Angioedema is defined as localized and self‐limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive ...mediator(s). When angioedema recurs without significant wheals, the patient should be diagnosed to have angioedema as a distinct disease. In the absence of accepted classification, different types of angioedema are not uniquely identified. For this reason, the European Academy of Allergy and Clinical Immunology gave its patronage to a consensus conference aimed at classifying angioedema. Four types of acquired and three types of hereditary angioedema were identified as separate forms from the analysis of the literature and were presented in detail at the meeting. Here, we summarize the analysis of the data and the resulting classification of angioedema.
Context. X-shaped radio galaxies (XRGs) exhibit a pair of bright primary lobes and a pair of weak secondary lobes (“wings”), which are oriented with an angle that gives the structure a cross-like ...shape. Though several theoretical models have been proposed to explain their origin, there is currently not a general consensus on a formation scenario. Aims. We analysed new multifrequency Karl G. Jansky Very Large Array (JVLA) radio data at 1.5, 5.5, 6, and 9 GHz of the candidate XRG in Abell 3670 (A3670) in order to characterise and classify it for the first time and to investigate its origin. Methods. We produced flux, spectral index, and radiative age maps of A3670 by means of the new radio data. We investigated the connection between the radio galaxy and its host, a brightest cluster galaxy (BCG) with two optical nuclei classified as a dumbbell galaxy. Finally we discussed the literature models and compared them to the observed properties of A3670. Results. We classify A3670 as a Fanaroff–Riley I-type XRG and measured a 1.4 GHz radio power of P1.4 = 1.7 × 1025 W Hz−1. By estimating the radiative age of the various source components, we find that the wings are Δt ≃ 20 Myr older than the lobes. We verified that the lobes and wings are aligned with the major and minor axes of the optical galaxy, respectively, and we estimated a black hole mass of MBH ∼ 109 M⊙, which is in agreement with the typical properties of the XRGs. Conclusions. Among the discussed scenarios, the jet-shell interaction model may best reproduce the observed properties of A3670. The gas of a stellar shell is responsible for the deflection of the jets, thus forming the wings. The presence of stellar shells in A3670 is plausible, but it needs further optical observations to be confirmed.
Summary
Background
The inherited deficiency of C1‐inhibitor (C1‐INH), which can be quantitative (type I) or qualitative (type II), is characterized by recurrent attacks of oedema, and it is known as ...hereditary angioedema due to C1‐INH deficiency (HAE‐C1‐INH). The frequency of symptoms varies widely among patients and in the same patient during life.
Objective
To identify laboratory markers of disease severity in HAE‐C1‐INH patients.
Methods
We studied 162 patients with differently severe HAE‐C1‐INH during remission, 31 HAE‐C1‐INH patients during attacks, and 81 normal controls, evaluating complement parameters, spontaneous plasma kallikrein activity, the capacity of plasma to inhibit exogenous kallikrein activity, and cleavage of high‐molecular‐weight kininogen (HK). Sixty‐five HAE‐C1‐INH patients were screened for mutations in the C1‐INH gene.
Results
As expected, plasma C1‐INH levels and activity and C4 levels were low in the HAE‐C1‐INH patients. Spontaneous plasma kallikrein activity in patients in remission was higher than in controls (P = 0.001) and increased during acute attacks (P = 0.01), whereas the capacity of inhibiting kallikrein activity was lower in patients in remission than in controls (P = 0.001) and further reduced during attacks (P = 0.001). HAE‐C1‐INH patients in remission had higher levels of cleaved HK than controls (P = 0.001), and these further increased during acute attacks (P = 0.001). Cleaved HK levels were higher in highly symptomatic HAE‐C1‐INH patients than in those with less frequent attacks (P = 0.001). Thirty‐five different mutations in the C1‐INH gene were equally distributed in patients with different attack frequencies.
Conclusions
Measuring plasma levels of cleaved HK may be a sensitive mean of assessing disease severity in HAE‐C1‐INH patients.
Background
Recommended management of attacks of hereditary angioedema (HAE) due to C1 esterase inhibitor (C1‐INH) deficiency (C1‐INH‐HAE) includes therapy with exogenous C1INH. ...Thrombotic/thromboembolic events (TEE) have been reported with plasma‐derived C1INH, but so far none with recombinant human C1INH (rhC1INH). This phase III, randomized, placebo (saline)‐controlled study evaluated the safety of rhC1INH 50 IU/kg for the treatment of acute attacks in 74 patients with C1‐INH‐HAE.
Methods
Monitoring for TEE and assessment of risk of deep vein thrombosis (DVT) by the Wells prediction rule were performed, and levels of fibrin degradation products (plasma D‐dimers) were assessed before study drug administration (baseline), 2 h, and 7 days posttreatment.
Results
Plasma D‐dimer levels were elevated in 80% of the patients (median 25th–75th percentiles: 2149 480–5105 μg/l; normal ≤250 μg/l) and were higher in patients with submucosal (abdominal, oropharyngeal–laryngeal) attacks (3095 890–10000 μg/l; n = 29) compared with subcutaneous (peripheral, facial) attacks (960 450–4060 μg/l; n = 35). Median plasma D‐dimer levels were comparable across treatment groups at baseline (1874 475–4568 μg/l rhC1INH; 2259 586–7533 μg/l saline) and 2 h postinfusion (2389 760–4974 μg/l rhC1INH; 2550 310–8410 μg/l saline); median plasma D‐dimer levels were decreased by Day 7 in both groups (425 232–3240 μg/l rhC1INH; 418 246–2318 μg/l saline). No increased risk of DVT was identified, nor any TEE reported in rhC1INH treated or controls.
Conclusion
Elevated plasma D‐dimer levels were associated with acute C1‐INH‐HAE attacks, particularly with submucosal involvement. However, rhC1INH therapy was not associated with thrombotic events.
We present the first quantitative detection of large-scale filamentary structure at z NOT approximately equal to 0.7 in the large cosmological volume probed by the VIMOS Public Extragalactic ...Redshift Survey (VIPERS). We use simulations to show the capability of VIPERS to recover robust topological features in the galaxy distribution, in particular the filamentary network. We then investigate how galaxies with different stellar masses and stellar activities are distributed around the filaments, and find a significant segregation, with the most massive or quiescent galaxies being closer to the filament axis than less massive or active galaxies. The signal persists even after downweighting the contribution of peak regions. Our results suggest that massive and quiescent galaxies assemble their stellar mass through successive mergers during their migration along filaments towards the nodes of the cosmic web. On the other hand, low-mass star-forming galaxies prefer the outer edge of filaments, a vorticity-rich region dominated by smooth accretion, as predicted by the recent spin alignment theory. This emphasizes the role of large-scale cosmic flows in shaping galaxy properties.
Background
The first classification of angioedema without wheals was recently reported and comprises different forms of the disease distinguished by aetiology, mediator of oedema and inheritance.
...Methods
In total, 1725 consecutive patients with angioedema without wheals were examined at our centre between 1993 and 2012. We excluded from the analysis 667 patients because of incomplete data or because angioedema was related to a specific factor.
Results
According to the new classification of angioedema, the 1058 patients included in this analysis were diagnosed with hereditary (HAE; n = 377) or acquired angioedema (AAE; n = 681). The former group included HAE with C1‐inhibitor (C1‐INH) deficiency (C1‐INH‐HAE; n = 353) and HAE with normal C1‐INH levels (n = 24), of which six had a factor XII mutation (FXII‐HAE) and 18 had disease of unknown origin (U‐HAE). The AAE group included disease with C1‐INH deficiency (C1‐INH‐AAE; n = 49), AAE related to angiotensin‐converting enzyme inhibitor treatment (n = 183), idiopathic histaminergic (IH‐AAE; n = 379) and idiopathic nonhistaminergic angioedema (InH‐AAE; n = 70). We compared hereditary and AAE with uncertain aetiopathogenesis: the FXII‐HAE and U‐HAE groups pooled (FXII/U‐HAE) versus InH‐AAE. The median age at onset of FXII/U‐HAE and InH‐AAE was 26 and 38 years, respectively. In addition, 56% of patients with FXII/U‐HAE and 81% of those with InH‐AAE reported more than five attacks per year (median duration of 48 h). The location of angioedema in patients with FXII/U‐HAE versus those with InH‐AAE was the following: face, 70% versus 86%; tongue, oral cavity or larynx, 55% versus 68%; limbs, 70% versus 56%; and gastrointestinal mucosa, 50% versus 20%. Prophylaxis with tranexamic acid was effective in all six patients with U‐HAE and in 37 of 38 with InH‐AAE who were started on this treatment.
Conclusion
Our findings in this cohort of patients with angioedema provide new information on the clinical characteristics, diagnosis and treatment of this disease.
To cite this article: Zanichelli A, Vacchini R, Badini M, Penna V, Cicardi M. Standard care impact on angioedema because of hereditary C1 inhibitor deficiency: a 21-month prospective study in a ...cohort of 103 patients. Allergy 2011; 66: 192-196. ABSTRACT: Background: Hereditary angioedema (HAE) due to the deficiency of C1 inhibitor (C1-INH) causes chronically recurrent cutaneous, abdominal and laryngeal angioedema that are disabling and potentially life-threatening. Objective: We designed a prospective study to quantify the residual disease in patients with HAE treated according to the existing consensus documents. Methods: Data were collected from diaries recording occurrence, duration, location and treatment of acute angioedema attacks. A total of 386 semesters properly completed were analyzed. Forty-seven of 103 patients were on prophylactic treatment, 41 with attenuated androgens and six with tranexamic acid. A total of 1532 angioedema attacks (one every 45.3 days) were registered. Results: Peripheral attacks were the most frequent (698), followed by abdominal (503) and combined locations (232), laryngeal edema was less common (99). Patients on prophylaxis with attenuated androgens had 7.7 attacks/year lasting 1.47 days, those on tranexamic acid had 8.1 attacks/year lasting 1.59 days, and those without prophylaxis had 8.9 attacks/year lasting 1.68. Plasma-derived C1-INH was used by 44 patients to treat a total of 376 acute attacks that resolved faster (1.1 day) than those not treated (1.85 day) or treated with tranexamic acid (1.79 day). No adverse events related to C1-INH infusion were reported. Conclusion: Our data demonstrate that tranexamic acid is not effective in the treatment of acute attacks and indicate that under the current therapeutic approach, the HAE related disability is effectively but partially reduced. Incomplete success does not appear to depend on limited efficacy of the drugs but on their limited use that can be overcome by implementing specific treatment strategies.
Aims. We compute photometric redshifts in the fourth public release of the Canada-France-Hawaii Telescope Legacy Survey. This unique multi-colour catalogue comprises $u^*, g', r', i', z'$ photometry ...in four deep fields of 1 deg2 each and 35 deg2 distributed over three wide fields. Methods. We used a template-fitting method to compute photometric redshifts calibrated with a large catalogue of 16 983 high-quality spectroscopic redshifts from the VVDS-F02, VVDS-F22, DEEP2, and the zCOSMOS surveys. The method includes correction of systematic offsets, template adaptation, and the use of priors. We also separated stars from galaxies using both size and colour information. Results. Comparing with galaxy spectroscopic redshifts, we find a photometric redshift dispersion, $\sigma_{\Delta z/(1+z_{\rm s})}$, of 0.028–0.30 and an outlier rate, $|\Delta z| \ge 0.15\times (1+z_{\rm s})$, of 3–4% in the deep field at $i'_{\rm AB}$ < 24. In the wide fields, we find a dispersion of 0.037–0.039 and an outlier rate of 3–4% at $i'_{\rm AB}$ < 22.5. Beyond $i'_{\rm AB}$ = 22.5 in the wide fields the number of outliers rises from 5% to 10% at $i'_{\rm AB}$ < 23 and $i'_{\rm AB}$ < 24, respectively. For the wide sample the systematic redshift bias stays below 1% to $i'_{\rm AB}$ < 22.5, whereas we find no significant bias in the deep fields. We investigated the effect of tile-to-tile photometric variations and demonstrated that the accuracy of our photometric redshifts is reduced by at most 21%. Application of our star-galaxy classifier reduced the contamination by stars in our catalogues from 60% to 8% at $i'_{\rm AB}$ < 22.5 in our field with the highest stellar density while keeping a complete galaxy sample. Our CFHTLS T0004 photometric redshifts are distributed to the community. Our release includes 592891 ($i'_{\rm AB}$ < 22.5) and 244701 ($i'_{\rm AB}$ < 24) reliable galaxy photometric redshifts in the wide and deep fields, respectively.
zCOSMOS is a large-redshift survey that is being undertaken in the COSMOS field using 600 hr of observation with the VIMOS spectrograph on the 8 m VLT. The survey is designed to characterize the ...environments of COSMOS galaxies from the 100 kpc scales of galaxy groups up to the 100 Mpc scale of the cosmic web and to produce diagnostic Information on galaxies and active galactic nuclei. The zCOSMOS survey consists of two parts: (1) zCOSMOS-bright, a magnitude-limited I-band ha < 22.5 sample of about 20,000 galaxies with 0.1 < z < 1.2 covering the whole 1.7 deg super(2) COSMOS ACS field, for which the survey parameters at z similar to 0.7 are designed to be directly comparable to those of the 2dFGRS at z similar to 0.1; and (2) zCOSMOS-deep, a survey of approximately 10,000 galaxies selected through color-selection criteria to have 1.4 < z < 3.0, within the central 1 deg super(2). This paper describes the survey design and the construction of the target catalogs and briefly outlines the observational program and the data pipeline. In the first observing season, spectra of 1303 zCOSMOS-bright targets and 977 zCOSMOS-deep targets have been obtained. These are briefly analyzed to demonstrate the characteristics that may be expected from zCOSMOS, and particularly zCOSMOS-bright, when it is finally completed between 2008 and 2009. The power of combining spectroscopic and photometric redshifts is demonstrated, especially in correctly identifying the emission line in single-line spectra and in determining which of the less reliable spectroscopic redshifts are correct and which are incorrect. These techniques bring the overall success rate in the zCOSMOS-bright so far to almost 90% and to above 97% in the 0.5 < z < 0.8 redshift range. Our zCOSMOS-deep spectra demonstrate the power of our selection techniques to Isolate high-redshift galaxies at 1.4 < z < 3, C and of VIMOS to measure their redshifts using ultraviolet absorption lines.
This paper presents a survey of microwave front-end receivers installed at radio telescopes throughout the world. This unprecedented analysis was conducted as part of a review of front-end ...developments for Italian radio telescopes, initiated by the Italian National Institute for Astrophysics in 2016. Fifteen international radio telescopes have been selected to be representative of the instrumentation used for radio astronomical observations in the frequency domain from 300 MHz to 116 GHz. A comprehensive description of the existing receivers is presented and their characteristics are compared and discussed. The observing performances of the complete receiving chains are also presented. An overview of ongoing developments illustrates and anticipates future trends in front-end projects to meet the most ambitious scientific research goals.
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