Noonan syndrome and related disorders are a group of clinically and genetically heterogeneous conditions caused by mutations in genes of the RAS/MAPK pathway. Noonan syndrome causes multiple congenital anomalies, which are frequently accompanied by hypertrophic cardiomyopathy (HCM). We report here a Tunisian patient with a severe phenotype of Noonan syndrome including neonatal HCM, facial dysmorphism, severe failure to thrive, cutaneous abnormalities, pectus excavatum and severe stunted growth, who died in her eighth month of life. Using whole exome sequencing, we identified a de novo mutation in exon 7 of the RAF1 gene: c.776C > A (p.Ser259Tyr). This mutation affects a highly conserved serine residue, a main mediator of Raf-1 inhibition via phosphorylation. To our knowledge the c.776C > A mutation has been previously reported in only one case with prenatally diagnosed Noonan syndrome. Our study further supports the striking correlation of RAF1 mutations with HCM and highlights the clinical severity of Noonan syndrome associated with a RAF1 p.Ser259Tyr mutation.

As part of the evaluation of the French plan for the elimination of measles and rubella, we conducted a seroprevalence survey in 2013, aimed at updating seroprevalence data for people 18–32 years old. A secondary objective was to estimate measles incidence in this population during the 2009–2011 outbreak, and thus estimate the exhaustiveness of measles mandatory reporting. We used a cross-sectional survey design, targeting blood donors 18–32 years old, living in France since 2009, who came to give blood in a blood collecting site. We included 4647 people in metropolitan France, 806 people in Réunion Island and 496 in the French Caribbean. A further 3942 individuals were interviewed in the south-east region of metropolitan France to estimate the exhaustiveness of measles mandatory reporting. One of the main findings of this survey is that the proportion of people 18–32 years old susceptible to both measles and rubella infections remained high in France in 2013, 9.2% and 5.4%, respectively, in metropolitan France, even after the promotion campaigns about vaccination catch-up during and following the major measles epidemic in 2009–2011. Applying our results to French census data would suggest that around 1 million people aged 18–32 years old are currently susceptible to measles in France, despite this age group being one of the vaccination targets of the national measles elimination plan. Another important finding is that only an estimated 45% of the true number of cases in this age group was actually notified, despite notification being mandatory.

The present commentary contains a clear and simple guide designed to identify ultra-processed foods. It responds to the growing interest in ultra-processed foods among policy makers, academic researchers, health professionals, journalists and consumers concerned to devise policies, investigate dietary patterns, advise people, prepare media coverage, and when buying food and checking labels in shops or at home. Ultra-processed foods are defined within the NOVA classification system, which groups foods according to the extent and purpose of industrial processing. Processes enabling the manufacture of ultra-processed foods include the fractioning of whole foods into substances, chemical modifications of these substances, assembly of unmodified and modified food substances, frequent use of cosmetic additives and sophisticated packaging. Processes and ingredients used to manufacture ultra-processed foods are designed to create highly profitable (low-cost ingredients, long shelf-life, emphatic branding), convenient (ready-to-consume), hyper-palatable products liable to displace all other NOVA food groups, notably unprocessed or minimally processed foods. A practical way to identify an ultra-processed product is to check to see if its list of ingredients contains at least one item characteristic of the NOVA ultra-processed food group, which is to say, either food substances never or rarely used in kitchens (such as high-fructose corn syrup, hydrogenated or interesterified oils, and hydrolysed proteins), or classes of additives designed to make the final product palatable or more appealing (such as flavours, flavour enhancers, colours, emulsifiers, emulsifying salts, sweeteners, thickeners, and anti-foaming, bulking, carbonating, foaming, gelling and glazing agents).

The aim of the present work was to address experimentally the possible impact of exposure to air pollution during gestation on the differentiation and function of the gonads of the offspring using a rabbit model. Rabbits were exposed daily to diluted diesel exhaust gas or filtered air from the 3rd until the 27th day of gestation, during which time germ cells migrate in genital ridges and divide, and fetal sex is determined. Offspring gonads were collected shortly before birth (28th day of gestation) or after puberty (7.5 months after birth). The structure of the gonads was analyzed by histological and immunohistological methods. Serum concentrations of testosterone and anti-Müllerian hormone were determined using ELISA. The morphology and the endocrine function of the gonads collected just at the arrest of the exposure were similar in polluted and control animals in both sexes. No differences were observed as well in gonads collected after puberty. Sperm was collected at the head of the epididymis in adults. Sperm motility and DNA fragmentation were measured. Among all parameters analyzed, only the sperm DNA fragmentation rate was increased three-fold in exposed males. Mechanisms responsible for these modifications and their physiological consequences are to be further clarified.

The anti-leishmania effects of HIV peptidase inhibitors (PIs) have been widely reported; however, the biochemical target and mode of action are still a matter of controversy in Leishmania parasites. Considering the possibility that HIV-PIs induce lipid accumulation in Leishmania amazonensis, we analysed the effects of lopinavir on the lipid metabolism of L. amazonensis promastigotes. To this end, parasites were treated with lopinavir at different concentrations and analysed by fluorescence microscopy and spectrofluorimetry, using a fluorescent lipophilic marker. Then, the cellular ultrastructure of treated and control parasites was analysed by transmission electron microscopy (TEM), and the lipid composition was investigated by thin-layer chromatography (TLC). Finally, the sterol content was assayed by gas chromatography–mass spectrometry (GC/MS). TEM analysis revealed an increased number of lipid inclusions in lopinavir-treated cells, which was accompanied by an increase in the lipophilic content, in a dose-dependent manner. TLC and GC–MS analysis revealed a marked increase of cholesterol-esters and cholesterol. In conclusion, lopinavir-induced lipid accumulation and affected lipid composition in L. amazonensis in a concentration–response manner. These data contribute to a better understanding of the possible mechanisms of action of this HIV-PI in L. amazonensis promastigotes. The concerted action of lopinavir on this and other cellular processes, such as the direct inhibition of an aspartyl peptidase, may be responsible for the arrested development of the parasite.

It is now generally agreed that the impact of the current nature, purpose and extent of food processing on human well-being, health and disease needs to be better understood and explained, in order to improve public health. The special issue of Public Health Nutrition devoted to the concept of ultra-processing of food, and the NOVA classification of which ultra-processed foods are one category, is a great step forward in this work. Coincidentally, a polemical ‘critical appraisal’ of ultra-processing was recently published in another journal. Debate and discussion are an essential part of the scientific endeavour. In this commentary, we correct inaccurate statements made about NOVA in the ‘appraisal,’ rebut points raised, and discuss the larger issue of scientific responsibility for publishing opposing views on controversial topics.

The species Phytomonas serpens is known to express some molecules displaying similarity to those described in trypanosomatids pathogenic to humans, such as peptidases from Trypanosoma cruzi (cruzipain) and Leishmania spp. (gp63). In this work, a population of P. serpens resistant to the calpain inhibitor MDL28170 at 70 µ m (MDLR population) was selected by culturing promastigotes in increasing concentrations of the drug. The only relevant ultrastructural difference between wild-type (WT) and MDLR promastigotes was the presence of microvesicles within the flagellar pocket of the latter. MDLR population also showed an increased reactivity to anti-cruzipain antibody as well as a higher papain-like proteolytic activity, while the expression of calpain-like molecules cross-reactive to anti-Dm-calpain (from Drosophila melanogaster) antibody and calcium-dependent cysteine peptidase activity were decreased. Gp63-like molecules also presented a diminished expression in MDLR population, which is probably correlated to the reduction in the parasite adhesion to the salivary glands of the insect vector Oncopeltus fasciatus. A lower accumulation of Rhodamine 123 was detected in MDLR cells when compared with the WT population, a phenotype that was reversed when MDLR cells were treated with cyclosporin A and verapamil. Collectively, our results may help in the understanding of the roles of calpain inhibitors in trypanosomatids.

The two central issues in China's accession to the World Trade Organization (WTO) were its economic size and the large role the state had played in economic decisions – the treatment of State-Owned Enterprises (SOEs) as central to addressing the latter point. While the Protocol of Accession imposed obligations on China that went beyond what was required of other WTO members, it has not been effective at resolving concerns about Chinese SOEs. This demonstrates, in part, the limitations in using the WTO as a vehicle to promote economic reform.

A proposal has been made to use the Tracking and Data Relay Satellite System (TDRSS) as an orbiting element for a very long baseline interferometry (VLBI) demonstration. The TDRSS is a satellite system designed to coherently track and relay data between other satellites and the central ground station. This system could also be used to coherently observe a celestial radio source. A ground-based frequency standard would be used to coherently drive the spacecraft receiver local oscillator and transmitter. The data will be telemetered to a ground station, where it will be recorded on a Mark III terminal.

An antenna in geostationary orbit was used for VLBI observations at 2.3 GHz, in combination with ground antennas in Australia and Japan. 23 of the 25 observed sources were detected on orbiter-ground baselines, with baseline lengths as large as 2.15 earth diameters. Brightness temperatures between 1012 K and 4 × 1012 K were measured for 10 sources.

Given evident multiple threats to food systems and supplies, food security, human health and welfare, the living and physical world and the biosphere, the years 2016–2025 are now designated by the UN as the Decade of Nutrition, in support of the UN Sustainable Development Goals. For these initiatives to succeed, it is necessary to know which foods contribute to health and well-being, and which are unhealthy. The present commentary outlines the NOVA system of food classification based on the nature, extent and purpose of food processing. Evidence that NOVA effectively addresses the quality of diets and their impact on all forms of malnutrition, and also the sustainability of food systems, has now accumulated in a number of countries, as shown here. A singular feature of NOVA is its identification of ultra-processed food and drink products. These are not modified foods, but formulations mostly of cheap industrial sources of dietary energy and nutrients plus additives, using a series of processes (hence ‘ultra-processed’). All together, they are energy-dense, high in unhealthy types of fat, refined starches, free sugars and salt, and poor sources of protein, dietary fibre and micronutrients. Ultra-processed products are made to be hyper-palatable and attractive, with long shelf-life, and able to be consumed anywhere, any time. Their formulation, presentation and marketing often promote overconsumption. Studies based on NOVA show that ultra-processed products now dominate the food supplies of various high-income countries and are increasingly pervasive in lower-middle- and upper-middle-income countries. The evidence so far shows that displacement of minimally processed foods and freshly prepared dishes and meals by ultra-processed products is associated with unhealthy dietary nutrient profiles and several diet-related non-communicable diseases. Ultra-processed products are also troublesome from social, cultural, economic, political and environmental points of view. We conclude that the ever-increasing production and consumption of these products is a world crisis, to be confronted, checked and reversed as part of the work of the UN Sustainable Development Goals and its Decade of Nutrition.

Cytomegalovirus (CMV) infection remains the leading cause of congenital virus infection in developed countries. Measuring the national prevalence of this infection, especially among women of childbearing age, is of great value to estimate the risk of congenital CMV infection, as well as to identify risk groups that should be targeted for behavioural interventions and/or vaccination once a CMV vaccine finally becomes available. In order to fulfil these objectives, a seroprevalence survey was conducted in 2010, using a nationally representative, population-based sample of 2536 people aged between 15 and 49 years, living in metropolitan France and attending private microbiological laboratories for blood testing. All blood samples were analysed in the same laboratory and screened for CMV-specific IgG using an enzyme-linked immunoassay technique (Elisa PKS Medac Enzyme immunoassay). The overall point estimate of CMV infection seroprevalence for individuals aged 15–49 years was 41.9%. The estimates were higher in women than in men (respectively 45.6% and 39.3%), and people born in a non-Western country were more likely to be CMV seropositive than those born in France or in another Western country (93.7% vs. 37.7%). Our results showed that a substantial percentage of women of childbearing age in France are CMV seronegative and therefore at risk of primary CMV infection during pregnancy. Educational measures and future vaccine are key issues to prevent infection in pregnant women and congenital CMV disease.

Strongyloides venezuelensis is a parasitic nematode of rodents that is frequently used to obtain heterologous antigens for immunological diagnosis of human strongyloidiasis. The aim of this study was to identify antigens from filariform larvae of S. venezuelensis for immunodiagnosis of human strongyloidiasis. Soluble and membrane fractions from filariform larvae of S. venezuelensis were obtained in phosphate saline (SS and SM) and in Tris–HCl buffer (TS and TM), and were analysed by Western blotting. Different antigenic components were recognized by IgG antibodies from the sera of strongyloidiasis patients. Highest recognition was observed for a 30–40 kDa mass range present in all antigenic fractions. The band encompassing this mass range was then excised and subjected to mass spectrometry for protein identification. Immunoreactive proteins identified in the soluble fractions corresponded to metabolic enzymes, whereas cytoskeletal proteins and galectins were more abundant in the membrane fractions. These results represent the first approach towards identification of S. venezuelensis antigens for use in immunodiagnostic assays for human strongyloidiasis.