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Yutaka Kozu, Yusuhiro Gon, Yuki Takano, Takao Noguchi, Shinichi Hayashi, Fumio Kumasawa, Kenji Mizumura, Shuichiro Maruoka, Shu Hashimoto
A 77-year-old man visited our hospital with chief complaints of difficulty in hearing, nasal discharge and fever. The patients was diagnosed with otitis media, and his fever continued at approximately 38°C despite the administration of clarithromycin. After that, dyspnea on exertion developed and chest X-ray examination indicated multiple infiltrative shadows. PR3-ANCA levels were high (238-fold of the normal levels), and granulomatosis with polyangiitis (GPA) was diagnosed on the basis of clinical symptoms, laboratory results, and pathological findings...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
J M Clain, A M Hummel, J H Stone, F C Fervenza, G S Hoffman, C G M Kallenberg, C A Langford, W J McCune, P A Merkel, P A Monach, P Seo, R F Spiera, E W St Clair, S R Ytterberg, And U Specks
Antineutrophil cytoplasmic antibodies (ANCA) appear to play an important role in the pathogenesis of ANCA-associated vasculitis (AAV). Yet, ANCA alone are not sufficient to generate disease, and some evidence suggests that infectious triggers may serve as inciting events for AAV disease activity. Antibodies of the IgM isotype often serve as markers of recent infection, and IgM ANCA have previously been identified in patients with AAV, though the frequency and clinical relevance of IgM ANCA is not well established...
January 11, 2017: Clinical and Experimental Immunology
Yuki Kirito, Daisuke Yamamoto, Tsuyoshi Uchiyama
A 72-year-old man with ulcerative colitis (UC) presented with complete left abducens nerve palsy. Although MRI showed no significant changes, cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin (IL)-6 levels. His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31.1 U/mL, but granulomatosis with polyangiitis was not observed. On the basis of the diagnosis of autoimmune cranial neuropathy, he was treated with steroid therapy. While tapering steroid therapy, his serum PR3-ANCA levels; cerebrospinal fluid findings, including IL-6 levels; and symptoms improved...
January 9, 2017: BMJ Case Reports
Mélanie Givaudan, Frédéric Vandergheynst, Patrick Stordeur, Annick Ocmant, Christian Melot, Valérie Gangji, Muhammad S Soyfoo
OBJECTIVE: Determine the frequency of granulomatosis with polyangiitis (GPA) associated with non-identified ANCA (non-MPO, non-PR3 ANCA) and secondarily compare their clinic with GPA associated with MPO-positive or PR3-positive ANCA. METHODS: In a monocentric retrospective observational study, clinical data of 398 patients with non-identified ANCA (titer of ANCA at least 1/80 by immunofluorescence on ethanol fixed PMN) was gathered over a period of 6 years. GPA patients from this population were compared with GPA patients with identified ANCA on the basis of clinical, biological, immunological and histological features...
January 9, 2017: Acta Clinica Belgica
E Csernok, N Kempiners, B Hellmich
BACKGROUND: Up to now indirect immunofluorescence (IIF) followed by an antigen-specific assay specific for proteinase 3 (PR3) or myeloperoxidase (MPO) has been the standard method for the detection of antineutrophil cytoplasmic antibodies (ANCA). The development of more sensitive and highly specific PR3-ANCA and MPO-ANCA immunoassays for the diagnosis of ANCA-associated vasculitis (AAV) has raised doubts about the two-stage diagnostic strategy currently recommended for ANCA detection...
January 5, 2017: Zeitschrift Für Rheumatologie
Anja Kerstein, Konstanze Holl-Ulrich, Antje Müller, Gabriela Riemekasten, Peter Lamprecht
Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA)...
January 2017: Deutsche Medizinische Wochenschrift
Alexander F R Kilpatrick, F Geoffrey N Cloke
We report the synthesis and structural characterisation of three homobimetallic complexes featuring divalent lanthanide metals (Ln = Yb, Eu and Sm) bridged by the silylated pentalene ligand [1,4-{Si(i)Pr3}2C8H4](2-) (= Pn(†)). Magnetic measurements and cyclic voltammetry have been used to investigate the extent of intermetallic communication in these systems, in the context of molecular models for organolanthanide based conducting materials.
January 5, 2017: Dalton Transactions: An International Journal of Inorganic Chemistry
Amy Hughes, Jade Clarson, Carine Tang, Ljiljana Vidovic, Deborah L White, Timothy P Hughes, Agnes S M Yong
Immunological control may contribute to achievement of deep molecular response in chronic myeloid leukemia (CML) patients on tyrosine kinase inhibitor (TKI) therapy and may promote treatment free remission (TFR). We investigated effector and suppressor immune responses in CML patients at diagnosis (n=21), on TKI (imatinib, nilotinib, dasatinib) prior to achieving major molecular response (pre-MMR, BCR-ABL1 >0.1%, n=8), MMR (BCR-ABL1 ≤0.1%, n=20), molecular response(4.5) (MR(4.5), BCR-ABL1 ≤0.0032%, n=16) and sustained TFR (BCR-ABL1 undetectable following cessation of TKI therapy, n=13)...
January 3, 2017: Blood
Anja Kerstein, Silke Schüler, Otávio Cabral-Marques, Juliane Fazio, Robert Häsler, Antje Müller, Silke Pitann, Frank Moosig, Sebastian Klapa, Christian Haas, Dieter Kabelitz, Gabriela Riemekasten, Steffen Wolters, Peter Lamprecht
Autoimmune diseases are initiated by a combination of predisposing genetic and environmental factors resulting in self-perpetuating chronic inflammation and tissue damage. Autoantibody production and an imbalance of effector and regulatory T-cells are hallmarks of autoimmune dysregulation. While expansion of circulating effector memory T-cells is linked to disease pathogenesis and progression, the causes driving alterations of the peripheral T-cell compartment have remained poorly understood so far. In granulomatosis with polyangiitis (GPA), a prototypical autoimmune disorder of unknown aetiology, we performed for the first time a combined approach using phenotyping, transcriptome and functional analyses of T-cell populations to evaluate triggers of memory T-cell expansion...
December 28, 2016: Journal of Autoimmunity
Frank B Cortazar, William F Pendergraft, Julia Wenger, Charles T Owens, Karen Laliberte, John L Niles
OBJECTIVE: To evaluate the effect of rituximab on pathogenic autoantibodies and total immunoglobulin levels, and to identify serious adverse events, in patients with ANCA vasculitis treated with continuous B cell depletion. METHODS: We conducted a retrospective analysis of 239 patients with ANCA vasculitis treated with rituximab-induced continuous B cell depletion. Two treatment cohorts were analyzed: an induction group (n= 53) and a maintenance group (n=237). The change in ANCA titers and total immunoglobulin levels over time were evaluated using mixed-effects models...
December 28, 2016: Arthritis & Rheumatology
Fabian Geist, Andrej Jackel, Peter Irmler, Michael Linseis, Sabine Malzkuhn, Martin Kuss-Petermann, Oliver S Wenger, Rainer F Winter
We report on the platinum complexes trans-Pt(BODIPY)(8-ethynyl-BODIPY)(PEt3)2 (EtBPtB) and trans-Pt(BODIPY)(4-ethynyl-1,8-naphthalimide)(PR3)2 (R = Et, EtNIPtB-1; R = Ph, EtNIPtB-2), which all contain two different dye ligands that are connected to the platinum atom by a direct σ bond. The molecular structures of all complexes were established by X-ray crystallography and show that the different dye ligands are in either a coplanar or an orthogonal arrangement. π-stacking and several CH···F and short CH···π interactions involving protons at the phosphine substituents lead to interesting packing motifs in the crystal...
December 27, 2016: Inorganic Chemistry
Hai-Lou Li, Ya-Jie Liu, Jing-Lin Liu, Li-Juan Chen, Junwei Zhao, Guo-Yu Yang
Three types of serine-decorated rare-earth containing arsenotungstates [H2N(CH3)2]6NaH[RE2W4O10(H2O)8(ser)2(B-α-AsW9 O33)2]*30H2O (RE3+ = Eu3+, Gd3+, Tb3+, Dy3+, Ho3+, Er3+, Tm3+, Yb3+, Y3+) (1), [H2N(CH3)2]6Na6-xRExH4-2x[RE4W8O19(H2O)10+y(OH)2(ser)2(B-α-AsW9 O33)4]*nH2O (RE3+ = Tb3+, x = 1, y = 2, n = 36; RE = Dy3+, Ho3+, Er3+, Yb3+, Y3+, x = 0, y = 0, n = 38; RE3+ = Tm3+, x = 1, y = 0, n = 38) (2) and [H2N(CH3)2]6-2xNa2+3xRExH10-6x+y[RE4W8O19(H2O)8 (OH)2(ser)4(B-α-AsW9O33)4]*Cly*nH2O (RE3+ = Ce3+, Pr3+, x = 1, y = 0, n = 65; RE3+ = Nd3+, Sm3+, x = 0, y = 0, n = 65; RE3+ = Eu3+, Gd3+, x = 1, y = 2, n = 45) (3) were synthesized in the participation of two organic solubilizers of dimethylamine hydrochloride and L-serine and structurally characterized...
December 16, 2016: Chemistry: a European Journal
Max M Hansmann, Guy Bertrand
(Phosphino)phosphaketenes (>P-P═C═O) behave as (phosphino)phosphinidene-carbonyl adducts (>P-P←:C═O). CO scrambling was observed using (13)C labeled CO, and exchange reactions with phosphines afford the corresponding (phosphino)phosphinidene-phosphine adducts (>P-P←:PR3). The latter react with isonitriles and singlet carbenes giving (phosphino)phosphinidene-isonitrile (>P-P←:CNR) and -carbene adducts (>P-P←:C<). Based on experimental results and DFT calculations, it is shown that these "ligand" exchange reactions occur via an associative mechanism as classically observed with transition metal complexes...
December 14, 2016: Journal of the American Chemical Society
Tao Li, Houjia Wei, Yong Fang, Lei Wang, Sheng Chen, Zaichao Zhang, Yue Zhao, Gengwen Tan, Xinping Wang
One-electron oxidation of the stibines Aryl3 Sb (1, Aryl=2,6-(i) Pr2 -4-OMe-C6 H2 ; 2, Aryl=2,4,6-(i) Pr3 -C6 H2 ) with AgSbF6 and NaBAryl(F)4 (Aryl(F) =3,5-(CF3 )2 C6 H3 ) afforded the first structurally characterized examples of antimony-centered radical cations 1(.+) [BAryl(F)4 ](-) and 2(.+) [BAryl(F)4 ](-) . Their molecular and electronic structures were investigated by single-crystal X-ray diffraction, electron paramagnetic resonance spectroscopy (EPR) and UV/Vis absorption spectroscopy, in conjunction with theoretical calculations...
January 9, 2017: Angewandte Chemie
Fenna F van de Watering, Martin Lutz, Wojciech I Dzik, Bas de Bruin, Joost N H Reek
Finding new catalysts for the release of molecular hydrogen from methanol is of high relevance in the context of the development of sustainable energy carriers. Herein, we report that the ruthenium complex Ru(salbinapht)(CO)(Pi-Pr3) {salbinapht=2-[({2'-[(2-hydroxybenzyl)amino]-[1,1'-binaphthalen]-2-yl}imino)methyl]phenolato} (2) catalyzes the methanol dehydrogenation reaction in the presence of base and water to yield H2, formate, and carbonate. Dihydrogen is the only gas detected and a turnover frequency up to 55 h(-1) at 82 °C is reached...
September 7, 2016: ChemCatChem
Kazuya Hirai, Naoto Miura, Masabumi Yoshino, Kanyu Miyamoto, Hironobu Nobata, Takuhito Nagai, Keisuke Suzuki, Shogo Banno, Hirokazu Imai
We herein report two cases of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA)-related nephritis in infectious endocarditis. In both cases, the patients were middle-aged men with proteinuria and hematuria, hypoalbuminemia, decreased kidney function, anemia, elevated C-reactive protein (CRP) levels, and PR3-ANCA positivity. Each had bacteremia, due to Enterococcus faecium in one and Streptococcus bovis in the other. One patient received aortic valve replacement therapy for aortic regurgitation with vegetation, and the other underwent tricuspid valve replacement therapy and closure of a ventricular septic defect to treat tricuspid regurgitation with vegetation...
2016: Internal Medicine
Jun-Cheng Zhang, Yan-Jie Liu, Xu Yan, Hong-Di Zhang, Jun Zhang, Xiaoxiong Wang, Wen-Peng Han, Yun-Ze Long, Xin-Yuan Sun
Self-activated phosphors are capable of generating optical emissions from the internal ion groups of host lattice before externally introducing luminescent ions. However, numerous self-activated phosphors only show luminescence at low temperature due to the thermally activated energy migration among ion groups at room temperature, severely confining their application conditions. In this letter, we propose a strategy to converting the low-temperature luminescence to a room-temperature one through changing the synthesis conditions to induce structural distortions and thus to limit energy migration...
November 30, 2016: Chemphyschem: a European Journal of Chemical Physics and Physical Chemistry
Marie-Cécile Dufour, Noël Magnin, Bernard Dumas, Sophie Vergnes, Marie-France Corio-Costet
BACKGROUND: The fight against grapevine diseases due to biotrophic pathogens usually requires the massive use of chemical fungicides with harmful environmental effects. An alternative strategy could be the use of compounds able to stimulate plant immune responses which significantly limit the development of pathogens in laboratory conditions. However, the efficiency of this strategy in natura is still insufficient to be included in pest management programs. To understand and to improve the mode of action of plant defense stimulators in the field, it is essential to develop reliable tools that describe the resistance status of the plant upon treatment...
November 22, 2016: BMC Genomics
Yasuhiro Ohki, Yuki Shimizu, Ryoichi Araake, Mizuki Tada, W M C Sameera, Jun-Ichi Ito, Hisao Nishiyama
A square-planar Co4 amide cluster, Co4 {N(SiMe3 )2 }4 (2), and an octahedral Co6 hydride cluster, Co6 H8 (P(i) Pr3 )6 (4), were obtained from metathesis-type amide to hydride exchange reactions of a Co(II) amide complex with pinacolborane (HBpin) in the absence/presence of P(i) Pr3 . The crystal structure of 4 revealed face-capping hydrides on each triangular [Co3 ] face, while the formal Co(II)2 Co(I)4 oxidation state of 4 indicated a reduction of the cobalt centers during the assembly process. Cluster 4 catalyzes the hydrosilylation of 2-cyclohexen-1-one favoring the conjugate reduction...
December 19, 2016: Angewandte Chemie
Mandy Sowa, Barbara Trezzi, Rico Hiemann, Peter Schierack, Kai Grossmann, Juliane Scholz, Valentina Somma, Renato Alberto Sinico, Dirk Roggenbuck, Antonella Radice
Rapidly progressive glomerulonephritis (RPGN) is mainly caused by anti-glomerular basement membrane (GBM) antibody-mediated glomerulonephritis, immune-complex or anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides and leads to rapid loss of renal function. Detection of ANCA and autoantibodies (autoAbs) to GBM and dsDNA enables early diagnosis and appropriate treatment of RPGN aiding in preventing end-stage renal disease.Determination of ANCA on neutrophils (ANCA) as well as autoAbs to myeloperoxidase (MPO-ANCA), proteinase 3 (PR3-ANCA), GBM, and dsDNA was performed by the novel multiplex CytoBead technology combining cell- and microbead-based autoAb analyses by automated indirect immunofluorescence (IIF)...
November 2016: Medicine (Baltimore)
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