Syndromic (phenotypic) diarrhea in early infancy

Syndromic diarrhea (SD), also known as phenotypic diarrhea (PD) or tricho-hepato-enteric syndrome (THE), is a congenital enteropathy presenting with early-onset of severe diarrhea requiring parenteral nutrition (PN). To date, no epidemiological data are available. The estimated prevalence is approximately 1/300,000–400,000 live births in Western Europe. Ethnic origin does not appear to be associated with SD. Infants are born small for gestational age and present with facial dysmorphism including prominent forehead and cheeks, broad nasal root and hypertelorism. Hairs are woolly, easily removed and poorly pigmented. Severe and persistent diarrhea starts within the first 6 months of life (≤ 1 month in most cases) and is accompanied by severe malabsorption leading to early and relentless protein energy malnutrition with failure to thrive. Liver disease affects about half of patients with extensive fibrosis or cirrhosis. There is currently no specific biochemical profile, though a functional T-cell immune deficiency with defective antibody production was reported. Microscopic analysis of the hair show twisted hair (pili torti), aniso- and poilkilotrichosis, and trichorrhexis nodosa. Histopathological analysis of small intestine biopsy shows non-specific villous atrophy with low or no mononuclear cell infiltration of the lamina propria, and no specific histological abnormalities involving the epithelium. The etiology remains unknown. The frequent association of the disorder with parental consanguinity and/or affected siblings suggests a genetic origin with an autosomal recessive mode of transmission. Early management consists of total PN. Some infants have a rather milder phenotype with partial PN dependency or require only enteral feeding. Prognosis of this syndrome is poor, but most patients now survive, and about half of the patients may be weaned from PN at adolescence, but experience failure to thrive and final short stature

Rationales, design and recruitment for the Elfe longitudinal study

Background Many factors act simultaneously in childhood to influence health status, life chances and well being, including pre-birth influences, the environmental pollutants of early life, health status but also the social influences of family and school. A cohort study is needed to disentangle these influences and explore attribution. Methods Elfe will be a nationally representative cohort of 20 000 children followed from birth to adulthood using a multidisciplinary approach. The cohort will be based on the INSEE Permanent Demographic Panel (EDP) established using census data and civil records. The sample size has been defined in order to match the representativeness criteria and to obtain some prevalence estimation, but also to address the research area of low exposure/rare effects. The cohort will be based on repeated surveys by face to face or phone interview (at birth and each year) as well as medical interview (at 2 years) and examination (at 6 years). Furthermore, biological samples will be taken at birth to evaluate the foetal exposition to toxic substances, environmental sensors will be placed in the child's homes. Pilot studies have been initiated in 2007 (500 children) with an overall acceptance rate of 55% and are currently under progress, the 2-year survey being carried out in October this year. Discussion The longitudinal study will provide a unique source of data to analyse the development of children in their environment, to study the various factors interacting throughout the life course up to adulthood and to determine the impact of childhood experience on the individual's physical, psychological, social and professional development

Calificaciones y Empleo (no. 49 2006)

Un tercio de los asalariados se forma cada año y, al mismo tiempo, un cuarto declara tener necesidades de formación no satisfechas. Dos cifras que trazan el marco de un cuadro en el que la formación continua no responde a los deseos de todos. Constatación sorprendente, también son los menos califi cados, y por lo tanto los más expuestos a los imprevistos del mercado de trabajo, los menos formados y al mismo tiempo los menos insatisfechos. Sin embargo, es para estos últimos que la formación puede ocupar un papel determinante. Esta falta aparente de apetito por la formación está vinculada en gran medida con la percepción que los aslariados tienen de su contexto, de sus desafíos y de sus benefi cios. Volver más visible la formación, mediante una mejor información con mayor claridad acerca de las ventajas que pueden esperarse de ella, podría sin duda despertar muchos apetitos. Dimensiones francesas y europeas de la formación y del empleo - convenio Piette/Céreq año 2006 Documento de trabajo resultado del Convenio entre el Centre d études et de recherches sur les qualifi cations (Céreq) de Francia y el Programa de Investigaciones Económicas sobre Tecnología, Trabajo y Empleo (Piette) del Conicet, Argentina. Traducción: Irène Brousse. Supervisión técnica: Julio C. Neffa. Corrección: Graciela Torrecillas, Coordinación y realización: Dominique Bally. Título original: Aux origines de linégale appétence des salariés pour la formation, Bref Céreq Nro 209, junio 2004. Céreq: 10, place de la Joliette - BP 21321 - 13567 Marseille Cedex 02, Francia. Tel. 04 91 13 28 28; Fax 04 91 13 28 80; e-mail: [email protected]; http://www.cereq.fr Ceil-Piette (Conicet): Saavedra 15 P.B. - CP 1083 - Buenos Aires, Argentina. Tel./Fax (5411) 4953 7651/9853; e-mail: [email protected]; http://www.ceil-piette.gov.ar-Los asalariados menos califi cados declaran tener menos necesidades de formación -La percepción del contexto influye en el deseo de formarse -De la información a la formación -La esperanza de un beneficio salarial a menudo no cumplid

Understanding copper sulfide formation from layered template and their use as power electrode materials in aqueous electrolyte

International audienceCopper sulfide has received increasing attention as an electrode material in past decades. In this study, we report the synthesis of copper sulfide with layered copper hydroxide salt (LHS) (Cu2(OH)3NO3) precursors using different protocols. X-ray diffraction suggests the presence of numerous non-stoichiometric phases (Cu1-xS) and not a pure covellite phase and SEM images show particles with flower-like shape but different in size. The solidstate kinetic parameters of the reaction refined by the JMAK method indicate a pseudomorphic transformation controlled by 1D diffusion, different in term of precursors phase, reagents and protocol applied. The microwave method needs less energy to achieve the transformation than the amine digestion (AD) method and the morphology of particles is also different. Tested in sodium acetate electrolyte, CuS provides a maximum capacity of 67 mAh/g for AD, which is much higher than for the commercial grade CuS. This is explained by the difference in nanostructuration of the flower-like shape particles obtained from the layered template. Finally, CuS is used as both a positive and negative electrode material in a complete aqueous battery system but its redox process, which is strongly diffusion limited especially in the lower potential domain, prevents the whole system from operating at high power

Interleaved Electroactive Molecules into LDH Working on Both Electrodes of an Aqueous Battery-Type Device

International audienceBy selecting two electroactive species immobilized in a layered double hydroxide backbone (LDH) host, one able to act as a positive electrode material and the other as a negative one, it was possible to match their capacity to design an innovative energy storage device. Each electrode material is based on electroactive species, riboflavin phosphate (RF) on one side and ferrocene carboxylate (FCm) on the other, both interleaved into a layered double hydroxide (LDH) host structure to avoid any possible molecule migration and instability. The intercalation of the electroactive guest molecules is demonstrated by X-ray diffraction with the observation of an interlayer LDH spacing of about 2 nm in each case. When successfully hosted into LDH interlayer space, the electrochemical behavior of each hybrid assembly was scrutinized separately in aqueous electrolyte to characterize the redox reaction occurring upon cycling and found to be a rapid faradic type. Both electrode materials were placed face to face to achieve a new aqueous battery (16C rate) that provides a first cycle-capacity of about 7 mAh per gram of working electrode material LDH/FCm at 10 mV/s over a voltage window of 2.2 V in 1M sodium acetate, thus validating the hybrid LDH host approach on both electrode materials even if the cyclability of the assembly has not yet been met

Interleaved Electroactive Molecules into LDH Working on Both Electrodes of an Aqueous Battery-Type Device

By selecting two electroactive species immobilized in a layered double hydroxide backbone (LDH) host, one able to act as a positive electrode material and the other as a negative one, it was possible to match their capacity to design an innovative energy storage device. Each electrode material is based on electroactive species, riboflavin phosphate (RF) on one side and ferrocene carboxylate (FCm) on the other, both interleaved into a layered double hydroxide (LDH) host structure to avoid any possible molecule migration and instability. The intercalation of the electroactive guest molecules is demonstrated by X-ray diffraction with the observation of an interlayer LDH spacing of about 2 nm in each case. When successfully hosted into LDH interlayer space, the electrochemical behavior of each hybrid assembly was scrutinized separately in aqueous electrolyte to characterize the redox reaction occurring upon cycling and found to be a rapid faradic type. Both electrode materials were placed face to face to achieve a new aqueous battery (16C rate) that provides a first cycle-capacity of about 7 mAh per gram of working electrode material LDH/FCm at 10 mV/s over a voltage window of 2.2 V in 1M sodium acetate, thus validating the hybrid LDH host approach on both electrode materials even if the cyclability of the assembly has not yet been met

Electrochemical Behavior of Morphology-Controlled Copper (II) Hydroxide Nitrate Nanostructures

International audienceNanostructure control is an important issue when using electroactive materials in energy conversion and storage devices. In this study, we report various methods of synthesis of nanostructured copper (II) hydroxide nitrate (Cu-2(OH)(3)NO3) with a layered hydroxide salt (LHS) structure using various synthesis methods and investigate the correlation between nanostructure, morphology, and their pseudocapacitive electrochemical behavior. The variations in nanostructure size and morphology were comprehensively explored by combining X-ray diffraction (XRD) and scanning electron microscopy (SEM), while the electrochemical activity was characterized using cyclic voltammetry. We demonstrate that Cu-2(OH)(3)NO3-LHS nanostructured submicron particles produced by alkaline precipitation with 88% of the copper cations can cycle with a two-electron redox process. Unfortunately, the electroactivity decreases rapidly from the first cycle due to the occurrence of structural transformations and subsequent electrochemical grinding. However, samples obtained by ultrasonication and microwave synthesis, two original synthesis methods for LHS materials, formed of nanosized crystalline domains agglomerated in micron-sized particles, represent a good compromise between capacity and cyclability. Moreover, by using pair distribution function analysis on electrode materials after repeated cycling, we were able to follow the chemical and structural changes occurring in Cu-2(OH)(3)NO3 materials during electrochemical cycling with first a quick transformation to Cu2O and then the appearance of Cu metal and copper acetate Cu(II)(2)(O2CCH3)(4)center dot 2H(2)O

Effects of tumor necrosis factor antagonist treatment on hepatitis C-related immunological abnormalities.

International audienceBACKGROUND: Chronic hepatitis C infection is frequently associated with a mixed cryoglobulinaemia and circulating auto-antibodies, especially anti-smooth muscle cells (SMA) and anti-liver/kidney/microsome type 1 (LKM-1) anti-tissue antibodies. Treatments with TNF antagonists favour the emergence of auto-antibodies, and particularly anti-dsDNA antibodies. OBJECTIVE: To determine the impact of TNF antagonists on hepatitis C-related immune abnormalities. METHODS: We prospectively monitored for 14 weeks, six patients with actively replicating chronic hepatitis C, initiating an anti-TNF treatment for an associated rheumatoid arthritis. RESULTS: Anti-nuclear and anti-dsDNA antibodies were induced in two and three patients, respectively. Treatment had no impact on the production of antibodies against extractable nuclear antigens, and it did not induce anti-tissues antibodies in any patient. Cryoglobulinaemia appeared in 2/6 patients, and it persisted in 2 others. No patient developed any news signs of autoimmunity. HCV viraemia remained unchanged. CONCLUSIONS: Induction of auto-antibodies by TNF antagonist treatments does not involve anti-tissues antibodies, even in patients with actively replicating chronic hepatitis C prone to produce anti-SMA and anti-LKM-1 antibodies. In contrast, TNF antagonists may favour emergence of cryoglobulinaemia in such patients

Intercalated Organic Redox-active Anions for Enhanced Capacity of Layered Double Hydroxides

International audienceA Layered Double Hydroxide (LDH) compound LDH ([Mg 2 Al(OH) 6 ] + x 2 H 2 O) intercalated with a redox active organic anion, Anthraquinone-2-sulfonate (AQS), has been envisioned as an electrode material for high power aqueous based battery. The purpose is to use this interlayer redox active molecule for the enhancement of the specific capacity at the LDH composite electrode, which should allow fast charge transfer at the negative electrode for high power storage applications. This is achieved by the reduction of AQS in charge and oxidation in discharge within a redox inactive LDH matrix. The first charge of this new material [Mg 2 Al(OH) 6 ] + [AQSO 3 ] − x 2 H 2 O leads to a capacity of 100 mAh g −1 at − 0.78 V vs Ag/AgCl (based on the weight of the active material) when operated in aqueous 1 M sodium acetate electrolyte. However, low cycling stability was observed, since a drastic loss in specific capacity occurs after the first charge. This study focuses at elucidating the mechanism behind this phenomenon via in situ UV/vis experiments. Subsequently, the dissolution of charged AQS anions into the electrolyte during the first charge of the anode has been identified and quantified. Such understanding of fading mechanism might lead to the design of improved LDH-based electrodes, which utilize redox active anions working in the positive potential range with enhanced cycling ability