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Background:Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disorder of unknown etiology characterized by systemic inflammation, high fever, salmon-colored skin rash, arthralgia, and arthritis. Patients with AOSD may also present with elevated inflammatory markers, hyperferritinemia, anemia, leukocytosis, hepatosplenomegaly, and lymphadenopathy. Glucocorticoids, disease-modifying anti-rheumatic drugs (DMARDs), and biological drugs, including the anti-IL-1 agent anakinra, are used in the management of AOSD.Objectives:The main purpose of this study was to evaluate the effectiveness of anakinra in suppressing disease activity, tapering glucocorticoids, and preventing undesired metabolic effects of glucocorticoids in patients with AOSD. This study also aimed to assess the tolerability of the anakinra treatment.Methods:In this retrospective single-center study, we included patients who were registered at our tertiary center with a diagnosis of AOSD according to the Yamaguchi criteria and had received treatment with anakinra. Patients were allowed to receive DMARDs or glucocorticoids prior to or concomitant with anakinra treatment. The primary outcome of our study was the proportion of patients who achieved complete remission of disease-related complications, including MAS, fever, arthralgia, arthritis, rash, hepatomegaly, splenomegaly, pericarditis, pleuritis, peritonitis, lymphadenopathy, anemia, hyperferritinemia, leukocytosis, elevated inflammatory markers (CRP and ESR), and elevated liver enzymes (AST and ALT) after anakinra treatment. Partial remission was defined as ≥ 50% improvement in the aforementioned findings. Any other outcome was considered a treatment failure. We analyzed the use and daily dose of glucocorticoids before and after anakinra treatment. As an aspect of glucocorticoid toxicity, we evaluated changes in body mass index (BMI) after anakinra treatment. Each patient was called for a physical examination, online methods were used when a physical examination was not performed. Other data sources included patient files and digital institutional records.Results:Our study included 29 patients with AOSD, 21 (72.4 %) of whom were female. The mean age and mean disease duration were 39.17 ± 13.63 and 6.59 ± 3.32 years, respectively. The mean duration of anakinra treatment was 22.35 ± 26.49 months. Eleven patients (37.9 %) achieved complete remission after anakinra treatment. Partial remission was achieved in 10 (34.5 %) patients, and treatment failed in 8 (27.6 %) patients. Anakinra significantly reduced the proportion of patients who experienced fever, arthritis, arthralgia, rash, lymphadenopathy, and elevated inflammatory markers. Anakinra was also associated with a significant improvement in anemia and leukocytosis (Figure 1). Twenty-seven patients (93.1 %) received glucocorticoids prior to the first application of anakinra. This number reduced to 20 (69 %) after treatment, indicating a significant reduction (p=0.04). Among patients who received anakinra the mean daily glucocorticoid dose decreased from 17.62 ± 13.2 mg to 4.79 ± 5.1 mg (p<0.001). However, the mean BMI increased from 25.23 ± 5.25 to 27.15 ± 6.25 (p=0.016). Anakinra demonstrated significant improvements in patient-reported disease activity scores and acute phase responses (Figure 2). Twenty patients (69%) were not receiving anakinra at the time the study was conducted. The most common reasons for treatment discontinuation were injection site reactions and a lack of efficacy. Mild adverse events occurred in 10 patients (34.5 %), with injection site reactions being the most frequent. No serious adverse events were observed during anakinra treatment.Conclusion:Anakinra was effective in resolving disease-related complications, suppressing inflammatory markers, and tapering glucocorticoids, with a low adverse event profile in patients with AOSD.REFERENCES:NIL.Figure 1.Frequencies of the disease-related clinical and laboratory findings before and after anakinra treatment.Figure 2.Clinical and laboratory determinants of improvement before and after anakinra treatment.Acknowledgements:NIL.Disclosure of Interests:None declared.

Particle Flow Algorithms (PFAs) attempt to measure each particle in a hadronic jet individually, using the detector subsystem that provides the best energy/momentum resolution. Calorimeters that can exploit the power of PFAs emphasize spatial granularity over single particle energy resolution. In this context, the CALICE Collaboration developed the Digital Hadron Calorimeter (DHCAL). The DHCAL uses Resistive Plate Chambers (RPCs) as active media and is read out with 1 × 1 cm 2 pads and digital (1-bit) resolution. In order to obtain a unique dataset of electromagnetic and hadronic interactions with unprecedented spatial resolution, the DHCAL went through a broad test beam program. In addition to conventional calorimetry, the DHCAL offers detailed measurements of event shapes, rigorous tests of simulation models and various analytical tools to improve calorimetric performance. Here we report on the results from the analysis of DHCAL data and comparisons with the Monte Carlo simulations.

Recently, longitudinal stent deformation (LSD) has been reported increasingly. Even though the reported cases included almost all stent designs, most cases were seen in the Element™ stent design (Boston Scientific, Natick, MA, USA). It is considered that stent design, lesion and procedural characteristics play a role in the etiology of LSD. Yet, the effect of LSD on long-term clinical outcomes has not been studied well. Element stents implanted between January 2013 and April 2015 in our hospital were examined retrospectively. Patients were grouped into two according to the presence of LSD, and their clinical, lesion and procedural characteristics were studied. Twenty-four LSD’s were detected in 1812 Element stents deployed in 1314 patients (1.83 % of PCI cases and 1.32 % of all Element stents). LMCA lesions (16.7 % vs 1.6 %, p < 0.001), complex lesions (75 % vs 35.1 %, p < 0.001), bifurcation lesions (37.5 % vs 18.3 %, p = 0.017), ostial lesions (33.3 % vs 12.8 %, p = 0.003), using of extra-support guiding catheter (54.2 % vs 22.3 %, p < 0.001) and extra-support guidewire (37.5 % vs 16.2 %, p = 0.005) were found to be more frequent in cases with LSD than in cases without it. In addition, the number of stents, stent inflation pressure and the use of post-dilatation were significantly different between the two groups. Two patients had an adverse event during the follow-up period. LSD is a rarely encountered complication, and is more common in complex lesions such as ostial, bifurcation and LMCA lesions. The use of extra-support guiding catheter, extra-support guidewires and low stent inflation pressure increases the occurrence of LSD. Nevertheless, with increased awareness of LSD and proper treatment, unwanted long-term outcomes can be successfully prevented.

Objectives: Previous studies showed the association between the major adverse cardiovascular outcomes and both higher neutrophil and lower lymphocyte counts. We aimed to investigate whether there is an association between the neutrophil-lymphocyte ratio (NLR) value and the development of coronary collateral circulation (CCC) in patients with coronary chronic total occlusion (CTO). Methods: A total of 274 patients with CTO were included in this study. Patients were then classified according to their Rentrop collateral grades as either poor (Rentrop grades 0-1) or good (Rentrop grades 2-3). Clinical information and analyses of blood samples were obtained from a review of the patients’ charts. Results: Although there was no difference between the two groups with regard to cardiovascular risk profiles, the NLR values were significantly higher in the patients who had poorly developed CCC (2.6 ± 0.5 vs 2.2 ± 0.4, p<0.001). NLR, high-sensitivity C-reactive protein (hs-CRP), white blood cell count (WBC), age, diabetes, fasting glucose levels and body mass index were found to have univariate association with poorly developed CCC (p<0.1). In a multivariate logistic regression model, NLR (odds ratio 1.88, 95% confidence interval (CI) 1.37–2.74; p<0.001), high-sensitivity C-reactive protein and WBC were found to be the independent predictors of poor CCC. In receiver operator characteristic curve analysis, the optimal cut-off value of NLR to predict poor CCC was found as 2.17, with 77% sensitivity and 65% specificity. Conclusion: NLR, as a novel cardiovascular risk marker, is an important, simple and inexpensive method which can be used by the cardiologist as a screening inflammation tool to estimate the development of CCC in patients with CTO.

Cor triatriatum dexter (CTD) is an extremely rare cardiac anomaly in which the right atrium is divided into two distinct chambers by a membrane. The persistence of the right valve of sinus venosus results in a complete septation of the right atrium. This anomaly is frequently associated with other right-sided cardiac abnormalities. Its clinical manifestation and the need for intervention are determined by the number and the size of the fenestrations on the membrane, associated cardiac anomalies and arrhythmias. We describe a case of CTD in a patient with complete atrioventricular (A-V) block.

Background Heart failure with preserved ejection fraction (HFpEF) is a syndrome in which patients have symptoms and signs of heart failure but preserved ejection fraction. Left atrial (LA) volume and function are known to be impaired in these patients. Two-dimensional speckle-tracking echocardiography (2D-STE) has recently enabled the quantification of LA deformation dynamics. In this study, we evaluated the use of 2D-STE for the diagnosis of HFpEF. Patients and methods The study included 83 patients with suspected HFpEF. Patients were divided into two groups after HFpEF had been diagnosed according to current guidelines. Parameters of diastolic dysfunction were evaluated, including left ventricular mass index (LVMI), LA volume index (LAVI), E/A ratio, deceleration time (DT), E/E’, and STE parameters such as global longitudinal LA strain during ventricular systole (GLAs-res) and strain during late diastole (GLAs-pump). Results The values of BNP, LVMI, DT, LAVI, and GLAs-res were significantly different between the two groups. In univariate analysis, a strong negative correlation was seen between GLAs-res and BNP ( r  = −0.567, p < 0.001) as well as between GLAs-res and DT ( r = −0.665, p  < 0.001), while a moderate negative correlation was found between GLAs-res and LVMI ( r = −0.458, p < 0.001) and GLAs-res and LAVI ( r = −0.316, p = 0.004). In logistic regression analysis, GLAs-res ( p = 0.049, OR = 0.71, 95 % CI = 0.451–0.99), BNP ( p = 0.025, OR = 1.08, 95 % CI = 1.01–1.14), and LAVI ( p = 0.042, OR = 1.59, 95 % CI = 1.02–2.48) were found to be independent predictors of HFpEF. Conclusion LA function as assessed by 2D-STE is impaired in patients with HFpEF. A GLAs-res value of < 17.5 % can be useful for the diagnosis of HFpEF.

Background There are few reports in the literature on the development of a fistulous connection between the left internal mammary artery (LIMA) and the pulmonary vasculature (PV) after coronary artery bypass grafting (CABG). This type of fistula may cause angina after CABG. Various mechanisms in the pathophysiology of this rare condition have been proposed. Methods We evaluated 537 consecutive patients with CABG surgery who underwent coronary angiography at our institution between January 2011 and March 2012. The post-CABG angiograms were evaluated for LIMA–PV fistula formation. Presence of a LIMA–PV fistula was defined as opacification of the PV or parenchyma after injection of radiopaque contrast medium into the LIMA. Results We found that 5 of 537 patients (0.93 %) had a LIMA–PV fistula on post-CABG coronary angiograms. The mean age of patients with a LIMA–PV fistula was 61.4 years (range, 51–72 years) and all patients were male. Coronary angiography was performed in the setting of myocardial infarction for 2 patients with a LIMA–PV fistula, and stable angina pectoris was the indication for coronary angiography in the remaining 3 patients. The mean diagnosis time of LIMA–PV fistula after CABG was 3.4 years (range, 1–9 years). None of the patients had a history of redo-CABG, perioperative mediastinitis, or pneumonia. Conclusion LIMA–PV fistulas may occur more frequently than reported on post-CABG angiogram findings. Angina in post-CABG patients may be associated with a LIMA–PV fistula, and selective cannulation of the LIMA with careful evaluation of the angiographic images may provide proper diagnosis and treatment of this entity.

The measurement of the luminosity recorded by the CMS detector installed at LHC interaction point 5, using proton–proton collisions at s=13TeV in 2015 and 2016, is reported. The absolute luminosity scale is measured for individual bunch crossings using beam-separation scans (the van der Meer method), with a relative precision of 1.3 and 1.0% in 2015 and 2016, respectively. The dominant sources of uncertainty are related to residual differences between the measured beam positions and the ones provided by the operational settings of the LHC magnets, the factorizability of the proton bunch spatial density functions in the coordinates transverse to the beam direction, and the modeling of the effect of electromagnetic interactions among protons in the colliding bunches. When applying the van der Meer calibration to the entire run periods, the integrated luminosities when CMS was fully operational are 2.27 and 36.3 fb-1 in 2015 and 2016, with a relative precision of 1.6 and 1.2%, respectively. These are among the most precise luminosity measurements at bunched-beam hadron colliders.

A bstract A search is presented for physics beyond the standard model (SM) using electron or muon pairs with high invariant mass. A data set of proton-proton collisions collected by the CMS experiment at the LHC at s = 13 TeV from 2016 to 2018 corresponding to a total integrated luminosity of up to 140 fb − 1 is analyzed. No significant deviation is observed with respect to the SM background expectations. Upper limits are presented on the ratio of the product of the production cross section and the branching fraction to dileptons of a new narrow resonance to that of the Z boson. These provide the most stringent lower limits to date on the masses for various spin-1 particles, spin-2 gravitons in the Randall-Sundrum model, as well as spin-1 mediators between the SM and dark matter particles. Lower limits on the ultraviolet cutoff parameter are set both for four-fermion contact interactions and for the Arkani-Hamed, Dimopoulos, and Dvali model with large extra dimensions. Lepton flavor universality is tested at the TeV scale for the first time by comparing the dimuon and dielectron mass spectra. No significant deviation from the SM expectation of unity is observed.