Prenatal molecular diagnosis in hypertrophic cardiomyopathy: report of the first case

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease that may cause premature sudden death, especially in teenagers and young adults. The recent progress in the molecular genetics of the disease has made genetic testing sometimes available in clinical practice. We report the case of a couple who still requested prenatal molecular testing after detailed information had been given through a multidisciplinary consultation. Prenatal diagnosis in HCM is associated with complex medical and psychological implications, in addition to general ethical considerations, as the potential value of the diagnosis is counterbalanced by the highly variable expression of the disease and the difficulty in predicting its evolution. The R403L mutation in the MYH7 gene had been previously identified in this family, characterized by a malignant form of HCM. In the specific context of this case, we decided to agree to the request of the parents and performed the prenatal diagnosis. To the best of our knowledge, this is the first report of a prenatal molecular diagnosis performed in the context of HCM. Copyright © 2004 John Wiley & Sons, Ltd.     

Atmospheric photochemistry and secondary aerosol formation of urban air in Lyon, France

Photochemical aging of volatile organic compounds (VOCs) in the atmosphere is an important source of secondary organic aerosol (SOA). To evaluate the formation potential of SOA at an urban site in Lyon (France), an outdoor experiment using a Potential Aerosol Mass (PAM) oxidation flow reactor (OFR) was conducted throughout entire days during January-February 2017. Diurnal variation of SOA formations and their correlation with OH radical exposure (OHexp), ambient pollutants (VOCs and particulate matters, PM), Relative Humidity (RH), and temperature were explored in this study. Ambient urban air was exposed to high concentration of OH radicals with OHexp in range of (0.2–1.2)×10¹² molecule/(cm³?sec), corresponding to several days to weeks of equivalent atmospheric photochemical aging. The results informed that urban air at Lyon has high potency to contribute to SOA, and these SOA productions were favored from OH radical photochemical oxidation rather than via ozonolysis. Maximum SOA formation (36 µg/m³) was obtained at OHexp of about 7.4 × 10¹¹molecule/(cm³?sec), equivalent to approximately 5 days of atmospheric oxidation. The correlation between SOA formation and ambient environment conditions (RH & temperature, VOCs and PM) was observed. It was the first time to estimate SOA formation potential from ambient air over a long period in urban environment of Lyon.     

Strong population differentiation of softshell clams (Mya arenaria) sampled across seven biogeographic marine ecoregions: possible selection and isolation by distance

Twenty-two Mya arenaria samples spanning seven marine ecoregions mostly situated in the Cold Temperate Northwest Atlantic (CTNA) biogeographic province were collected between 2001 and 2010 and genotyped at seven highly polymorphic microsatellite loci to test for population differentiation. Results showed strong regional differentiation with six genetic clusters: (1) Northern Gulf of St. Lawrence (GSL), (2) Magdalen Archipelago, (3) Southern GSL, (4) Lower Atlantic Canada, (5) US Coasts and (6) Northern Europe. Population structure was supported no matter the statistical approach and generally does not reflect the geographical limits of marine ecoregions. A latitudinal cline in allelic richness provides evidence for a northward post-glacial expansion range for this species. While geographical distance explains the genetic variation detected in southern CTNA, increased heterogeneity observed in northern CTNA can be explained by isolation by distance, marine landscaping and presumable selective processes acting at the Mar5 locus. Exclusion of Mar5 from analyses resulted in the detection of three genetic clusters instead of six.     

Recent Evolution of Natural Hazard Management Policy in France,the Example of Serre-Chevalier (French Alps)

Since the 1960s, the development of the Serre-Chevalier winter and summer resort has led to a rapid extension of the urban fabric on the territory of the village of St-Chaffrey. On July 9, 1981, the new neighborhoods built on the alluvial fan of the Verdarel torrent were partly devastated by a channeled debris flow. This disaster prompted the civil authorities to set up an extensive fluvial regulation system, which rapidly contributed to recreating a climate of confidence and security favorable to continuing urban expansion. The combination of catastrophic floods in July 1995, and a new security-oriented national policy on natural hazards, nevertheless led to a return to precautionary measures. The St-Chaffrey town council has therefore been forced by the State to accept a building-free zone on the land-use plan. This measure is perceived as a real denial of the local efficacy of the flood and landslide risk management policy as carried out by the council of St-Chaffrey during the previous 20 years.     

Phytoremediation of zinc and cadmium: A study of arbuscular mycorrhizal hyphae

Arbuscular mycorrhizal fungi (AMF) are microscopic fungi naturally occurring in soil that form a symbiosis with plant roots, producing a highly elaborated hyphal mycelium network in soil.In vitro lab experiments were conducted to determine whether extraradical mycorrhizal hyphae are directly involved in sequestration and uptake of essential zinc and nonessential cadmium by plant roots under toxic concentrations. The research is a continuation of an initial study presented in the Spring 2005 issue of Remediation that focused on the specific role of AMF in the speciation of heavy metals. Thus, this article presents a more expanded view. Results show that zinc and cadmium heavy metals are sequestered and translocated to plant roots via extraradical AMF hyphae. Root/growth media accumulation factors reached 5:1 and 18:1 for zinc and cadmium, respectively. Phytoremediation of heavy metal–contaminated soils can be enhanced by mycorrhizal inoculation. © 2005 Wiley Periodicals, Inc.     

Graft Copolymers, for Erodible Resins, from α-Hydroxyacids Oligomers Macromonomers and Acrylic Monomers

Environmental concerns result in a progressive withdrawal of antifouling paints containing organotin derivatives. The nature of the binders is critical with regard to the erosion of the protecting film through factors such as bond cleavage, dissolution, and diffusion of the degradation products. The versatility of acrylic polymers, due to the possibility of varying their chemical structure had conducted, in the first stage, to combine different types of repeating units in the macromolecular backbone. Formulation and evaluation, in natural sites, of these binders, with a well-defined hydrophobic/hydrophilic balance and with hydrolyzable pendant groups, have shown the possibility to prepare new organotin free resins which can be formulated and which are erodible in seawater over a long period (more than 2 years). A further step has been engaged with the development of graft copolymers containing biocompatible and hydrolyzable oligomers of -hydroxyacids. Their preparation requires the synthesis of -methacryloyloxyoligo--hydroxyacid macromonomers. Copolymers prepared from a mixture of the macromonomer and of an alkyl ester of acrylic acid were formulated with a biocide and deposited on a plate. Their ability to release cuprous oxide, as a model molecule, has been checked and quantified by the inductively coupled plasma analytical method. The uptake of water in the paint, which is enhanced by the hydrolysis of -hydroxyacid oligomers, as determined by the enzymatic measurement of liberated L-lactic acid, conducts to the polymer erosion and to a protecting bioactive surface.     

Motivating workers in emerging countries: universal tools and local adaptations

Two case studies are presented of highly successful factories operating in emerging countries. Sgs‐Thomson in Morocco and Danone in Mexico provide exemplars of ways in which corporate cultures can promote productive, efficient, and interpersonally supportive communities by building upon deep‐seated local values that give these communities special meaning to members. In Morocco, Islamic norms and values combined with Total Quality Management values to transform Sgs‐Thomson's factory culture. In Mexico, norms and values regarding family and the pursuit of a higher moral purpose underpinned a workplace transformation combining economic and social progress. This essay also develops the implications of these cases for the dissemination, adoption, and malleability of global management practices. Copyright © 2002 John Wiley & Sons, Ltd.     

Surface roughness prediction in milling based on tool displacements

In this paper, an experimental device using non-contact displacement sensors for the investigation of milling tool behaviour is presented. It enables the recording of high frequency tool vibrations during milling operations. The aim of this study is related to the surface topography prediction using tool displacements and based on tool center point methodology. From the recorded signals and the machining parameters, the tool deformation is modeled. Then, from the calculated deflection, the surface topography in 3D can be predicted. In recent studies, displacements in XY plane have been measured to predict the surface topography in flank milling. In this article, the angular deflection of the tool is also considered. This leads to the prediction of surfaces obtained in flank milling as well as in end milling operations. Validation tests were carried out: the predicted profiles were compared to the measured profile. The results show that the prediction corresponds well in shape and amplitude with the measurement.