French national protocol for the management of congenital ichthyosis.

Congenital ichthyoses (CI) comprise a heterogeneous group of monogenic genetic skin diseases characterized by diffuse scaling, often associated with skin inflammation. Diagnosis of the individual form of ichthyosis is complex and is guided by clinical expertise. CI usually has a major impact on quality of life (QOL) and thus requires lifelong treatment. To date, there are no curative therapies, although various symptomatic treatment options exist. The present protocol for the management of CI has been drawn up in accordance with the recommendations published in 2012 by the French National Authority for Health, based on a literature review, with the help and validation of members of the French network for rare skin diseases (FIMARAD). It provides a summary of evidence and expert-based recommendations and is intended to help clinicians with the management of these rare and often complex diseases.

An engineered PET depolymerase to break down and recycle plastic bottles.

Present estimates suggest that of the 359 million tons of plastics produced annually worldwide1, 150-200 million tons accumulate in landfill or in the natural environment2. Poly(ethylene terephthalate) (PET) is the most abundant polyester plastic, with almost 70 million tons manufactured annually worldwide for use in textiles and packaging3. The main recycling process for PET, via thermomechanical means, results in a loss of mechanical properties4. Consequently, de novo synthesis is preferred and PET waste continues to accumulate. With a high ratio of aromatic terephthalate units-which reduce chain mobility-PET is a polyester that is extremely difficult to hydrolyse5. Several PET hydrolase enzymes have been reported, but show limited productivity6,7. Here we describe an improved PET hydrolase that ultimately achieves, over 10 hours, a minimum of 90 per cent PET depolymerization into monomers, with a productivity of 16.7 grams of terephthalate per litre per hour (200 grams per kilogram of PET suspension, with an enzyme concentration of 3 milligrams per gram of PET). This highly efficient, optimized enzyme outperforms all PET hydrolases reported so far, including an enzyme8,9 from the bacterium Ideonella sakaiensis strain 201-F6 (even assisted by a secondary enzyme10) and related improved variants11-14 that have attracted recent interest. We also show that biologically recycled PET exhibiting the same properties as petrochemical PET can be produced from enzymatically depolymerized PET waste, before being processed into bottles, thereby contributing towards the concept of a circular PET economy.

Heavy metals mobility in harbour contaminated sediments: the case of Port-en-Bessin.

Metallic contaminants associated with sediments showed various behaviours depending on physicochemical conditions. A contaminated sediment core from a harbour in the Bay of Seine was sampled to derive information about metal solubilization from anoxic sediment. In these anaerobic surroundings, physicochemical processes depended on the organic matter cycle, on vertical variation of redox conditions and on precipitation conditions of iron and manganese. In the studied core, anoxic conditions were developed at -15 cm depth. A three-step sequential extraction procedure, modified from the BCR method (now the SM&T), was applied to the anoxic sediment in order to evaluate the potential mobility of fixed metals. Zinc was the most labile metal, recovered in the first extraction stages, and was associated with the non-residual fraction of sediment. Lead was the least labile metal, with up to 70% associated with the residual fraction of the sediment. Copper was associated with organic matter, and its mobility was controlled by the concentration and degradation of the organic fraction. Discharge of organic-rich dredged sediments at sea results in degradation of contained organic matter and may affect the environmental impact of these metals significantly. These results therefore help to improve the waste management of such contaminated sediments.

Metals in sediments and the edible cockle Cerastoderma edule from two Moroccan Atlantic lagoons: Moulay Bou Selham and Sidi Moussa.

Metals (Cd, Cu, Pb and Zn) were determined over a 3-year period in sediments and bivales Cerastoderma edule from two lagoon ecosystems on the Moroccan Atlantic coastline, Moulay Bou Selham and Sidi Moussa. Sediment metal concentrations were seasonally influenced, and were highest, generally, in winter--possibly signifying an increased contribution from run-off from adjacent arable land and roads. Neither site can be considered highly contaminated; however, Cd was enriched at Sidi Moussa, probably as a result of waste discharge from phosphate industries 20 km along the coast to the northeast. This ability to act as a sink for metals at distance from sources demonstrates the need for surveillance in these sensitive environments. Copper and Zn concentrations in edible cockles C. edule appear to be regulated over the concentration ranges currently found in lagoon sediments. In contrast, body burdens of Pb and, to a lesser extent Cd, reflect sediment levels and might be useful in future risk assessments. Metal concentrations Cerastoderma edule are seasonally related to the reproductive cycle, however, and sample timing will need to be standardised in future monitoring exercises.