggested.

Synthesis and Aerobic Oxidation Catalysis of Mesoporous Todorokite-Type Manganese Oxide Nanoparticles by Crystallization of Precursors.

The pursuit of a high surface area while maintaining high catalytic performance remains a challenge due to a trade-off relationship between these two features in some cases. In this study, mesoporous todorokite-type manganese oxide (OMS-1) nanoparticles with high specific surface areas were synthesized in one step by a new synthesis approach involving crystallization (i.e., solid-state transformation) of a precursor produced by a redox reaction between MnO4- and Mn2+ reagents. The use of a low-crystallinity precursor with small particles is essential to achieve this solid-state transformation into OMS-1 nanoparticles. The specific surface area reached up to ca. 250 m2 g-1, which is much larger than those (13-185 m2 g-1) for Mg-OMS-1 synthesized by previously reported methods including multistep synthesis or dissolution/precipitation processes. Despite ultrasmall nanoparticles, a linear correlation between the catalytic reaction rates of OMS-1 and the surface areas was observed without a trade-off relationship between particle size and catalytic performance. These OMS-1 nanoparticles exhibited the highest catalytic activity among the Mn-based catalysts tested for the oxidation of benzyl alcohol and thioanisole with molecular oxygen (O2) as the sole oxidant, including highly active ß-MnO2 nanoparticles. The present OMS-1 nanomaterial could also act as a recyclable heterogeneous catalyst for the aerobic oxidation of various aromatic alcohols and sulfides under mild reaction conditions. The mechanistic studies showed that alcohol oxidation proceeds with oxygen species caused by the solid, and the high surface area of OMS-1 significantly contributes to an enhancement of the catalytic activity for aerobic oxidation.

Base-Assisted Aerobic C-H Oxidation of Alkylarenes with a Murdochite-Type Oxide Mg6MnO8 Nanoparticle Catalyst.

Heterogeneously catalyzed aerobic oxidative C-H functionalization under mild conditions is a chemical process to obtain desired oxygenated products directly. Nanosized murdochite-type oxide Mg6MnO8 (Mg6MnO8-MA) was successfully synthesized by the sol-gel method using malic acid. The specific surface area reached up to 104 m2 g-1, which is about 7 times higher than those (2-15 m2 g-1) of Mg6MnO8 synthesized by previously reported methods. Mg6MnO8-MA exhibited superior catalytic performance to those of other Mn- and Mg-based oxides, including manganese oxides with Mn-O-Mn active sites for the oxidation of fluorene with molecular oxygen (O2) as the sole oxidant under mild conditions (40 °C). The present catalytic system was applicable to the aerobic oxidation of various substrates. The catalyst could be recovered by simple filtration and reused several times without obvious loss of its high catalytic performance. The correlation between the reactivity and the pKa of the substrates, basic properties of catalysts, and kinetic isotope effects suggest a basicity-controlled mechanism of hydrogen atom transfer. The 18O-labeling experiments, kinetics, and mechanistic studies showed that H abstraction of the hydrocarbon proceeds via a mechanism involving O2 activation. The structure of Mg6MnO8 consisting of isolated Mn4+ species located in a basic MgO matrix plays an important role in the present oxidation.

Template-Free Synthesis of Mesoporous ß-MnO2 Nanoparticles: Structure, Formation Mechanism, and Catalytic Properties.

Mesoporous ß-MnO2 nanoparticles were synthesized by a template-free low-temperature crystallization of Mn4+ precursors (low-crystallinity layer-type Mn4+ oxide, c-distorted H+-birnessite) produced by the reaction of MnO4- and Mn2+. The Mn starting materials, pH of the reaction solution, and calcination temperatures significantly affect the crystal structure, surface area, porous structure, and morphology of the manganese oxides formed. The pH conditions during the precipitation of Mn4+ precursors are important for controlling the morphology and porous structure of ß-MnO2. Nonrigid aggregates of platelike particles with slitlike pores (ß-MnO2-1 and -2) were obtained from the combinations of NaMnO4/MnSO4 and NaMnO4/Mn(NO3)2, respectively. On the other hand, spherelike particles with ink-bottle shaped pores (ß-MnO2-3) were formed in NaMnO4/Mn(OAc)2 with pH adjustment (pH 0.8). The specific surface areas for ß-MnO2-1, -2, and -3 were much higher than those for nonporous ß-MnO2 nanorods synthesized using a typical hydrothermal method (ß-MnO2-HT). On the other hand, c-distorted H+-birnessite precursors with a high interlayer metal cation (Na+ and K+) content led to the formation of α-MnO2 with a 2 × 2 tunnel structure. These mesoporous ß-MnO2 materials acted as effective heterogeneous catalysts for the aerobic oxidation of 5-hydroxymethylfurfural (HMF) to 2,5-furandicarboxylic acid (FDCA) as a bioplastic monomer and for the transformation of aromatic alcohols to the corresponding aldehydes, where the catalytic activities of ß-MnO2-1, -2, and -3 were approximately 1 order of magnitude higher than that of ß-MnO2-HT. ß-MnO2-3 exhibited higher catalytic activity (especially for larger molecules) than the other ß-MnO2 materials, and this is likely attributed to the nanometer-sized spaces.

Association of mucosal-associated invariant T cells with different disease phases of polymyalgia rheumatica.

OBJECTIVES: Although T cells are thought to be involved in the pathogenesis of PMR, whether innate-like T cells are involved in the process remains unknown. METHODS: The serum levels of 27 cytokines/chemokines in patients with PMR were measured by a multiplex immunoassay (Bio-Plex Assay). The cytokine-producing capacity of T and innate-like T cells was assessed by intracellular cytokine staining and flow cytometry. The frequency and activated status of T and innate-like T cells were investigated by flow cytometry and their associations with clinical parameters were assessed. RESULTS: The levels of inflammatory cytokines were associated with disease activity in PMR. The cytokine-producing capacity by CD8+ T and innate-like T cells was associated with disease activity. The frequency of HLA-DR+ CD38+ cells among CD8+ T cells was increased in patients with active disease. The frequencies of HLA-DR+ CD38+ cells among CD4+ T, mucosal-associated invariant T (MAIT) and γδ T cells were higher in patients with inactive disease. The frequency of HLA-DR+ CD38+ MAIT cells was associated with the PMR activity score and CRP levels in patients in remission. CONCLUSION: The inflammatory cytokine-producing capacity and expression of activation markers of CD8+ T and innate-like T cells were associated with the disease activity of PMR. MAIT cell activation in patients in remission may contribute to the subclinical activity of the disease.

One-pot aerobic oxidative sulfonamidation of aromatic thiols with ammonia by a dual-functional ß-MnO2 nanocatalyst.

High-surface-area ß-MnO2 (ß-MnO2-HS) nanoparticles could act as effective heterogeneous catalysts for the one-pot oxidative sulfonamidation of various aromatic and heteroaromatic thiols to the corresponding sulfonamides using molecular oxygen (O2) and ammonia (NH3) as respective oxygen and nitrogen sources, without the need for any additives.

Relationship between characteristics of spinopelvic alignment and quality of life in Japanese patients with ankylosing spondylitis: a cross-sectional study.

BACKGROUND: Studies on characteristic spinal deformities in Japanese patients with ankylosing spondylitis (AS) and data demonstrating a relationship between health-related quality of life (HRQOL) and spinopelvic alignment in these patients are lacking. METHODS: In this cross-sectional study, 50 patients with AS and without a surgical history, vertebral body fracture, or scoliosis as well as 30 control patients with degenerative lumbar kyphoscoliosis (DLKS) were included. Data collected included patient sex, age, spinopelvic parameters on sagittal full-spine standing radiographs, and HRQOL questionnaire responses. Student's t-test was used to compare the characteristics of spinopelvic parameters between the groups. A multiple regression analysis was performed to analyze correlations between spinopelvic parameters and HRQOL in the AS group. RESULTS: Global kyphosis (GK; T1-12 angle) was significantly greater in the AS group than in the DLKS group (P < 0.001), whereas the pelvic tilt (PT; posterior PT angle) was smaller in the AS group (P = 0.006). Radiographic parameters correlated with HRQOL in the AS group. Multiple regression analysis identified the sagittal vertical axis (SVA) and sacral slope (SS) as factors influencing the SRS-22 total score and SVA and GK as factors influencing Japanese Orthopaedic Association Back Pain Evaluation Questionnaire mental health (subdomain). CONCLUSIONS: Patients with AS did not use lumbar lordosis or posterior PT to compensate for their large thoracic kyphosis due to spinopelvic ankylosis. These patients showed a unique compensation pattern. The correlation/regression analysis revealed a correlation between radiographic parameters and HRQOL in patients with AS, with particular importance of SVA, SS, and GK for clinical results in AS.

Role of Cation Structure in CO2 Separation by Ionic Liquid/Sulfonated Polyimide Composite Membrane.

The development of suitable separation technologies for the separation of carbon dioxide is a pressing technological requirement. The application of ion gel membranes for this purpose continues to stimulate a great deal of research, and in this study we focus on the chemical structure of the ionic liquid component in the ion gel, and its interactions with the sulfonated polyimide polymer. Whilst such membranes are known to give promising carbon dioxide separation properties together with mechanical strength and thin-film-processability, we further elaborate on how changing the cation of the ionic liquid from a typical imidazolium cation to a protic variant effects the physicochemical, thermal, and structural properties of the membranes, and how these changes further influence the carbon dioxide separation properties. We compare and contrast our findings with our earlier study on protic and aprotic ammonium-based ionic liquids, and highlight that for CO2 absorption behavior in the imidazolium systems, the importance of directionality of interactions (ion pairs exhibit a large energy stabilization only for a specific geometrical arrangement of cation and anion, e.g., hydrogen bonding rather than Coulombic interaction) between cation and anion applies not only to the protic system, but also to the nominally aprotic cation. Finally, we demonstrate that the phase separation behavior in the ion gels is an important factor in determining the carbon dioxide separation behavior.

Effect of MnO2 Crystal Structure on Aerobic Oxidation of 5-Hydroxymethylfurfural to 2,5-Furandicarboxylic Acid.

Aerobic oxidation of 5-hydroxymethylfurfural (HMF) to 2,5-furandicarboxylic acid (FDCA) as a bioplastics monomer is efficiently promoted by a simple system based on a nonprecious-metal catalyst of MnO2 and NaHCO3. Kinetic studies indicate that the oxidation of 5-formyl-2-furancarboxylic acid (FFCA) to FDCA is the slowest step for the aerobic oxidation of HMF to FDCA over activated MnO2. We demonstrate through combined computational and experimental studies that HMF oxidation to FDCA is largely dependent on the MnO2 crystal structure. Density functional theory (DFT) calculations reveal that vacancy formation energies at the planar oxygen sites in α- and γ-MnO2 are higher than those at the bent oxygen sites. ß- and λ-MnO2 consist of only planar and bent oxygen sites, respectively, with lower vacancy formation energies. Consequently, ß- and λ-MnO2 are likely to be good candidates as oxidation catalysts. On the other hand, experimental studies reveal that the reaction rates per surface area for the slowest step (FFCA oxidation to FDCA) decrease in the order of ß-MnO2 > λ-MnO2 > γ-MnO2 ≈ α-MnO2 > δ-MnO2 > ε-MnO2; the catalytic activity of ß-MnO2 exceeds that of the previously reported activated MnO2 by three times. The order is in good agreement not only with the DFT calculation results, but also with the reduction rates per surface area determined by the H2-temperature-programmed reduction measurements for MnO2 catalysts. The successful synthesis of high-surface-area ß-MnO2 significantly improves the catalytic activity for the aerobic oxidation of HMF to FDCA.

Usefulness of Cardiac Magnetic Resonance in the Diagnosis of Löffler Endocarditis Secondary to Eosinophilic Granulomatosis with Polyangiitis.

A 40-year-old man who was diagnosed with bronchial asthma and eosinophilia was transferred to our hospital due to a worsening respiratory status. He was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA), and eosinophilic pneumoniae. Cardiac magnetic resonance (CMR) imaging indicated Löffler endocarditis. Treatment was initiated using intravenous methylprednisolone, cyclophosphamide, and heparin as anticoagulation therapy. Three months later, CMR showed the improvement of the LV myocardium. In this case, the early diagnosis of Löffler endocarditis by CMR could prevent systemic embolism and CMR was useful for assessing the curative effects of steroid and immunosuppressant therapy.

A case of ankylosing spondylitis with concurrent Takayasu arteritis.

We herein report a case involving a 56-year-old man who had experienced neck and lower back pain since the age of 23 years. Ankylosing spondylitis (AS) was diagnosed at 41 years of age, and treatment with sulfasalazine was initiated. At 44 years of age, the patient developed respiratory distress on exertion and chest pain. Aortic regurgitation (AR) was diagnosed via echocardiography, and the patient presented to our hospital for close examination and treatment. Coronary computed tomography angiography revealed no lesions in the coronary artery; however, magnetic resonance angiography revealed stenotic lesions in the left common carotid artery and left subclavian artery. Based on the findings of a physical examination, fundus examination, and blood tests, the patient was diagnosed with AS with concurrent Takayasu arteritis (TA). Upon administration of steroids to alleviate inflammation caused by an autoimmune mechanism, the patient's chest symptoms and inflammatory findings improved. AR was treated with aortic valve replacement and prosthetic blood vessel replacement, after which the patient progressed well. Intraoperative aortic biopsy revealed findings pathologically consistent with TA. Although AS with concurrent AR is well described, AS with concurrent TA, as in the present case, is rare.

PARI Regulates Stalled Replication Fork Processing To Maintain Genome Stability upon Replication Stress in Mice.

DNA replication is frequently perturbed by intrinsic, as well as extrinsic, genotoxic stress. At damaged forks, DNA replication and repair activities require proper coordination to maintain genome integrity. We show here that PARI antirecombinase plays an essential role in modulating the initial response to replication stress in mice. PARI is functionally dormant at replisomes during normal replication, but upon replication stress, it enhances nascent-strand shortening that is regulated by RAD51 and MRE11. PARI then promotes double-strand break induction, followed by new origin firing instead of replication restart. Such PARI function is apparently obstructive to replication but is nonetheless physiologically required for chromosome stability in vivo and ex vivo Of note, Pari-deficient embryonic stem cells exhibit spontaneous chromosome instability, which is attenuated by differentiation induction, suggesting that pluripotent stem cells have a preferential requirement for PARI that acts against endogenous replication stress. PARI is a latent modulator of stalled fork processing, which is required for stable genome inheritance under both endogenous and exogenous replication stress in mice.

Heterogeneously-Catalyzed Aerobic Oxidation of 5-Hydroxymethylfurfural to 2,5-Furandicarboxylic Acid with MnO2.

A simple non-precious-metal catalyst system based on costeffective and ubiquitously available MnO2 , NaHCO3 , and molecular oxygen was used to convert 5-hydroxymethylfurfural (HMF) to 2,5-difurandicarboxylic acid (FDCA) as a bioplastics precursor in 91 % yield. The MnO2 catalyst could be recovered by simple filtration and reused several times. The present system was also applicable to the aerobic oxidation of other biomass-derived substrates and the gram-scale oxidation of HMF to FDCA, in which 2.36 g (86 % yield) of the analytically pure FDCA could be isolated.

A Giant Gastrointestinal Stromal Tumor of the Stomach with Extramural Growth.

A 76-year-old man presented to our hospital with abdominal distention and loss of appetite. The 10% of weight lost relative to this patient in 1 month. Abdominal computed tomography and magnetic resonance imaging revealed a giant mass, with a major axis of 23 cm, containing solid components, not involving the upper abdominal organs. Esophagogastroduodenoscopy showed extramural compression from the middle gastric body to the antrum, as well as a normal mucosal surface. These findings were suggestive of a gastrointestinal stromal tumor attached to the anterior wall of the stomach without metastasis or invasion. Partial gastrectomy was performed for tumor resection, and the patient was subsequently treated with adjuvant imatinib. We report a rare case of a large extramural gastrointestinal stromal tumor of the stomach that was larger than 20 cm in diameter and present a pertinent literature review.

Involvement of Mucosal-associated Invariant T cells in Ankylosing Spondylitis.

OBJECTIVE: Ankylosing spondylitis (AS) is characterized by chronic inflammation of the axial and peripheral joints and ligamentous attachments. Gut immunity is thought to be involved in AS, because a prominent coexistence of gut and joint inflammation has been observed in patients with AS. Mucosal-associated invariant T (MAIT) cells are preferentially located in the gut lamina propria and produce inflammatory cytokines such as interleukin 17 (IL-17) and tumor necrosis factor-α (TNF-α), which are therapeutic targets for AS. This study aimed to investigate the involvement of MAIT cells in AS. METHODS: The frequency of MAIT cells and their cytokine production were determined in patients with AS and healthy controls (HC). The expression of a MAIT cell activation marker (CD69) was analyzed in patients with AS by using flow cytometry. RESULTS: The frequency of MAIT cells in the peripheral blood was lower in patients with AS compared with HC. The levels of IL-17 produced by MAIT cells after activation were higher in patients with AS than in the HC. CD69 expression on MAIT cells correlated with the Ankylosing Spondylitis Disease Activity Score in patients with AS. CONCLUSION: These results suggest the involvement of MAIT cells in the pathogenesis of AS.

A case of idiopathic portal hypertension associated with nodular regenerative hyperplasia-like nodule of the liver and mixed connective tissue disease.

A 51-year-old woman was diagnosed with mixed connective tissue disease (MCTD) in 2011. She underwent treatment with prednisolone. Her hepatobiliary enzyme level increased, and multiple nodules were found in both liver lobes in abdominal imaging studies. Ultrasonography revealed large and small hyperechoic lesions with indistinct or well-defined borders. No findings of classic hepatocellular carcinoma or liver cirrhosis were observed on contrast-enhanced computed tomography, but some nodules showed an enhanced effect of the central lesion that was characteristic of focal nodular hyperplasia (FNH) in an arterial phase. On gadolinium-ethoxybenzyl-diethylenetriamine penta-acetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging, slightly high-intensity nodules, 10-40mm in size, were observed on T1- and T2-weighted images. The nodules showed highest intensities in the hepatocyte phase and were enhanced with the uptake of Gd-EOB-DTPA as compared with the background liver. FNH was suspected based on the imaging findings, but we performed a liver tumor biopsy for differential diagnosis of the malignant lesion. Based on the immunohistopathological examination results, the final diagnosis was idiopathic portal hypertension associated with nodular regenerative hyperplasia (NRH)-like nodule of the liver. Benign nodular hepatocellular lesions are caused by abnormal hepatic circulation and were previously known as anomalous portal tract syndrome. Our case of atypical NRH with large nodules may be included in this disease entity. Here, we report a rare case of MCTD with NRH-like nodules and idiopathic portal hypertension with a review of literature.

Transient Elastography-Based Liver Profiles in a Hospital-Based Pediatric Population in Japan.

BACKGROUND & AIMS: The utility of transient elastography (FibroScan) is well studied in adults but not in children. We sought to assess the feasibility of performing FibroScans and the characteristics of FibroScan-based liver profiles in Japanese obese and non-obese children. METHODS: FibroScan examinations were performed in pediatric patients (age, 1-18 yr) who visited Osaka City University Hospital. Liver steatosis measured by controlled attenuation parameter (CAP), and hepatic fibrosis evaluated as the liver stiffness measurement (LSM), were compared among obese subjects (BMI percentile ≥ 90%), non-obese healthy controls, and non-obese patients with liver disease. RESULTS: Among 214 children examined, FibroScans were performed successfully in 201 children (93.9%; median, 11.5 yr; range, 1.3-17.6 yr; 115 male). CAP values (mean ± SD) were higher in the obese group (n = 52, 285 ± 60 dB/m) compared with the liver disease (n = 40, 202 ± 62, P < 0.001) and the control (n = 107, 179 ± 41, P < 0.001) group. LSM values were significantly higher in the obese group (5.5 ± 2.3 kPa) than in the control (3.9 ± 0.9, P < 0.001), but there were no significant differences in LSM between the liver disease group (5.4 ± 4.2) and either the obese or control group. LSM was highly correlated with CAP in the obese group (ρ = 0.511) but not in the control (ρ = 0.129) or liver disease (ρ = 0.170) groups. CONCLUSIONS: Childhood obesity carries a high risk of hepatic steatosis associated with increased liver stiffness. FibroScan methodology provides simultaneous determination of CAP and LSM, is feasible in children of any age, and is a non-invasive and effective screening method for hepatic steatosis and liver fibrosis in Japanese obese children.