This study investigated interstitial pneumonia associated with amyopathic dermatomyositis, dermatomyositis and polymyositis, paying particular attention to muscular and/or cutaneous manifestations ...and their chronology relative to lung involvement. Patients included four males and 13 females, aged 51.7+/-10.8 yrs, who had surgical lung biopsy. Diagnoses included dermatomyositis (10 patients), polymyositis (four patients) and amyopathic dermatomyositis (three patients). Solitary respiratory manifestations preceded the onset of any skin or muscle disease in four cases (24%). Reticular and ground glass opacities were the most frequent computed tomography (CT) findings. Pathological review showed nonspecific interstitial pneumonia (eleven, 65%; cellular, two; cellular and fibrotic, five; fibrotic, four), usual interstitial pneumonia (two), organising pneumonia (two), lymphocytic interstitial pneumonia (one), and unclassifiable interstitial pneumonia (one). Nonspecific interstitial pneumonia was the most common histological pattern of interstitial pneumonia in patients with amyopathic dermatomyositis (three of three) and in patients with respiratory symptoms as the initial clinical manifestation of the connective tissue disease (three of four). Survival at 5 yrs was 50%. This study shows the clinician should remain alert to potential muscular or cutaneous manifestations whenever a pathological diagnosis of nonspecific interstitial pneumonia is made.
The aim of the present study was to investigate to what extent interstitial lung disease (ILD) in common variable immunodeficiency disorder (CVID)-associated granulomatous disease (GD) is similar to ...pulmonary sarcoidosis 20 patients with CVID/GD were included in a retrospective study conducted by the Groupe Sarcoïdose Francophone. Medical records were centralised. Patients were compared with 60 controls with sarcoidosis. Clinical examination showed more frequent crackles in patients than controls (45% versus 1.7%, respectively; p<0.001). On thoracic computed tomography scans, nodules (often multiple and with smooth margins), air bronchograms and halo signs were more frequent in patients than controls (80% versus 42%, respectively; p=0.004) as well as bronchiectasis (65% versus 23%, respectively; p<0.001). The micronodule distribution was perilymphatic in 100% of controls and in 42% of patients (p<0.001). Bronchoalveolar lavage analysis showed lower T-cell CD4/CD8 ratios in patients than in controls (mean ± sd 1.6 ± 1.1 versus 5.3 ± 4, respectively; p<0.01). On pathological analysis, nodules and consolidations corresponded to granulomatous lesions with or without lymphocytic disorders in most cases. Mortality was higher in patients than controls (30% versus 0%, respectively) and resulted from common variable immunodeficiency complications. ILD in CVID/GD presents a specific clinical picture and evolution that are markedly different from those of sarcoidosis.
CONTEXT: Hoverflies are often used as bio-indicators for ecosystem conservation, but only few studies have actually investigated the key factors explaining their richness in woodlands. OBJECTIVES: In ...a fragmented landscape in southwest France, we investigated the joint effects of woodland area, structural heterogeneity, connectivity and history on the species richness of forest-specialist hoverflies, and whether there was a time lag in the response of hoverflies to habitat changes, and tested the effect of spatiotemporal changes. METHODS: Current species richness was sampled in 48 woodlands using 99 Malaise traps. Structural variables were derived from a rapid habitat assessment protocol. Old maps and aerial photographs were used to extract past and present spatial patterns of the woodlands since 1850. Relationships between species richness and explanatory variables were explored using generalized linear models. RESULTS: We show that current habitat area, connectivity, historical continuity and the average density of tree-microhabitats explained 35 % of variation in species richness. Species richness was affected differently by changes in patch area between 1979 and 2010, depending on woodland connectivity. In isolated woodlands, extinction debt and colonization credit were revealed, showing that even several decades are not sufficient for hoverflies to adapt to landscape-scale habitat conditions. CONCLUSIONS: These findings emphasise the importance of maintaining connectedness between woodlands, which facilitates the dispersion in a changing landscape. Our results also highlight the benefits of using a change-oriented approach to explain the current distribution patterns of species, especially when several spatial processes act jointly.
Cornelia de Lange syndrome is a multisystemic developmental disorder mainly related to de novo heterozygous NIPBL mutation. Recently, NIPBL somatic mosaicism has been highlighted through buccal cell ...DNA study in some patients with a negative molecular analysis on leukocyte DNA. Here, we present a series of 38 patients with a Cornelia de Lange syndrome related to a heterozygous NIPBL mutation identified by Sanger sequencing. The diagnosis was based on the following criteria: (i) intrauterine growth retardation and postnatal short stature, (ii) feeding difficulties and/or gastro‐oesophageal reflux, (iii) microcephaly, (iv) intellectual disability, and (v) characteristic facial features. We identified 37 novel NIPBL mutations including 34 in leukocytes and 3 in buccal cells only. All mutations shown to have arisen de novo when parent blood samples were available. The present series confirms the difficulty in predicting the phenotype according to the NIPBL mutation. Until now, somatic mosaicism has been observed for 20 cases which do not seem to be consistently associated with a milder phenotype. Besides, several reports support a postzygotic event for those cases. Considering these elements, we recommend a first‐line buccal cell DNA analysis in order to improve gene testing sensitivity in Cornelia de Lange syndrome and genetic counselling.
The study of devices to amplify the terahertz (THz) radiations is a subject of intense research among the scientific community owing to its interdisciplinary applications. We explore the possibility ...of amplification of THz radiations by investigating the transport measurements of GaN/AlGaN structures. We investigate the transport characteristics of AlGaN/GaN high-electron-mobility transistor (HEMT), transmission lines model (TLM), and interdigitated finger (IDF) structures as functions of geometry, temperature, and operating conditions. In the transport measurements of HEMT structure at 300 K, the pulsed mode operation attains higher value of the drain current in comparison to the continuous-wave operation mode of the relatively larger geometries which is typically related to the self-heating effect. Nevertheless, the pulsed mode operation transport measurements of the relatively smaller HEMT structures overlap and attain even lower drain current values than that of the CW operation mode measurements whose origin is attributed to the trap manifestation at low temperatures. The major part of the applied voltage on the symmetric IDF structure is spread over the contacts whilst less than
10
%
will effectively be applied on the channel. Moreover, the transport measurements of 5
μ
m long TLM structure at 7 K show a kink at 0.1 V which is originated from the onset of the optical phonon transit time resonance mechanism. The present investigations of AlGaN/GaN structures should be helpful to obtain terahertz amplification in the electronic devices.
Objective
Findings from the WEGENT trial and other short‐term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with ...polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA). This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period.
Methods
Long‐term outcomes in patients enrolled in the WEGENT trial were analyzed according to their randomized treatment group (AZA or MTX). Parameters at trial entry were evaluated as potential prognostic factors for death, relapse, or damage in multivariate models.
Results
Data from 10 years of followup were available for 112 (88.8%) of the 126 original trial participants. The median followup time was 11.9 years (95% confidence interval 95% CI 11.3–12.5 years). In patients receiving AZA and those receiving MTX, the 10‐year overall survival rates were 75.1% (95% CI 64.8–86.9%) and 79.9% (95% CI 70.3–90.8%) (P = 0.56), respectively, and relapse‐free survival rates were 26.3% (95% CI 17.3–40.1%) and 33.5% (95% CI 23.5–47.7%) (P = 0.29), respectively. No between‐treatment differences were observed with regard to rates of relapse, adverse events, damage, survival without severe side effects, and survival without relapse and severe side effects. In analyses limited to the 97 patients with GPA, no between‐treatment differences in survival rates were observed. The 10‐year relapse‐free survival rate was lower in patients with GPA than in patients with MPA. However, in the multivariate analysis, anti–proteinase 3 antineutrophil cytoplasmic antibody (ANCA) positivity, and not GPA, was retained as being independently associated with the relapse rate.
Conclusion
The results of this long‐term analysis confirm that AZA and MTX are comparable treatment options for maintaining remission of GPA or MPA. Despite achieving good overall survival with these treatments, relapse rates, adverse events, and damage remain matters of concern and further studies are needed to reduce their frequency in these ANCA‐associated vasculitides.
The present study is motivated by a need to produce stability modes to assist in the understanding and control of unsteady separated flows. The flow configuration is a NACA 0015 aerofoil with laminar ...leading-edge separation and turbulent recirculation. In previous water tunnel experiments, this flow configuration was measured in an unperturbed (uncontrolled) separated state, and a harmonically perturbed (controlled) reattached state. This study presents numerical data of the unperturbed case, and recovers stability modes to describe the evolution of perturbations in this environment. The unperturbed flow is numerically generated using large eddy simulation. Its temporal properties are quantified via a Fourier analysis of the velocity time history at selected points in space. The leading-edge shear layer instability is characterized by instantaneous vortex structures, and the bluff body shedding is illustrated by proper orthogonal decomposition modes. Statistical measures of the velocity field agree well with the water tunnel measurements. Finally a stability analysis is undertaken using a triple decomposition to distinguish between the time averaged field, the unsteady scales of motion, and a coherent wave (perturbation). This analysis identifies that perturbations in the region immediately downstream of the separated shear layer have the highest spatial growth rates. The associated frequency is of the order of the sub-harmonic of the shear layer instability.
This study compares the impact of A and B-substitution in parent stoichiometric and La-deficient LaFeO
3
perovskites for subsequent development as suitable substrate in three-way catalysis. CO and ...propene oxidation were investigated to probe their oxidative behavior. It was found that the activity cannot be related neither to the specific surface area nor to the nature of oxidic iron species but more likely to intra and extra lattice copper species as well as related defective sites.
SPI: The spectrometer aboard INTEGRAL Vedrenne, G.; Roques, J.-P.; Schönfelder, V. ...
Astronomy & astrophysics,
11/2003, Letnik:
411, Številka:
1
Journal Article
Recenzirano
Odprti dostop
SPI is a high spectral resolution gamma-ray telescope on board the ESA mission INTEGRAL (International Gamma Ray Astrophysics Laboratory). It consists of an array of 19 closely packed germanium ...detectors surrounded by an active anticoincidence shield of BGO. The imaging capabilities of the instrument are obtained with a tungsten coded aperture mask located 1.7 m from the Ge array. The fully coded field-of-view is $16\deg$, the partially coded field of view amounts to $31\deg$, and the angular resolution is $2.5\deg$. The energy range extends from 20 keV to 8 MeV with a typical energy resolution of 2.5 keV at 1.3 MeV. Here we present the general concept of the instrument followed by a brief description of each of the main subsystems. INTEGRAL was successfully launched in October 2002 and SPI is functioning extremely well.
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