Measurement of 7-dehydrocholesterol and cholesterol in hair can be used in the diagnosis of Smith-Lemli-Opitz syndrome

7-dehydrocholesterol (7-DHC) and cholesterol (CHOL) are biomarkers of Smith-Lemli-Opitz Syndrome (SLOS), a congenital autosomal recessive disorder characterized by elevated 7-DHC level in patients. Hair samples have been shown to have great diagnostic and research value, which has long been neglected in the SLOS field. In this study, we sought to investigate the feasibility of using hair for SLOS diagnosis. In the presence of antioxidants (2,6-ditert-butyl-4-methylphenol and triphenylphosphine), hair samples were completely pulverized and extracted by micro-pulverized extraction in alkaline solution or in n-hexane. After microwave-assisted derivatization with N,O-Bis(trimethylsilyl)trifluoroacetamide, the analytes were measured by GC-MS. We found that the limits of determination for 7-DHC and CHOL were 10 ng/mg and 8 ng/mg, respectively. In addition, good linearity was obtained in the range of 50–4000 ng/mg and 30–6000 ng/mg for 7-DHC and CHOL, respectively, which fully meets the requirement for SLOS diagnosis and related research. Finally, by applying the proposed method to real hair samples collected from 14 healthy infants and two suspected SLOS patients, we confirmed the feasibility of hair analysis as a diagnostic tool for SLOS. In conclusion, we present an optimized and validated analytical method for the simultaneous determination of two SLOS biomarkers using human hair.     

The International Atomic Energy Agency action plan on radiation protection of patients and staff in interventional procedures: Achieving change in practice

IntroductionThe International Atomic Energy Agency (IAEA) organized the 3rd international conference on radiation protection (RP) of patients in December 2017. This paper presents the conclusions on the interventional procedures (IP) session.Material and methodsThe IAEA conference was conducted as a series of plenary sessions followed by various thematic sessions. “Radiation protection of patients and staff in interventional procedures” session keynote speakers presented information on: 1) Risk management of skin injuries, 2) Occupational radiation risks and 3) RP for paediatric patients. Then, a summary of the session-related papers was presented by a rapporteur, followed by an open question-and-answer discussion.ResultsSixty-seven percent (67%) of papers came from Europe. Forty-four percent (44%) were patient studies, 44% were occupational and 12% were combined studies. Occupational studies were mostly on eye lens dosimetry. The rest were on scattered radiation measurements and dose tracking. The majority of patient studies related to patient exposure with only one study on paediatric patients. Automatic patient dose reporting is considered as a first step for dose optimization. Despite efforts, paediatric IP radiation dose data are still scarce. The keynote speakers outlined recent achievements but also challenges in the field. Forecasting technology, task-specific targeted education from educators familiar with the clinical situation, more accurate estimation of lens doses and improved identification of high-risk professional groups are some of the areas they focused on.ConclusionsManufacturers play an important role in making patients safer. Low dose technologies are still expensive and manufacturers should make these affordable in less resourced countries. Automatic patient dose reporting and real-time skin dose map are important for dose optimization. Clinical audit and better QA processes together with more studies on the impact of lens opacities in clinical practice and on paediatric patients are needed.     

How does the cumulative impacts approach support Maritime Spatial Planning?

Maritime Spatial Planning (MSP) needs to incorporate spatial information on human impacts. As human activities and uses increase in marine and coastal waters around the world, pressures in ecosystems are also increasing, leading to multiple adverse effects on different species and habitats. The European Directive on MSP aims to achieve an integrated approach to marine governance, whilst securing and maintaining the healthy status of marine and coastal waters, in accordance with the Marine Strategy Framework Directive. The latter requires Member States to develop assessments not only on pressures and impacts, but also on the state of the marine environment and then take measures towards reaching a Good Environmental Status by 2020.The Portuguese Maritime Spatial Plan – Plano de Ordenamento do Espaço Marítimo (POEM) was developed between 2009 and 2012. In 2014 a law establishing the Basis for the Spatial Planning and Management of the National Maritime Space was enacted and in 2015 the framework for the elaboration of a new national Maritime Spatial Plan, named Situation Plan, was established. Portugal will face, in the next five years, the challenge of planning and managing its marine space, whilst promoting its sustainable use and protection.This study adapted a cumulative effects assessment model to understand how the impacts from multiple threats affect the marine and coastal ecosystems and, how this information can be used to improve the management process. Information was gathered on intensity and distribution of activities and uses for the Portuguese continental subdivision marine area, quantified and mapped their cumulative impacts in marine ecosystems, and overlapped with the POEM. Results show that impacts are spreading from the coast up to the Contiguous Zone. Higher scores appear in Transitional and Coastal Waters in the north (Viana do Castelo/Figueira da Foz), centre (Peniche/Setúbal) and south (Lagos/Faro). In some areas with higher ranks, statutes of nature conservation are already in place, but potential activities may still occur on top of existing ones. This study shows that the adapted model is a helpful tool to clarify ocean planning, identify areas of potential conflicts among users and support the decision making process.     

Loss of second and sixth conserved cysteine residues from trypsin inhibitor-like cysteine-rich domain-type protease inhibitors in Bombyx mori may induce activity against microbial proteases

Previous studies have indicated that most trypsin inhibitor-like cysteine-rich domain (TIL)-type protease inhibitors, which contain a single TIL domain with ten conserved cysteines, inhibit cathepsin, trypsin, chymotrypsin, or elastase. Our recent findings suggest that Cys^2nd and Cys^6th were lost from the TIL domain of the fungal-resistance factors in Bombyx mori, BmSPI38 and BmSPI39, which inhibit microbial proteases and the germination of Beauveria bassiana conidia. To reveal the significance of these two missing cysteines in relation to the structure and function of TIL-type protease inhibitors in B. mori, cysteines were introduced at these two positions (D36 and L56 in BmSPI38, D38 and L58 in BmSPI39) by site-directed mutagenesis. The homology structure model of TIL domain of the wild-type and mutated form of BmSPI39 showed that two cysteine mutations may cause incorrect disulfide bond formation of B. mori TIL-type protease inhibitors. The results of Far-UV circular dichroism (CD) spectra indicated that both the wild-type and mutated form of BmSPI39 harbored predominantly random coil structures, and had slightly different secondary structure compositions. SDS-PAGE and Western blotting analysis showed that cysteine mutations affected the multimerization states and electrophoretic mobility of BmSPI38 and BmSPI39. Activity staining and protease inhibition assays showed that the introduction of cysteine mutations dramaticly reduced the activity of inhibitors against microbial proteases, such as subtilisin A from Bacillus licheniformis, protease K from Engyodontium album, protease from Aspergillus melleus. We also systematically analyzed the key residue sites, which may greatly influence the specificity and potency of TIL-type protease inhibitors. We found that the two missing cysteines in B. mori TIL-type protease inhibitors might be crucial for their inhibitory activities against microbial proteases. The genetic engineering of TIL-type protease inhibitors may be applied in both health care and agricultural industries, and could lead to new methods for breeding fungus-resistant transgenic crops and antifungal transgenic silkworm strains.     

Impact of the Arundo scale Rhizaspidiotus donacis (Hemiptera: Diaspididae) on the weight of Arundo donax (Poaceae: Arundinoideae) rhizomes in Languedoc southern France and Mediterranean Spain

Arundo donax L. (Poaceae) is native to Mediterranean Europe and invasive in the Rio Grande Basin of North America. Rhizomes from nine sites in France and Spain infested with a candidate control agent, the armoured scale Rhizaspidiotus donacis (Hemiptera: Diaspididae) weighed 50% less than those from nine sites without scale.     

Focal contacts of spreading platelets with the substratum

Contacts with glass substratum formed by the spreading rabbit platelets were examined by an antibody-exclusion method; monoclonal antibodies against 80 kD bovine serum protein were used. It was found that platelets form focal contacts in the course of spreading. The size of the largest focal contacts formed by platelets is smaller than that of the contacts formed by fibroblasts. The antibody-exclusion method revealed focal contacts of platelets much more clearly than interference reflection microscopy (IRM). The similarity of reactions involved in spreading platelets and of large nucleus-containing tissue cells is discussed.     

Does re-creation of extensive pasture-forest mosaics provide an economically sustainable way of nature conservation in Sweden''s forest dominated regions?

There is a major risk that many of the remaining semi-natural pastures in Swedish forest dominated regions will lose their grazing in the near future with lost biodiversity as a result. The reason is the high costs of grazing small pastures with cattle from generally small herds. The approaching decoupling of the present EU income support per head of cattle will increase the risk. Calculations based on economies of scale in beef production and opportunity cost of forest and arable land suggest that re-creating extensive pasture-forest mosaics consisting of existing semi-natural pastures and adjacent arable fields and forests can secure economically sustainable grazing. The risk of local extinction of grassland species due to habitat isolation is also lower in large mosaics than in small, scattered pastures.     

Incidence,prevalence, mortality,disability-adjusted life years and risk factors of cancer in Australia and comparison with OECD countries, 1990–2015: findings from the Global Burden of Disease Study 2015

BackgroundComparative evidence on the burden, trend, and risk factors of cancer is limited. Using data from the Global Burden of Disease (GBD) study, we aimed to assess cancer burden – incidence, prevalence, mortality, disability-adjusted life years (DALYs) – and attributable risk factors for Australia between 1990 and 2015, and to compare them with those of 34 members of the Organisation for Economic Co-operation and Development (OECD).MethodsThe general GBD cancer estimation methods were used with data input from vital registration systems and cancer registries. A comparative risk assessment approach was used to estimate the population-attributable fractions due to risk factors.ResultsIn 2015 there were 198,880 (95% uncertainty interval [UI]: 183,908–217,365) estimated incident cancer cases and 47,562 (95% UI: 46,061–49,004) cancer deaths in Australia. Twenty-nine percent (95% UI: 28.2–29.8) of total deaths and 17.0% (95% UI: 15.0–19.1) of DALYs were caused by cancer in Australia in 2015. Cancers of the trachea, bronchus and lung, colon and rectum, and prostate were the most common causes of cancer deaths. Thirty-six percent (95% UI: 33.1–37.9) of all cancer deaths were attributable to behavioral risks. The age-standardized cancer incidence rate (ASIR) increased between 1990 and 2015, while the age-standardized cancer death rate (ASDR) decreased over the same period. In 2015, compared to 34 other OECD countries Australia ranked first (highest) and 24^th based on ASIR and ASDR, respectively.ConclusionThe incidence of cancer has increased over 25 years, and behavioral risks are responsible for a large proportion of cancer deaths. Scaling up of prevention (using strategies targeting cancer risk factors), early detection, and treatment of cancer is required to effectively address this growing health challenge.