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Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this article is to demonstrate the superiority of gevokizumab as compared to placebo, on top of current standard of care, in reducing the risk of Behçet's disease uveitis (BDU) exacerbations.Methods: Randomized, double-masked, placebo-controlled, parallel group, event-driven trial in BDU patients having recently experienced an ocular exacerbation, subsequently undergoing a tapering procedure from high-dose corticosteroids and receiving 60 mg gevokizumab or placebo every 4 weeks subcutaneously (EYEGUARD B-ClinicalTrials.gov NCT 01965145).Results: A total of 83 patients (40 gevokizumab, 43 placebo) were included. Gevokizumab did not significantly affect the risk of occurrence of ocular exacerbations. However, data suggested that gevokizumab could preserve visual acuity, reduce the uveitis severity, decrease the emergence of macular edema, and have a corticosteroid sparing effect. Gevokizumab was well tolerated.Conclusions: While the primary efficacy endpoint was not met with gevokizumab, the control of IL-1β pathway in patients with BDU may still be a relevant target. [ABSTRACT FROM AUTHOR]

The synthesis and characterization of peripherally ({1-[(Z or E)-phenylazo]-2-naphthyl} oxy) tetra-substituted metal-free and metallo (Zn(II), Co(II) and Cu(II)) phthalocyanines are described in this study. Aggregation properties of these compounds were investigated in the concentration range of 20–1 μM in tetrahydrofuran. Spectroscopic and photophysical properties of these phthalocyanines have been investigated in tetrahydrofuran. Thin films of metallophthalocyanines were prepared by spin coating technique. Results show that transmittance values of the films change with respect to the metal ions in the visible and Q absorptions regions. Films were also annealed at 100, 200 and 300 °C and their optical properties were investigated as a function of annealing temperature. The spectrophotometric measurements of transmittance spectra were carried out in the wavelength range 200–1000 nm. Results showed that transmittance values of ZnPc thin films were increased as a result of heat treatment. [ABSTRACT FROM AUTHOR]

Objective. Familial Mediterranean fever (FMF) is the most common form of autoinflammatory diseases. We aimed to evaluate the small bowel mucosa by capsule endoscopy (CE) in FMF patients for investigation of other possible causes of abdominal pain. Material and methods. The study group consisted of 41 patients with FMF. A standard questionnaire was used to record the gastrointestinal symptoms, other clinical findings, Mediterranean fever gene (MEFV) mutations, and history of medications including non-steroidal anti-inflammatory drugs (NSAIDs). Gastroscopy, colonoscopy and small bowel CE were performed in all patients, and biopsies were taken from terminal ileum and duodenum. Results. The mean age of the patients was 34 ± 11 years, 63% of them were female, and 76.5% of them were carrying MEFV exon 10 mutations. Only one patient used NSAIDs in addition to colchicine. In endoscopic investigations, gastric erosion was detected in only one patient, and no significant findings were detected in colonoscopy. CE showed small bowel mucosal defects in 44% (erosions in 26.8%, ulcer in 17.1%) and edema in 29.3% of the patients. Most (64%) of the ulcer and erosions were localized to jejunum, and only 24% were in ileum. Mitotic changes as an indirect finding of colchicine toxicity were not different from the changes observed in samples of independent group of patients with irritable bowel syndrome. Conclusion. Mucosal defect was observed in half of the FMF patients, which may be associated with underlying inflammation or chronic colchicine exposure. Detection of nonspecific chronic inflammation without mitotic changes supports that mucosal defects may be associated with the autoinflammatory process. [ABSTRACT FROM AUTHOR]

In this study, the syntheses and characterization of [2,3,7,8,12,13,17,18-octakis [2′-(3-quinolincarboxy)ethylhio] porphyrazinato N21N22N23N24] magnesium(II), quaternized [2,3,7,8,12,13,17,18-octakis [2′-(3-quinolincarboxy)ethylhio] porpyrazinato N21N22N23N24] magnesium(II), [2,3,7,8,12,13,17,18-octakis[2′-(3-quinolinecarboxy)-ethylthio]21H23H N21N22N23N24porphyrazine and [2,3,7,8,12,13,17,18-octakis[2′-(3-quinolinecarboxy)-ethylthio]porphyrazinato N21N22N23N24} zinc are described. The new compounds have been characterized by elemental analysis, FT-IR,1H-NMR, and mass spectra. General trends are described for fluorescence quantum yields of these compounds in dimethylsulfoxide (DMSO). In this study, the effects quaternization of the substituents on the photophysical and spectroscopic properties are also reported. [ABSTRACT FROM PUBLISHER]

Co(II) and Cu(II) complexes and metal-free t-butylphenyl peripherally substituted porphyrazine (Pz) have been screened for in vitro antifungal (Aspergillus niger) and antioxidant (free radical scavenging, superoxide radical scavenging, and reducing power) activities. The results were compared with synthetic antioxidants, e.g., butylated hydroxyanisole (BHA), butylated hydroxytoluene (BHT), trolox, or α-tocopherol. The free radical scavenging activity of H2Pz was higher than the CuPz complex. However, CuPz complex showed higher superoxide radical scavenging activity than BHA, BHT, and trolox while H2Pz and CoPz showed weaker activity than BHA, BHT, and trolox. The reducing power of all complexes was similar to that of BHT and α-tocopherol on a per molar basis. The ligand and complexes have antifungal activity against A. niger. The compounds have significant superoxide radical scavenging activity against various antioxidant systems in vitro. [ABSTRACT FROM AUTHOR]

We report two patients who suffered from symmetrical polyarthritis simulating rheumatoid arthritis. Acute phase response was almost within normal limits, and autoantibodies including rheumatoid factor were negative. Both of them were diagnosed as having amyloid arthropathy (AmyA) secondary to kappa multiple myeloma based on deposition of κ-light chain-immunoreactive amyloid in biopsied tissue and Bence Jones protein in urine. Systemic AL amyloidosis may be important in the differential diagnosis of chronic polyarthralgia. [ABSTRACT FROM AUTHOR]

Objective. Amyloid A protein quantification in fat tissue is a new immunochemical method for detecting AA amyloidosis, a rare but serious disease. The objective was to assess diagnostic performance in clinical AA amyloidosis. Methods. Abdominal subcutaneous fat tissue of patients with AA amyloidosis was studied at the start of an international clinical trial with eprodisate (NC-503; 1,3-propanedisulfonate; Kiacta™), an antiamyloid compound. All patients had renal findings, i.e. proteinuria (≥1 g/day) or reduced creatinine clearance (20 - 60 ml/min). Controls were patients with other types of amyloidosis and arthritic patients without amyloidosis. Amyloid A protein was quantified by ELISA using monoclonal antihuman serum amyloid A antibodies. Congo red stained slides were scored by light microscopy in a semiquantitative way (0 to 4+). Results. Ample fat tissue (>50 mg) was available for analysis in 154 of 183 patients with AA amyloidosis and in 354 controls. The sensitivity of amyloid A protein quantification for detection of AA amyloidosis (>11.6 ng/mg fat tissue) was 84% (95% CI: 77 - 89%) and specificity 99% (95% CI: 98 - 100%). Amyloid A protein quantification and semiquantitative Congo red scoring were concordant. Men had lower amyloid A protein values than women (p < 0.0001) and patients with familial Mediterranean fever had lower values than patients with arthritis (p < 0.001) or other inflammatory diseases (p < 0.01). Conclusions. Amyloid A protein quantification in fat tissue is a sensitive and specific method for detection of clinical AA amyloidosis. Advantages are independence from staining quality and observer experience, direct confirmation of amyloid AA type, and potential for quantitative monitoring of tissue amyloid over time. [ABSTRACT FROM AUTHOR]

Supramolecular porphyrazines with eight pyridine donor groups bound through ethylthio ester bridges on the periphery have been prepared. The pyridine donors were quaternized with iodomethane to octacationic porphyrazine. The nonanuclear supramolecular porphyrazine was prepared by the coordination of peripheral pyridine donors with VO(acac)2. The paramagnetic nonanuclear structure was studied in powder and in solution form by EPR. EPR studies together with the other spectral data confirmed the presence of identical pyridine-coordinated VO(acac)2 paramagnetic centers attached to the peripheral positions of the porphyrazine core. [ABSTRACT FROM AUTHOR]

In the present study we describe a lutetium(III) bis(phthalocyaninate) complex which contains eight acetylbenzo(15-crown-5) units. The starting compound was diethyl (3,4-dicyanophenyl)acetoxybenzo(15-crown-5)malonate (1). The synthesis of the desired compound was accomplished by the reaction of the o-dicyano compound (1) with lutetium acetate in hexanol in the presence of 1,8-diazabicyclo[5,4,0]undec-7-ene (DBU) as a strong base. The ESR spectrum confirmed the radical nature of the complex. [ABSTRACT FROM AUTHOR]

Octakis(hexylthio)vanadyl porphyrazinate, (VOPz), was prepared from vanadyl(IV) sulfate and metal-free porphyrazine (H2Pz), which was synthesized by the cyclotetramerization of the corresponding dinitrile derivative and further treatment with trifluoroacetic acid. Intense absorption peaks at 699 and 352 nm in the electronic spectrum indicated the C4v symmetry of VOPz. ESR studies, both in solution and in powder form, confirmed the proposed structure. [ABSTRACT FROM AUTHOR]

Chlorooctakis(octylthio)porphyrazinatoiron(III) (FePzCl) was prepared by the reaction of metal-free porphyrazine with iron(II) acetate and further treatment with HCl solution. The monomeric bisaxial complex FePz(py)2 as well as the bridged complex [FePz(pyz)]n were formed as stable complexes by reacting FePzCl with pyridine or pyrazine, respectively. These complexes were characterized by elemental analysis and spectroscopic methods. [ABSTRACT FROM AUTHOR]