Presence Of ANA Research Articles

Exploring the Light-Emitting Agents in Renilla Luciferases by an Effective QM/MM Approach.

Bioluminescence is a fascinating natural phenomenon, wherein organisms produce light through specific biochemical reactions. Among these organisms, Renilla luciferase (RLuc) derived from the sea pansy Renilla reniformis is notable for its blue light emission and has potential applications in bioluminescent tagging. Our study focuses on RLuc8, a variant of RLuc with eight amino acid substitutions. Recent studies have shown that the luminescent emitter coelenteramide can adopt multiple protonation states, which may be influenced by nearby residues at the enzyme's active site, demonstrating a complex interplay between protein structure and bioluminescence. Herein, using the quantum mechanical consistent force field method and the semimacroscopic protein dipole-Langevin dipole method with linear response approximation, we show that the phenolate state of coelenteramide in RLuc8 is the primary light-emitting species in agreement with experimental results. Our calculations also suggest that the proton transfer (PT) from neutral coelenteramide to Asp162 plays a crucial role in the bioluminescence process. Additionally, we reproduced the observed emission maximum for the amide anion in RLuc8-D120A and the pyrazine anion in the presence of a Na+ counterion in RLuc8-D162A, suggesting that these are the primary emitters. Furthermore, our calculations on the neutral emitter in the engineered AncFT-D160A enzyme, structurally akin to RLuc8-D162A but with a considerably blue-shifted emission peak, aligned with the observed data, possibly explaining the variance in emission peaks. Overall, this study demonstrates an effective approach to investigate chromophores' bimolecular states while incorporating the PT process in emission spectra calculations, contributing valuable insights for future studies of PT in photoproteins.

Cation/Anion-Dual regulation in Na3MnTi(PO4)3 cathode achieves the enhanced electrochemical properties of Sodium-Ion batteries

Na3MnTi(PO4)3 (NMTP) emerges as a promising cathode material with high-performance for sodium-ion batteries (SIBs). Nevertheless, its development has been limited by several challenges, including poor electronic conductivity, the Mn3+ Jahn-Teller effect, and the presence of a Na+/Mn2+ cation mixture. To address these issues, we have developed a cation/anion-dual regulation strategy to activate the redox reactions involving manganese, thereby significantly enhancing the performance of NMTP. This strategy simultaneously enhances the structural dynamics and facilitates rapid ion transport at high rates by inducing the formation of sodium vacancy. The combined effects of these modifications lead to a substantial improvement in specific capacity (79.1 mAh/g), outstanding high-rate capabilities (35.9 mAh/g at 10C), and an ultralong cycle life (only 0.040 % capacity attenuation per cycle over 250 cycles at 1C for Na3.34Mn1.2Ti0.8(PO3.98F0.02)3) when used as a cathode material in SIBs. Furthermore, its performance in full cell demonstrates impressive rate capability (44.4 mAh/g at 5C) and exceptional cycling stability (with only 0.116 % capacity decay per cycle after 150 cycles at 1C), suggesting its potential for practical applications. This work presents a dual regulation strategy targeting different sites, offering a significant advancement in the development of NASICON phosphate cathodes for SIBs.

Two-pore channels regulate endomembrane tension to enable remodeling and resolution of phagolysosomes

Phagocytes promptly resolve ingested targets to replenish lysosomes and maintain their responsiveness. The resolution process requires that degradative hydrolases, solute transporters, and proteins involved in lipid traffic are delivered and made active in phagolysosomes. It also involves extensive membrane remodeling. We report that cation channels that localize to phagolysosomes were essential for resolution. Specifically, the conductance of Na+ by two-pore channels (TPCs) and the presence of a Na+ gradient between the phagolysosome lumen and the cytosol were critical for the controlled release of membrane tension that permits deformation of the limiting phagolysosome membrane. In turn, membrane deformation was a necessary step to efficiently transport the cholesterol extracted from cellular targets, permeabilizing them to hydrolases. These results place TPCs as regulators of endomembrane remodeling events that precede target degradation in cases when the target is bound by a cholesterol-containing membrane. The findings may help to explain lipid metabolism dysfunction and autophagic flux impairment reported in TPC KO mice and establish stepwise regulation to the resolution process that begins with lysis of the target.

Effect of biosolid-derived dissolved organic matter on orthophosphate sorption to soils depends on clay mineralogy and solution composition.

Dissolved organic matter (DOM) from biosolids can alter the sorption of orthophosphate (inorganic phosphorus (IP)) to soils and, therefore, affect the bioavailability of IP. It is not clear how clay mineralogy and solution composition interfere with DOM effects on IP sorption by soils. Hence, we studied the effect of DOM on IP sorption to five semi-arid soils dominated by either illite/smectite (I/S) or kaolinite clays. IP sorption isotherms were constructed in either NaCl or CaCl2 background solution, with and without the addition of DOM. The IP sorption capacity maxima (SMAX, Langmuir model) of the I/S soils were 33-102% higher in the presence of CaCl2, as compared to NaCl. Although DOM had no effect on the IP-SMAX in the presence of CaCl2, it increased the IP-SMAX by 35-59% in the presence of the NaCl solution. Surprisingly, DOM sorption to the I/S soils was 30-90% greater in the presence of a Na+-dominated solution, as compared to a Ca2+-dominated solution. In contrast to the I/S soils, the SMAX of the kaolinitic soil was not affected by the background electrolyte (Na+, Ca2+) or the addition of DOM. Furthermore, the addition of IP reduced the sorption of DOM to the kaolinitic soil (by up to 50%) in both background electrolyte solutions. These results highlight the contrasting roles of divalent and monovalent cations in conjunction with DOM in IP sorption to semi-arid I/S soils. We propose a new approach based on two conceptual mechanisms to explain the DOM's enhancement of IP sorption to I/S soils. (1) Under dispersion conditions in the Na+-dominated solutions, Ca2+-mediated DOM-IP complexes bind to the clay's negative planar surfaces. (2) Under flocculation conditions in the Ca+-dominated solutions, the distance between adjacent platelets decreases, reducing both the electronegative charge spillover and Ca2+ bridge-mediated DOM sorption. In contrast, the addition of DOM to kaolinite, a multi-platelet clay with a low isomorphic negative charge, reduces IP sorption due to competitive sorption on the clay's broken edges.

P-505 Role of intramuscular immunoglobulin in improving the live birth rate in ANA positive women with recurrent pregnancy loss

Abstract Study question In ANA positive women with Recurrent Pregnancy Loss, could addition of intramuscular Immunoglobulin along with appropriate adjuvant therapy improve the live birth rate ? Summary answer In ANA positive RPL patients, addition of Intramuscular immunoglobulin along with glucocorticoids and low dose aspirin improves the live birth rate What is known already RPL is a frustrating condition which affects 1-3% of women trying to conceive. Presence of ANA is associated with autoimmunity, which affects trophoblast development and vascular remodeling process thereby resulting in pregnancy loss. Latest ESHRE guidelines also suggest ANA testing for explanatory purpose. Aspirin has anti-platelet and anti-inflammatory effects, and glucocorticoids exhibit a beneficial effect in autoimmune diseases. Therefore, they are considered conventional adjuvant therapies in ANA-positive women with RPL. Addition of Immunoglobulin for RPL patients benefits by the down-regulation of primary antibody production, the modulation of complement activation and Suppresses Nk cell cytotoxicity Study design, size, duration 72 ANA positive patients with Unexplained RPL were enrolled in the study. Anatomical, metabolic ,genetic and other immunological causes for RPL were excluded. Out of 72 patients 36 patients were allotted to the treatment group, who received intramuscular immunoglobulins along with Low dose aspirin and glucocorticoids, remaining 36 patients were allotted to the control group, who received only Aspirin and glucocorticoids. Duration of study extended from jan2021 till Dec 2022 Participants/materials, setting, methods 72 patients with unexplained RPL underwent ANA screening using Indirect Immunofluorescence technique in the pre conception period. Patients with a titre ≥1:80 were considered to be significant and enrolled in the study. Patients were allotted to the Treatment and Control group based on personal choice of the patient after informed consent. Live birth rate was the primary outcome analyzed. Main results and the role of chance There were no significant differences in socio-demographic characteristics between group. Patient characteristics – Age, BMI, Ovarian reserve, sperm factor and number of previous Pregnancy losses were matched.Treatment group who received Intramuscular immunoglobulin once in 3 weeks right from the time of positive pregnancy test till 28 weeks along with low dose aspirin and glucocorticoids had a live birth rate of (23/36 ) 63.8 % whereas Control group who received only Low dose Aspirin (75 mg) and Glucocorticoids had a live birth rate of 41.6 % (15/36). Other obstetric outcomes which were analysed were Pre Eclampsia and GDM which were comparable between both the groups.Preterm labour was marginally higher in the control group. Limitations, reasons for caution Because of limited sample size results need to be interpreted with caution. Screening for ANA antibodies in patients were done only preconceptionally, and we were not aware about their ANA titres in pregnancy. This study involves only patients who conceived with IVF treatment and doesn't include spontaneous natural conception Wider implications of the findings ANA screening in RPL cases is suggested to prognosticate the outcome. Addition of Immunoglobulins significantly increases the live birth rate in these cases. Role of Immunoglobulin in patients with altered cellular immunity has to be further studied which is likely to benefit other cases of unexplained RPL. Trial registration number not applicable

The associations between myositis autoantibodies and clinical presentations in dermatomyositis.

The myositis autoantibodies have been widely used clinically in recent years for the identification of an autoantibody-associated clinical phenotype in dermatomyositis (DM) patients. However, correlations between myositis autoantibodies and clinical presentations in different populations are lacking, especially in Taiwan. To investigate the correlations among cutaneous manifestations, myositis autoantibodies, and systemic diseases, including interstitial lung disease (ILD) and internal malignancy. A retrospective study of patients with histopathologically confirmed cutaneous manifestations of DM was conducted during 2005 to 2020 in Taiwan. A commercial line blot immunoassay technique was used to detect myositis autoantibodies. A total of 88 DM patients were enrolled, with a mean age of onset of 49.4 years old. The most common systemic features were myositis (56.8%, 50/88), internal malignancy (22.7%, 20/88), dysphagia (19.3%, 17/88), and ILD (17%, 15/88). Among the enrolled patients, 32 patients received serum myositis autoantibodies examination. The most common autoantibodies were ANA (50.7%, 37/73), followed by anti-TIF1-γ (34.4%, 11/32) and anti-MDA5 (31.3%, 10/32) antibodies. Patients with Gottron sign (OR 5.6), arthritis (OR 23.35), or the presence of anti-MDA5 antibody (OR 11.14) were more susceptible to progressing to ILD, whereas patients with pruritus (OR 1.04), dysphagia (OR 6.73), and the presence of ANA (OR 6.29) had significantly higher risks of developing internal malignancies. Physicians should pay special attention to certain clinical features, which can help with the early detection of systemic diseases. Cancer screening and myositis autoantibodies examination should be conducted in all DM patients if applicable.

Safety and Efficacy of PD-1/PD-L1 Inhibitors in Cancer Patients With Preexisting Autoantibodies.

BackgroundProgrammed cell death protein-1/programmed cell death ligand-1 (PD-1/PD-L1) inhibitors therapy is now a routine scheme in cancers. However, the effect of preexisting autoantibodies on the safety and efficacy of PD-1/PD-L1 inhibitors in cancer patients is not well understood.MethodsThe present retrospective cohort study evaluated the safety and efficacy of PD-1/PD-L1 inhibitors in patients with preexisting autoantibodies. Patients who received PD-1/PD-L1 inhibitors in the Department of Medical Oncology, Peking Union Medical College Hospital between November 2017 and August 2021 were reviewed.Results67 (37.9%) of the 177 patients, 27 (20.3%) of the 133 patients, and 16 (11.0%) of 146 patients who received PD-1/PD-L1 inhibitors were positive for ANA, anti-Ro52, and antithyroid antibodies, respectively. Preexisting ANA and anti-Ro52 antibody were not associated with the increased risk of immune-related adverse events (irAEs), while thyroid dysfunction was more frequent in patients with positive antithyroid antibody (75.0% versus 13.8%, p < 0.001). The median progression-free survival (PFS, 13.1 versus 7.0 months, p = 0.015) was significantly longer in the ANA-positive patients, while the median overall survival (OS, 14.5 versus 21.8 months, p = 0.67) did not differ significantly between the ANA-positive and ANA-negative groups. Moreover, the preexisting anti-Ro52 and antithyroid antibodies were not significantly associated with PFS and OS.ConclusionsThe presence of ANA and anti-Ro52 antibody were not associated with a higher risk of irAEs, whereas patients positive for antithyroid antibody should monitor closely immune-related thyroid dysfunction. Preexisting ANA might be a predictor of longer PFS, while anti-Ro52 and antithyroid antibodies had no significant effect on survival outcomes in patients receiving PD-1/PD-L1 inhibitors therapy.

Long Term Follow up of Patients With Primary Obstetric Antiphospholipid Syndrome.

Introduction: Primary obstetric antiphospholipid syndrome (OAPS) is defined by specific morbidities and/or losses of pregnancy in the presence of persistent antiphospholipid antibodies (aPL). This variant of APS is usually treated during pregnancy and the post-partum period. Data on occurrence of thrombotic event during long term follow-up of OAPS patients is limited. Methods: A multi-centre retrospectively cohort of female patients with primary APS (pAPS) was assembled during 2004–2019. Patients were grouped according to disease presentation as pure OAPS or thrombotic APS (tAPS) for those presenting with thrombosis. Clinical and serological data were compared between groups. Results: Of 219 pAPS female patients 67 (30.6%) were diagnosed with OAPS and 152 (69.4%) with tAPS. During >10 years of follow-up 24/67 (35.8%) OAPS and 71/152 (50%) tAPS suffered a new thrombotic event (p = 0.06), while obstetric morbidity was more likely in the OAPS group (31.3 vs. 10.5%, p < 0.001) respectively. Among patients with OAPS at presentation heart valve disease and the presence of ANA were related to thrombosis following diagnosis (25 vs. 4.7%, p = 0.02; and 45.8 vs. 20.8%, p = 0.04 respectively). Conclusion: Thrombotic event following diagnosis were common among female patients with pAPS regardless of disease presentation. Heart valve disease and ANA positivity may be risk factors for thrombosis during follow-up of patients presenting with pure OAPS.

Prevalence of autoantibodies in pediatric patients with idiopathic pulmonary hemosiderosis: a scoping review of the literature in the period 1980–2021

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology. Due to the frequent findings of autoimmune antibodies - autoantibodies, immunologic causation of the diffuse alveolar hemorrhage in IPH has been proposed, to assess the prevalence/frequency and type of autoantibodies in pediatric patients with IPH. In addition, the patient demographics, diagnostic modalities used to diagnose IPH, treatment, and outcomes were also evaluated. Scoping review: The PubMed, Medline, and Embase databases were searched with appropriate MeSH terms to identify relevant papers consistent with the defined inclusion criteria. Thirteen observational studies comprising a total of 352 pediatric patients were included in this review. The majority of subjects were girls 217 out of 352 (61.6%). The mean and median ages of patients ranged from 3.1-6.5years to 2.3-7years, respectively. In the 10 studies that specified the number of patients in their cohorts with either at least one positive autoantibody or no antibody, the overall prevalence of autoantibodies was 76 out of 288 patients (26.4%). The prevalence of specific antibodies was as follows: ANA, 20.3%; ANCA, 17%; anti-dsDNA, 9.1%; RF, 12%; anti-SMA, 23.2%; and celiac antibodies, 25.9%. Cow's milk protein allergy was present in 16.2% of the children. The significance of an association between IPH and the presence of autoantibodies has not been clarified. The autoantibodies could be suggestive of an overall immune dysregulation rather than causation. However, limited evidence based on a single study suggests that the presence of ANA may be associated with a higher risk of recurrence and worse outcomes. Further research, including prospective studies, will be crucial to explore a possible genetic linkage between vasculitides, systemic rheumatologic diseases, and IPH.

Frequency of ANA/DFS70 in relatives of patients with rheumatoid arthritis compared to patients with rheumatoid arthritis and a healthy population, and its association with health status

IntroductionDFS70 ANAs have attracted interest due to their frequency in individuals with no clinical evidence of systemic autoimmune rheumatic disease (SARD), groups with genetic risk for rheumatoid arthritis (RA) were not assessed. ObjectiveTo determine the frequency of ANA and DFS70 ANA in blood relatives (BR) of people with RA compared to patients with early RA (ERA), and control individuals, and its association with health status. MethodologyA cross-sectional study with an analytical component. Sixty ERA patients, 60 BR and 120 control individuals paired by age and sex were studied. Hep2-ANA and DFS70 ANA were studied. The absolute and relative frequencies and associations were established using logistic regression models, with a significance level of 95% Results43% ANA in ERA, 30% in BR, and 25%-8% in control individuals 1:80. The fine dense granular pattern based on conventional Hep2 was found in 12%-9% of the positive samples, and 1.66% of the total samples. There was no detection of DFS70 ANAs in patients with ERA. In ERA there was an association between the presence of ANA and inflamed joints (P = .02), CRP (P = .01), DAS28CRP (P = .03) and HAQ (P = .04). There was an association between ANA and elevated CRP (P = .05) in the BR. In the control individuals, there was an association between ANA and painful joints (P = .02). In DFS70 ANA individuals we observed an association between a normal ESR P = .032, BR (-), P = .044 and absence of painful joints, p = .039 ConclusionsThe frequency of DFS70 ANA in the groups studied was low, none of the patients with ERA was positive. The presence of DFS70 ANA was only confirmed in systemically healthy individuals.

Frequency of ANA/DFS70 in Relatives of Patients with Rheumatoid Arthritis Compared to Patients with Rheumatoid Arthritis and a Healthy Population, and its Association with Health Status.

DFS70 ANAs have attracted interest due to their frequency in individuals with no clinical evidence of systemic autoimmune rheumatic disease, groups with genetic risk for rheumatoid arthritis (RA) were not assessed. To determine the frequency of ANA and DFS70 ANA in blood relatives (BR) of people with RA compared to patients with early RA (ERA), and control individuals, and its association with health status. A cross-sectional study with an analytical component. Sixty ERA patients, 60 BR and 120 control individuals paired by age and sex were studied. Hep2-ANA and DFS70 ANA were studied. The absolute and relative frequencies and associations were established using logistic regression models, with a significance level of 95%. 43% ANA in ERA, 30% in BR, and 25.8% in control individuals 1:80. The fine dense granular pattern based on conventional Hep2 was found in 12.9% of the positive samples, and 1.66% of the total samples. There was no detection of DFS70 ANAs in patients with ERA. In ERA there was an association between the presence of ANA and inflamed joints (p=.02), CRP (p=.01), DAS28CRP (p=.03) and HAQ (p=.04). There was an association between ANA and elevated CRP (p=.05) in the BR. In the control individuals, there was an association between ANA and painful joints (p=02). In DFS70 ANA individuals we observed an association between a normal ESR p=.032, BR (-), p=.044 and absence of painful joints, p=.039. The frequency of DFS70 ANA in the groups studied was low, none of the patients with ERA was positive. The presence of DFS70 ANA was only confirmed in systemically healthy individuals.

Fitorreguladores e luminosidade na indução à calogênese em explantes foliares de &lt;i&gt;Eugenia involucrata&lt;/i&gt; DC.

Eugenia involucrata é uma espécie florestal nativa do Brasil com grande potencial para uso madeireiro, frutícola e medicinal. O presente estudo objetivou avaliar a influência de fitorreguladores e da luminosidade sobre a calogênese em discos foliares de Eugenia involucrata. Foi conduzido um experimento bifatorial (2x5), cujos tratamentos consistiram na combinação da ausência ou presença de luz durante o cultivo e de cinco diferentes empregos de fitorreguladores: ANA, 2,4-D, BAP e as combinações ANA + BAP e 2,4-D + BAP, todos a 5 μM, em meio nutritivo MS. Após 45 dias de cultivo in vitro foi observado que a ausência de luz durante o período de cultivo reduz a oxidação fenólica favorecendo, assim, a calogênese , especialmente na presença de ANA e das combinações ANA + BAP e 2,4-D + BAP. O uso de 2,4-D e de BAP, isoladamente, não é favorável à calogênese em discos foliares de Eugenia involucrata.

Relationships between increased circulating YKL-40, IL-6 and TNF-α levels and phenotypes and disease activity of primary Sjögren's syndrome

BackgroundThere is now no single score or marker useful for evaluating disease activity of primary Sjögren's syndrome (pSS). This study was designed to explore the associations of circulating YKL-40, interleukin 6 (IL-6) and tumor necrosis factor α (TNF-α) with systemic activity and phenotypes of pSS. MethodsThis study included 58 pSS patients and 30 healthy controls (HC). The sera were measured by multiplex immunoassay for YKL-40, IL-6 and TNF-α concentrations. The disease activity of pSS patients was evaluated by European league against rheumatism (EULAR) SS disease activity index (ESSDAI). Local severity was assessed in accordance with the Tarpley score. ResultsSerum YKL-40, IL-6 and TNF-α levels significantly elevated in pSS patients compared with those in HC (all P < 0.001). These cytokines correlated with ESSDAI, ESR, CRP, and IgG (all P < 0.05). Serum YKL-40 level correlated markedly with age (r = 0.405, P = 0.002), neutrophil count (r = 0.399, P = 0.002) and neutrophil-to-lymphocyte ratio (NLR) (r = 0.401, P = 0.002), while IL-6 did weakly with NLR (r = 0.296, P = 0.024) and C3 (r = 0.288, 0.036). Serum levels of all three cytokines were substantially lower in patients with eye/mouth dryness vs. those without (all P < 0.05). Additionally, patients with pulmonary, renal involvement or anemia had remarkably higher concentrations of YKL-40 (all P < 0.05), while those with leukocytopenia had lower levels (P = 0.01). Fever or anemia patients showed higher serum concentrations of IL-6 (both P < 0.05), while serum levels of TNF-α were much higher in patients with presence of ANA, anti-SSA or anti-SSB antibodies (All P < 0.05). Serum IL-6 level correlated strongly with YKL-40 (r = 0.452, P < 0.001) and TNF-α (r = 0.743, P < 0.001) in pSS patients. A significant correlation was also found between YKL-40 and TNF-α (r = 0.308, P = 0.022) . ConclusionThe circulating YKL-40, IL-6 and TNF-α levels increase in pSS, and all of them are significantly correlated with indicators (ESSDAI, ESR, CRP, and IgG) for systemic inflammation of pSS. Each cytokine is separately associated with specific pSS phenotype.

The role of food transfers in wild golden lion tamarins (Leontopithecus rosalia): Support for the informational and nutritional hypothesis

Callitrichidae is a unique primate family not only in terms of the large number of food transfers to infants but also for the prevalence of transfers that are initiated by the adults. It has been hypothesized that, as well as provisioning infants, callitrichid food transfers might function to teach the receiver what food types to eat. If food provisioning has a teaching function, we would expect successful food transfers to be more likely with food types that are novel to the juveniles. We would also expect juveniles to learn about foods from those transfers. We introduced different types of food (some familiar, some novel) to wild groups of golden lion tamarins (Leontopithecus rosalia). While novel foods were not more successfully transferred than familiar food in the experiment, transfers were more successful (i.e., the receiver obtained food) when the donor had previous experience with that food. Moreover, we found evidence suggesting that food transfers influenced the future foraging choices of juveniles. Our findings are consistent with the first and third criteria of the functional definition of teaching, which requires that tutors (the adults) modify their behavior in the presence of a naïve individual (a juvenile), and that the naïve individual learns from the modified behavior of the demonstrator. Our findings are also consistent with the provisioning function of food transfer. Social learning seems to play an important role in the development of young tamarins’ foraging preferences.

Sjögren's syndrome with juvenile onset

The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.

Comparative Genomics of Thiohalobacter thiocyanaticus HRh1T and Guyparkeria sp. SCN-R1, Halophilic Chemolithoautotrophic Sulfur-Oxidizing Gammaproteobacteria Capable of Using Thiocyanate as Energy Source.

The genomes of Thiohalobacter thiocyanaticus and Guyparkeria (formerly known as Halothiobacillus) sp. SCN-R1, two gammaproteobacterial halophilic sulfur-oxidizing bacteria (SOB) capable of thiocyanate oxidation via the “cyanate pathway”, have been analyzed with a particular focus on their thiocyanate-oxidizing potential and sulfur oxidation pathways. Both genomes encode homologs of the enzyme thiocyanate dehydrogenase (TcDH) that oxidizes thiocyanate via the “cyanate pathway” in members of the haloalkaliphilic SOB of the genus Thioalkalivibrio. However, despite the presence of conservative motives indicative of TcDH, the putative TcDH of the halophilic SOB have a low overall amino acid similarity to the Thioalkalivibrio enzyme, and also the surrounding genes in the TcDH locus were different. In particular, an alternative copper transport system Cus is present instead of Cop and a putative zero-valent sulfur acceptor protein gene appears just before TcDH. Moreover, in contrast to the thiocyanate-oxidizing Thioalkalivibrio species, both genomes of the halophilic SOB contained a gene encoding the enzyme cyanate hydratase. The sulfur-oxidizing pathway in the genome of Thiohalobacter includes a Fcc type of sulfide dehydrogenase, a rDsr complex/AprAB/Sat for oxidation of zero-valent sulfur to sulfate, and an incomplete Sox pathway, lacking SoxCD. The sulfur oxidation pathway reconstructed from the genome of Guyparkeria sp. SCN-R1 was more similar to that of members of the Thiomicrospira-Hydrogenovibrio group, including a Fcc type of sulfide dehydrogenase and a complete Sox complex. One of the outstanding properties of Thiohalobacter is the presence of a Na+-dependent ATP synthase, which is rarely found in aerobic Prokaryotes.Overall, the results showed that, despite an obvious difference in the general sulfur-oxidation pathways, halophilic and haloalkaliphilic SOB belonging to different genera within the Gammaproteobacteria developed a similar unique thiocyanate-degrading mechanism based on the direct oxidative attack on the sulfane atom of thiocyanate.

Aluminates with Fluorinated Schiff Bases: Influence of the Alkali Metal⁻Fluorine Interactions in Structure Stabilization.

New heterometallic aluminium-alkali metal compounds have been prepared using Schiff bases with electron withdrawing substituents as ligands. The synthesis of these new species was achieved via the reaction of AlMe3 with the freshly prepared alkali-metallated ligand. The derivatives formed were characterized by NMR in solution and by single crystal X-ray diffraction in the solid state. Aluminate derivatives with lithium and sodium were prepared and a clear influence of the alkali metal in the final outcome is observed. The presence of a Na···F interaction in the solid state has a stabilization effect and the species [NaAlMe3L]2 can de isolated for the first time, which was not possible when using Schiff bases without electron withdrawing substituents as ligands.

Predictors of Flare Following Etanercept Withdrawal in Patients with Rheumatoid Factor-negative Juvenile Idiopathic Arthritis Who Reached Remission while Taking Medication.

To evaluate the rate of flare after etanercept (ETN) withdrawal in patients with juvenile idiopathic arthritis (JIA) who attained clinical remission while taking medication, and to identify predictors of flare. Patients were included with oligo- (oJIA) and rheumatoid factor-negative polyarticular JIA (pJIA) who received a first course of ETN for at least 18 months, maintained clinically inactive disease (CID) for at least 6 months during treatment, and were followed for 12 months after ETN withdrawal. Demographic and clinical features were collected at onset, at baseline (initiation of ETN), and at time of disease flare. After ETN withdrawal, 66 of the 110 patients enrolled (60%) flared with arthritis (of whom 7 flared with concurrent anterior uveitis; none with uveitis alone). The median time to flare was 4.3 months (interquartile range 2.5-6.4) with no evident differences between oJIA and pJIA. The number and type of joints involved at baseline and characteristics of ETN treatment/discontinuation were not associated with flare. Patients who flared were more frequently males (p = 0.034), positive for antinuclear antibody (ANA; p = 0.047), and had higher values of C-reactive protein (CRP; p = 0.012) at baseline. These variables remained significantly associated with flare in a multivariate logistic analysis, a model accounting for only 14% of the variability of the occurrence of the flare. Our results show that a significant proportion of patients with JIA who maintain CID for at least 6 months experience a relapse after ETN withdrawal. Male sex, presence of ANA, and elevated CRP at baseline were associated with higher risk of flare.

Chronic Hepatitis C Virus (HCV)-associated Cryoglobulinemia and its possible impact on the skin in Egyptian Patients

Background: Chronic hepatitis C virus (HCV) infection may have extremely variable clinical consequences and is more than just a liver disease; it has been associated with numerous extra-hepatic manifestations (EHM). According to various international studies Mixed Cryoglobulinemia (MC) was found to be the most common EHM, however its local prevalence in Egyptian HCV patients was not clearly studied. The aim of our study was to investigate the frequency of cryoglobulinaemia in sera of chronic HCV patients and their association with clinical symptoms especially, vasculitis. Method: One hundred patients with chronic HCV infection attending the outpatient clinic of the National Hepatology and Tropical Medicine Research Institute were interviewed. Patients with decompensated liver disease, on interferon therapy, having end-stage renal disease or coexisting viral infection like hepatitis B surface antigen positive patients were all excluded from the research. All patients were subjected to general and dermatological examination for liver affection signs; cryoglobulinemia related clinical manifestations and/or associated dermatoses. Cryoglobulins, CBC, LFT. AFP, ALP, KFT, ANA and RF were assessed. Results: Overall 15% of 100 patients were positive for presence of cryoglobulins in their sera. We found a relatively high incidence of clinical symptoms commonly accompanying cryoglobulinemic cases in the form of Purpura, Arthralgia, Generalized weakness, Peripheral Neuropathy and Reynaud’s phenomenon with prevalence of 26.67%, 46.67%, 53.33%, 40% and 6.67% respectively. Our data also demonstrated that 46.67% (7) of our 15 cryopositive patients had concomitant skin manifestations in the form of Pruritus 40% (6) and Vasculitis 26.67% (4) (P=0.004) which was significant in comparison with prevalence of vasculitis in all patients 4.7% (4 patients). Generalized weakness and fatigue, which is the most prevalent symptoms related to Chronic Hepatitis C (CHC) patients whether positive or negative for cryoglobulinemia, were present in 52% of all patients. Other associated dermatological diseases in all patients were Lichen Planus, Psoraiasis, Urticaria, Necrolytic Acral Erythema (NAE) and Vitiligo with prevalence of (1%), (1%), (5%), (1%) and (2%) respectively. The most common dermatological disease was pruritus 36% (36) of all patients and 40% (6) in cryopositive patients. Regarding liver condition there was no significant correlation between presence of cryoglobulins and biochemical parameters. However, we found a significant correlation between presence of cryoglobulinemia and presence of ANA in sera of HCV patients with incidence of 40%) in

Chronic Hepatitis C Virus (HCV)-associated Cryoglobulinemia and its possible impact on the skin in Egyptian Patients

Background: Chronic hepatitis C virus (HCV) infection may have extremely variable clinical consequences and is more than just a liver disease; it has been associated with numerous extra-hepatic manifestations (EHM). According to various international studies Mixed Cryoglobulinemia (MC) was found to be the most common EHM, however its local prevalence in Egyptian HCV patients was not clearly studied. The aim of our study was to investigate the frequency of cryoglobulinaemia in sera of chronic HCV patients and their association with clinical symptoms especially, vasculitis. Method: One hundred patients with chronic HCV infection attending the outpatient clinic of the National Hepatology and Tropical Medicine Research Institute were interviewed. Patients with decompensated liver disease, on interferon therapy, having end-stage renal disease or coexisting viral infection like hepatitis B surface antigen positive patients were all excluded from the research. All patients were subjected to general and dermatological examination for liver affection signs; cryoglobulinemia related clinical manifestations and/or associated dermatoses. Cryoglobulins, CBC, LFT. AFP, ALP, KFT, ANA and RF were assessed. Results: Overall 15% of 100 patients were positive for presence of cryoglobulins in their sera. We found a relatively high incidence of clinical symptoms commonly accompanying cryoglobulinemic cases in the form of Purpura, Arthralgia, Generalized weakness, Peripheral Neuropathy and Reynaud’s phenomenon with prevalence of 26.67%, 46.67%, 53.33%, 40% and 6.67% respectively. Our data also demonstrated that 46.67% (7) of our 15 cryopositive patients had concomitant skin manifestations in the form of Pruritus 40% (6) and Vasculitis 26.67% (4) (P=0.004) which was significant in comparison with prevalence of vasculitis in all patients 4.7% (4 patients). Generalized weakness and fatigue, which is the most prevalent symptoms related to Chronic Hepatitis C (CHC) patients whether positive or negative for cryoglobulinemia, were present in 52% of all patients. Other associated dermatological diseases in all patients were Lichen Planus, Psoraiasis, Urticaria, Necrolytic Acral Erythema (NAE) and Vitiligo with prevalence of (1%), (1%), (5%), (1%) and (2%) respectively. The most common dermatological disease was pruritus 36% (36) of all patients and 40% (6) in cryopositive patients. Regarding liver condition there was no significant correlation between presence of cryoglobulins and biochemical parameters. However, we found a significant correlation between presence of cryoglobulinemia and presence of ANA in sera of HCV patients with incidence of 40%) in