785 research outputs found

    Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature

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    Background Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory syndrome and is caused by a severely dysregulated immune response. It has rarely been associated with neonatal lupus. Case presentation We present a female neonate with MAS born to a mother who had cutaneous lupus erythematosus with circulating anti-nuclear antibodies (ANA), anti-SSA, anti-SSB and anti-extractable nuclear antigen (anti-ENA) antibodies. Because of neonatal lupus (NLE) with a total atrioventricular block, epicardial pacemaker implantation was required on the sixth day of life. Following surgery she developed non-remitting fever and disseminated erythematous skin lesions. A diagnosis of MAS was made based on these symptoms, with hyperferritinemia, elevated transaminases, hypertriglyceridemia, and a skin biopsy that showed hemophagocytosis. Our patient was treated with steroids for 3 months with good effect. No relapse has occurred. Conclusions MAS is a rare complication of neonatal lupus that may be difficult to diagnose, but needs to be treated promptly. In this article, pathogenesis and overlap of MAS and hemophagocytic lymphohistiocytosis (HLH) has been described. Diagnosis of MAS can be difficult. Different diagnostic criteria are used in both diagnosing MAS and HLH. Validated criteria for diagnosis of MAS in other disease than systemic onset JIA have not been validated yet. In NLE, diagnosing MAS is even more difficult, since skin lesions are already common in NLE. We show the potential additional value of skin biopsy in diagnosing MAS.Transplantation and immunomodulationDevelopmen

    On the Formation Height of the SDO/HMI Fe 6173 Doppler Signal

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    The Helioseismic and Magnetic Imager (HMI) onboard the Solar Dynamics Observatory (SDO) is designed to study oscillations and the mag- netic field in the solar photosphere. It observes the full solar disk in the Fe I absorption line at 6173\AA . We use the output of a high-resolution 3D, time- dependent, radiation-hydrodynamic simulation based on the CO5BOLD code to calculate profiles F({\lambda},x,y,t) for the Fe I 6173{\AA} line. The emerging profiles F({\lambda},x,y,t) are multiplied by a representative set of HMI filter transmission profiles R_i({\lambda},1 \leq i \leq 6) and filtergrams I_i(x,y,t;1 \leq i \leq 6) are constructed for six wavelengths. Doppler velocities V_HMI(x,y,t) are determined from these filtergrams using a simplified version of the HMI pipeline. The Doppler velocities are correlated with the original velocities in the simulated atmosphere. The cross- correlation peaks near 100 km, suggesting that the HMI Doppler velocity signal is formed rather low in the solar atmosphere. The same analysis is performed for the SOHO/MDI Ni I line at 6768\AA . The MDI Doppler signal is formed slightly higher at around 125 km. Taking into account the limited spatial resolution of the instruments, the apparent formation height of both the HMI and MDI Doppler signal increases by 40 to 50 km. We also study how uncertainties in the HMI filter-transmission profiles affect the calculated velocities.Comment: 15 pages, 11 Figure

    Beautiful Mirrors and Precision Electroweak Data

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    The Standard Model (SM) with a light Higgs boson provides a very good description of the precision electroweak observable data coming from the LEP, SLD and Tevatron experiments. Most of the observables, with the notable exception of the forward-backward asymmetry of the bottom quark, point towards a Higgs mass far below its current experimental bound. The disagreement, within the SM, between the values for the weak mixing angle as obtained from the measurement of the leptonic and hadronic asymmetries at lepton colliders, may be taken to indicate new physics contributions to the precision electroweak observables. In this article we investigate the possibility that the inclusion of additional bottom-like quarks could help resolve this discrepancy. Two inequivalent assignments for these new quarks are analysed. The resultant fits to the electroweak data show a significant improvement when compared to that obtained in the SM. While in one of the examples analyzed, the exotic quarks are predicted to be light, with masses below 300 GeV, and the Higgs tends to be heavy, in the second one the Higgs is predicted to be light, with a mass below 250 GeV, while the quarks tend to be heavy, with masses of about 800 GeV. The collider signatures associated with the new exotic quarks, as well as the question of unification of couplings within these models and a possible cosmological implication of the new physical degrees of freedom at the weak scale are also discussed.Comment: 21 pages, 4 embedded postscript figures, LaTeX. Two minor corrections performe

    Severe anal bleeding in Proteus syndrome: a case report

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    Proteus syndrome was originally described by Cohen and Hayden in 1979. The disorder was named Proteus syndrome by Wiedmann and colleagues in 1983 after Proteus, the giant Greek god of the sea. Proteus syndrome is a rare, sporadic, congenital polymorphic condition. Approximately 200 cases have been reported in the literature, but none has been associated with anal bleeding from hemorrhoids. We describe the case of a 21-year-old man with Proteus syndrome with severe anal bleeding. A hemorrhoidectomy was assumed to be too risky because of the massive venous abnormalities seen on CT. The patient was successfully treated by Doppler-guided haemorrhoidal artery ligation (DG-HAL). Six months after surgery, the patient has had no further episodes of anal bleeding

    Testing the Nature of Kaluza-Klein Excitations at Future Lepton Colliders

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    With one extra dimension, current high precision electroweak data constrain the masses of the first Kaluza-Klein excitations of the Standard Model gauge fields to lie above 4\simeq 4 TeV. States with masses not much larger than this should be observable at the LHC. However, even for first excitation masses close to this lower bound, the second set of excitations will be too heavy to be produced thus eliminating the possibility of realizing the cleanest signature for KK scenarios. Previous studies of heavy ZZ' and WW' production in this mass range at the LHC have demonstrated that very little information can be obtained about their couplings to the conventional fermions given the limited available statistics and imply that the LHC cannot distinguish an ordinary ZZ' from the degenerate pair of the first KK excitations of the γ\gamma and ZZ. In this paper we discuss the capability of lepton colliders with center of mass energies significantly below the excitation mass to resolve this ambiguity. In addition, we examine how direct measurements obtained on and near the top of the first excitation peak at lepton colliders can confirm these results. For more than one extra dimension we demonstrate that it is likely that the first KK excitation is too massive to be produced at the LHC.Comment: 38 pages, 10 Figs, LaTex, comments adde

    Vulnerable plaques and patients: state-of-the-art

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    Despite advanced understanding of the biology of atherosclerosis, coronary heart disease remains the leading cause of death worldwide. Progress has been challenging as half of the individuals who suffer sudden cardiac death do not experience premonitory symptoms. Furthermore, it is well-recognized that also a plaque that does not cause a haemodynamically significant stenosis can trigger a sudden cardiac event, yet the majority of ruptured or eroded plaques remain clinically silent. In the past 30 years since the term 'vulnerable plaque' was introduced, there have been major advances in the understanding of plaque pathogenesis and pathophysiology, shifting from pursuing features of 'vulnerability' of a specific lesion to the more comprehensive goal of identifying patient 'cardiovascular vulnerability'. It has been also recognized that aside a thin-capped, lipid-rich plaque associated with plaque rupture, acute coronary syndromes (ACS) are also caused by plaque erosion underlying between 25% and 60% of ACS nowadays, by calcified nodule or by functional coronary alterations. While there have been advances in preventive strategies and in pharmacotherapy, with improved agents to reduce cholesterol, thrombosis, and inflammation, events continue to occur in patients receiving optimal medical treatment. Although at present the positive predictive value of imaging precursors of the culprit plaques remains too low for clinical relevance, improving coronary plaque imaging may be instrumental in guiding pharmacotherapy intensity and could facilitate optimal allocation of novel, more aggressive, and costly treatment strategies. Recent technical and diagnostic advances justify continuation of interdisciplinary research efforts to improve cardiovascular prognosis by both systemic and 'local' diagnostics and therapies. The present state-of-the-art document aims to present and critically appraise the latest evidence, developments, and future perspectives in detection, prevention, and treatment of 'high-risk' plaques occurring in 'vulnerable' patients

    Lifetimes of High-Degree p Modes in the Quiet and Active Sun

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    We study variations of the lifetimes of high-degree solar p-modes in the quiet and active Sun with the solar activity cycle. The lifetimes in the degree range 300 - 600 and frequency 2.5 - 4.5 mHz were computed from SOHO/MDI data in an area including active regions and quiet Sun using the time-distance technique. We applied our analysis to the data in four different phases of solar activity: in 1996 (at minimum), 1998 (rising phase), 2000 (at maximum) and 2003 (declining phase). The results from the area with active regions show that the lifetime decreases as activity increases. The maximal lifetime variations are between solar minimum in 1996 and maximum in 2000; the relative variation averaged over all mode degree values and frequencies is a decrease of about 13%. The lifetime reductions relative to 1996 are about 7% in 1998 and about 10% in 2003. The lifetime computed in the quiet region still decreases with solar activity although the decrease is smaller. On average, relative to 1996, the lifetime decrease is about 4% in 1998, 10% in 2000 and 8% in 2003. Thus, measured lifetime increases when regions of high magnetic activity are avoided. Moreover, the lifetime computed in quiet regions also shows variations with activity cycle.Comment: 13 pages, 5 figures; Accepted for publication in Solar Physic

    Evidence for a mixed mass composition at the `ankle' in the cosmic-ray spectrum

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    We report a first measurement for ultra-high energy cosmic rays of the correlation between the depth of shower maximum and the signal in the water Cherenkov stations of air-showers registered simultaneously by the fluorescence and the surface detectors of the Pierre Auger Observatory. Such a correlation measurement is a unique feature of a hybrid air-shower observatory with sensitivity to both the electromagnetic and muonic components. It allows an accurate determination of the spread of primary masses in the cosmic-ray flux. Up till now, constraints on the spread of primary masses have been dominated by systematic uncertainties. The present correlation measurement is not affected by systematics in the measurement of the depth of shower maximum or the signal in the water Cherenkov stations. The analysis relies on general characteristics of air showers and is thus robust also with respect to uncertainties in hadronic event generators. The observed correlation in the energy range around the `ankle' at lg(E/eV)=18.519.0\lg(E/{\rm eV})=18.5-19.0 differs significantly from expectations for pure primary cosmic-ray compositions. A light composition made up of proton and helium only is equally inconsistent with observations. The data are explained well by a mixed composition including nuclei with mass A>4A > 4. Scenarios such as the proton dip model, with almost pure compositions, are thus disfavoured as the sole explanation of the ultrahigh-energy cosmic-ray flux at Earth.Comment: Published version. Added journal reference and DOI. Added Report Numbe
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