Acute Weston Hurst necrotizing hemorrhagic leukoencephalitis

The clinical and pathological findings of a 43-year-old woman, diagnosed as having acute hemorrhagic leukoencephalitis at postmortem examination, are presented. The acute hemorrhagic leukoencephalitis affects mainly young adults and is the most fulminant from of demyelinating disease. It is frequently preceded by a respiratory infection. Diagnosis is facilitated by CT scanning and MRI, which reveal the massive lesion in the cerebral white matter. Many cases terminate fatally in 2 or 4 days, but in others survival is longer. The pathological findings are distinctive.     

Alcohol-induced changes of {beta}-lactoglobulin - retinol-binding stoichiometry

It has been demonstrated using CD that ethanol induces importantsecondary structure changes of ß-lactoglobulin. CDspectra indicate that ß-lactoglobulin secondary structure,which is mainly composed of ß-strands, becomes mostly-helical under the influence of the solvent polarity changes.The midpoint of ß-strand/-helix transition in ß-lactoglobulinis observed at dielectric constant {small tilde}60 (35% ethanol;v/v). According to CD measurements, the ethanol-dependent secondarystructure changes are reversible. The alkylation of lysines-NH₂ in ß-lactoglobulin weakens the central ß-barrelstructure, since the ß-strand/-helix transition midpointof alkylated ß-lactoglobulin is shifted to lower ethanolconcentration (25% ethanol; v/v). ß-Lactoglobulinstructural changes are triggering the dissociation of the ß-lactoglobulin- retinol complex as judged from complete quenching of its fluorescencein ethanol concentration >30% (v/v). However, in 20% ethanol(v/v), ß-lactoglobulin still retains most of its nativesecondary structure as shown by CD and, in this condition, oneß-lactoglobulin molecule binds an additional secondretinol molecule. This suggests that the highly populated speciesobserved around 20% ethanol (v/v) might represent an intermediatestate able to bind two molecules of retinol.     

An electrical conductivity and TEM study of the sulphurization by gaseous H2S of NiO single crystals

An electrical conductivity and transmission electron microscopy study of the very early stages of the sulphurization by H₂S of NiO single crystals were carried out at low pressure (p<20 Torr) and at T<700 K. For treatments at pH₂S < 10^-4 Torr 2 main stages of reaction were found: (a) a topotactical reduction of the oxide producing Ni islands with a diameter of about 20 nm and (b) an epitaxial overgrowth of Ni₃S₂ from the metallic islands. The maximal conversion rate of the NiO sulphurization is very low and depends on the states of the crystal surface. For treatments at pH₂S 20 Torr a non-stoichiometric nickel sulphide was identified.     

Adaptive control of linear systems with Markov perturbations

The stochastic model considered is a linear jump diffusion process X for which the coefficients and the jump processes depend on a Markov chain Z with finite state space. First, we study the optimal filtering and control problem for these systems with non-Gaussian initial conditions, given noisy observations of the state X and perfect measurements of Z. We derive a new sufficient condition which ensures the existence and the uniqueness of the solution of the nonlinear stochastic differential equations satisfied by the output of the filter. We study a quadratic control problem and show that the separation principle holds. Next, we investigate an adaptive control problem for a state process X defined by a linear diffusion for which the coefficients depend on a Markov chain, the processes X and Z being observed in independent white noises. Suboptimal estimates for the process X, Z and approximate control law are investigated for a large class of probability distributions of the initial state. Asymptotic properties of these filters and this control law are obtained. Upper bounds for the corresponding error are given     

Autoimmune Neutropenia and Immune-Dysregulation in a Patient Carrying a TINF2 Variant

In recent years, the knowledge about the immune-mediated impairment of bone marrow precursors in immune-dysregulation and autoimmune disorders has increased. In addition, immune-dysregulation, secondary to marrow failure, has been reported as being, in some cases, the most evident and early sign of the disease and making the diagnosis of both groups of disorders challenging. Dyskeratosis congenita is a disorder characterized by premature telomere erosion, typically showing marrow failure, nail dystrophy and leukoplakia, although incomplete genetic penetrance and phenotypes with immune-dysregulation features have been described. We report on a previously healthy 17-year-old girl, with a cousin successfully treated for acute lymphoblastic leukemia, who presented with leukopenia and neutropenia. The diagnostic work-up showed positive anti-neutrophil antibodies, leading to the diagnosis of autoimmune neutropenia, a slightly low NK count and high TCR-αβ+-double-negative T-cells. A next-generation sequencing (NGS) analysis showed the 734C>A variant on exon 6 of the TINF2 gene, leading to the p.Ser245Tyr. The telomere length was short on the lymphocytes and granulocytes, suggesting the diagnosis of an atypical telomeropathy showing with immune-dysregulation. This case underlines the importance of an accurate diagnostic work-up of patients with immune-dysregulation, who should undergo NGS or whole exome sequencing to identify specific disorders that deserve targeted follow-up and treatment.     

Implication of milk and milk products in food-borne diseases in France and in different industrialised countries

A study was carried out to estimate the proportion of diseases due to milk and milk products among food-borne diseases recorded in France and in other countries since 1980. Particular attention was given to whether the milk involved was heat-treated or not. Four etiologic agents were considered: Salmonella spp., Staphylococcus aureus, Listeria monocytogenes, and pathogenic Escherichia coli. An overview of food-borne disease annual reports from seven countries indicated that milk and milk products were implicated in 1-5% of the total bacterial outbreaks; however, details about the type of product and milk involved were usually not provided. When considering 60 outbreaks and four single cases described in the literature and implicating milk and milk products, confirmed or suspected food vehicles were distributed as follows: milk, 39.1%, cheese, 53.1%, other milk products, 7.8%. Overall, 32.8% of the food vehicles were made from pasteurised milk; 37.5% from raw milk; 10.9% from milk stated as "unpasteurised"; and 18.8% from unspecified milk. Salmonella spp. were responsible for 29 outbreaks, L. monocytogenes for 10 outbreaks and four well-documented single cases, pathogenic E. coli for 11 outbreaks, and S. aureus for 10 outbreaks. Analysis of unpublished data about food-borne disease outbreaks, listeriosis excluded, collected by the coordinator of the French surveillance system from 1992 to 1997, revealed 69 documented outbreaks for which milk and milk products were confirmed as the vehicle by the isolation of the etiologic agent. The food vehicles were distributed as follows: milk, 10%; cheese, 87%; others, 3%. UHT milk accounted for 1.5%, raw milk and raw milk products for 48%, and milk and milk products from unspecified milk for 50.5% of the 69 outbreaks. S. aureus was by far the most frequent pathogen associated with these outbreaks (85.5% of the outbreaks), followed by Salmonella (10.1%). This study demonstrates the limitations of the surveillance systems and the difficulties in estimating the contribution of milk and milk products to food-borne diseases. In particular, it was not possible to find out in many outbreaks what heat treatment, if any, the milk had undergone.     

Effect of micropores diffusion on kinetics of CH4 decomposition over a wood-derived carbon catalyst

In order to optimise hydrogen production from biomass gasification, catalytic conversion of methane contained in a surrogate biomass syngas (CH₄ 14%; CO 19%; CO₂ 14%; H₂ 16%; H₂O 30%; N₂ 7%) is investigated over a fixed bed of porous wood char as a function of temperature (800–1000 °C) and space time (1.6–6.2 min g L⁻¹). Determination of Thiele modulus evidences a change of kinetic regime from chemically- to diffusion-controlled when the temperature increases; this finding is particularly relevant when porous chars having an average pore width of 1 nm are used as catalysts. Mass diffusion transfers are accounted for by a model introducing an internal effectiveness factor. Knudsen diffusion in micropores is shown to limit the conversion rate of methane per unit mass of catalyst, and explains why such a rate is not proportional to the BET surface area, especially when the latter is higher than typically 300 m²/g. It is concluded that diffusion limitations in micropores should be taken into account, otherwise underestimated activation energy and intrinsic kinetic constant are obtained in some experimental conditions.     

Prediction of the rheology parameters of ripened semi-hard cheeses using fluorescence spectra in the UV and visible ranges recorded at a young stage

Storage modulus (G′), loss modulus (G″), strain, tan (δ) and complex viscosity (η*) of 20 semi-hard cheeses were measured by dynamic oscillatory analysis after 2, 30 and 60 days of ripening. On the same cheeses and at the same ages, tryptophan and riboflavin fluorescence spectra were recorded. The aim was to predict the rheology parameters of ripened cheeses from spectra recorded on these cheeses at a young stage. Using partial least square, tryptophan fluorescence spectra recorded at 20 °C on 2-days-old cheeses predicted G′, G″, strain, tan (δ) and η* measured at 80 °C on the 60-days-old cheeses with correlation coefficients (R) of 0.98, 0.97, 0.98, 0.98 and 0.97, respectively. Riboflavin fluorescence spectra gave slightly lower correlation coefficients of 0.88, 0.88, 0.92, 0.87 and 0.88, respectively. Dependent only on visible light, the riboflavin fluorescence spectra potentially provide viable and economic prediction of the rheology of ripe cheese.     

In Vitro Models for Studying Secondary Plant Metabolite Digestion and Bioaccessibility

There is an increased interest in secondary plant metabolites, such as polyphenols and carotenoids, due to their proposed health benefits. Much attention has focused on their bioavailability, a prerequisite for further physiological functions. As human studies are time consuming, costly, and restricted by ethical concerns, in vitro models for investigating the effects of digestion on these compounds have been developed and employed to predict their release from the food matrix, bioaccessibility, and assess changes in their profiles prior to absorption. Most typically, models simulate digestion in the oral cavity, the stomach, the small intestine, and, occasionally, the large intestine. A plethora of models have been reported, the choice mostly driven by the type of phytochemical studied, whether the purpose is screening or studying under close physiological conditions, and the availability of the model systems. Unfortunately, the diversity of model conditions has hampered the ability to compare results across different studies. For example, there is substantial variability in the time of digestion, concentrations of salts, enzymes, and bile acids used, pH, the inclusion of various digestion stages; and whether chosen conditions are static (with fixed concentrations of enzymes, bile salts, digesta, and so on) or dynamic (varying concentrations of these constituents). This review presents an overview of models that have been employed to study the digestion of both lipophilic and hydrophilic phytochemicals, comparing digestive conditions in vitro and in vivo and, finally, suggests a set of parameters for static models that resemble physiological conditions.