Zespół padaczkowy z drgawkami połowiczymi i porażeniem połowiczym. Wyniki badania za pomocą rezonansu magnetycznego u trzyletniego chłopca

The term ‘hemiconvulsion-hemiplegia-epilepsy syndrome’ (HHE) was first used by Gastaut et al. to describe the sequential combination of unilateral or predominandy unilateral clonic seizures (hemiconvulsion), occurring during the first 2 years of life, immediately followed by an ipsilateral flaccid hemiplegia lasting 7 or more days. In the following phase partial epileptic seizures occur. We report a case of HHE syndrome in a 3-year-old boy with partial seizures (hemiconvulsion lasting 15–30 minutes) followed by left hemiplegia and hyporeflexia. Magnetic resonance imaging showed diffuse and high signal hyperintensity of the whole right cerebral hemisphere. Diffusion-weighted images showed a reduction of the apparent diffusion coefficient in the subcortical region. Magnetic resonance arteriography showed a narrow flow signal in the distal territory of the right middle cerebral artery. The authors emphasize the importance of neuroradiological findings in early diagnosis and in the follow-up of HHE syndrome.Określenia zespół padaczkowy z drgawkami połowiczymi i z porażeniem połowiczym” (hemiconvulsion-hemiplegia epilepsy – HHE) użyli po raz pierwszy Gastaut i wsp., aby opisać połączenie jednostronnych lub głównie jednostronnych drgawek klonicznych, pojawiających się w pierwszych dwóch latach życia, po których następuje tożstronne wiotkie porażenie połowicze utrzymujące się przez co najmniej tydzień. Następnie występują napady padaczkowe częściowe. W artykule przedstawiono przypadek zespołu HHE u trzyletniego chłopca z napadami częściowymi (drgawki połowicze trwające 15–30 minut), po których nastąpiło lewostronne porażenie połowicze z osłabionymi odruchami głębokimi. W badaniu za pomocą rezonansu magnetycznego uwidoczniono rozlane zmiany hiperintensywne w całej prawej półkuli mózgu. W obrazowaniu dyfuzji stwierdzono zmniejszenie współczynnika dyfuzji w obszarach podkorowych. W arteriografii rezonansu magnetycznego uwidoczniono wąski sygnał przepływu w dystalnych gałęziach prawej tętnicy środkowej mózgu. Autorzy podkreślają znaczenie wyników badań obrazowych układu nerwowego we wczesnym rozpoznaniu i obserwacji chorych na HHE

Quality Checks Logit Human Reliability (LHR): A New Model to Evaluate Human Error Probability (HEP)

In the years, several approaches for human reliability analysis (HRA) have been developed. The aim of the present research is to propose a hybrid model to evaluate Human Error Probability (HEP). The new approach is based on logit-normal distribution, Nuclear Action Reliability Assessment (NARA), and Performance Shaping Factors (PSFs) relationship. In the research, shortcomings related to literature approaches are analyzed, especially the limitations of the working time. For this reason, PSFs after 8 hours (work standard) during emergency conditions were estimated. Therefore, the correlation between the advantages of these three methodologies allows proposing a HEP analysis during accident scenarios and emergencies; a fundamental issue to ensure the safety and reliability in industrial plants is emergency Mmnagement (EM). Applying EM methodology, two main aspects are analyzed: system reliability and human reliability. System reliability is strongly related to the reliability of its weakest component. During incidental situations, the weakest parts of the whole system are workers (human reliability) and accidental scenarios influence the operator’s ability to make decisions. This article proposes a new approach called Logit Human Reliability (LHR) that considers internal and external factors to estimate human reliability during emergencies. LHR has been applied in a pharmaceutical accident scenario, considering 24 hours of working time (more than 8 working hours). The results highlighted that the LHR method gives output data more in conformity with data banks than the conventional methods during the stress phase in an accident scenario

Occurrence of Coxiella burnetii in goat and ewe unpasteurized cheeses: Screening and genotyping

Q fever is a zoonosis caused by Coxiella burnetii which infects humans as well as several animal species; sheep, goats and cattle are the primary animal reservoir. The main route of human exposure to Coxiella burnetii is inhalation of contaminated aerosols from excreta, especially birth products, while the role of unpasteurized dairy products in the transmission of Q fever to humans remains still controversial. The aim of this work was to evaluate the presence of Coxiella burnetii in unpasteurized cheese samples (n=84) by PCR and to genotype the circulating strains by Multispacer sequence typing (MST) analysis. Coxiella burnetii DNA was detected in 27/84 (32.14%) cheeses and positivity rate of handicraft cheeses reached 17.24%, while positivity rate of non-handicraft cheeses reached 65.38%. In addition, the MST profile of Coxiella burnetii detected in 5 cheese samples have shown the circulation of ST12 and ST32 genotypes in Tuscany

MATERIALS SELECTION AND DEVELOPMENT FOR CENTRIFUGAL COMPRESSORS OPERATING IN EXTREME SOUR & ACID GAS SERVICE

TutorialThe rising demand for oil and gas has led us to exploit oil fields and gas reservoirs with increasing amounts of contaminants. Reserves of natural gas are abundant, but about 40% of the fields contain high concentrations of hydrogen sulfide, carbon dioxide, and chlorides. The harshening environment represents a challenge for the suitability of common classes of materials used for design and manufacturing of centrifugal compressor components. According to NACE MR0175/ISO15156, a fit-for-purpose approach has been extensively used to prove the steel’s resistance in simulated field conditions and characterize the environmental limits of most martensitic and precipitation hardening stainless steels in terms of partial pressure of carbon dioxide, hydrogen sulfide, and chlorides. The literature available on the subject generally agrees that the contaminants’ concentration levels exceed the possibility of risk presented by stainless steels to turbo-machine reliability, and in worst cases to operators’ safety. Nickel-based alloys are the most robust solution to overcome the above limitations, guaranteeing high mechanical strength and resistance against stress corrosion cracking mechanisms in expected compressors’ environments. This paper describes the advance in nickel-based alloys’ application and production of centrifugal compressors (CC) for extreme sour and acid conditions, with work that reveals the development of alternative manufacturing routes for rotor and stator components in turbomachinery. A detailed explanation of nickel-based alloys’ metallurgy, application and manufacturing capability is also given, along with recent developments of new alloys to bridge the gap between iron-base and nickel-base alloys, providing cost effective materials with enhanced corrosion resistance

Environmental Genomics: A Tale of Two Fishes

The influence of the environment on two congeneric fishes, Gillichthys mirabilis and Gillichthys seta, that live in the Gulf of California at temperatures of 10-25 degrees C, and up to 42-44 degrees C, respectively, was addressed by analyzing their genomes. Compared with G. mirabilis, G. seta showed some striking features. Substitution rates in the mitochondrial genes were found to be extremely fast, in fact faster than in noncoding control regions (D-loops), from which a divergence time of less than 0.66-0.75 Mya could be estimated. In the nuclear genome, 1) both AT --> GC/GC --> AT and transversion: transition ratios in coding sequences (CDSs) were relatively high; moreover, the ratios of nonsynonymous/synonymous changes (Ka/Ks) suggested that some genes were under positive selection; 2) DNA methylation showed a very significant decrease; and 3) a GC-rich minisatellite underwent a 4-fold amplification in the gene-rich regions. All these observations clearly indicate that the environment (temperature and the accompanying hypoxia) can rapidly mold the nuclear as well as the mitochondrial genome. The stabilization of gene-rich regions by the amplification of the GC-rich minisatellite and by the GC increase in nuclear CDSs is of special interest because it provides a model for the formation of the GC-rich and gene-rich isochores of the genomes of mammals and birds

Defoliation, Recovery and Increasing Mortality in Italian Forests: Levels, Patterns and Possible Consequences for Forest Multifunctionality

Forest health and multifunctionality are threatened by global challenges such as climate change. Forest health is currently assessed within the pan-European ICP Forests (International Co-operative Programme on Assessment and Monitoring of Air Pollution Effects on Forests) programme through the evaluation of tree crown conditions (defoliation). This paper analyses the results of a 24-year assessment carried out in Italy on 253 permanent plots distributed across the whole forested area. The results evidenced a substantial stability of crown conditions at the national level, according to the usual defoliation thresholds Defoliation > 25% and Defoliation > 60%, albeit with species-specific patterns. Within this apparent temporal stability, an increased fraction of extremely defoliated and dead trees was observed. Extreme defoliation mostly occurred in years with severe summer drought, whereas mortality was higher in the years after the drought. The results for singular species evidenced critical conditions for Castanea sativa Mill. and Pinus species, whereas Quercus species showed a progressive decrease in defoliation. Deciduous species, such as Fagus sylvatica L., Ostrya carpinifolia Scop. and Quercus pubescens Willd. suffer the loss of leaves in dry years as a strategy to limit water loss by transpiration but recover their crown in the following years. The recurrence of extreme heat waves and drought from the beginning of the XXI century may increase the vulnerability of forests, and increased tree mortality can be expected in the future

Composizione e attività biologica di “idrolati” di agrumi

Gli idrolati, o acque aromatiche, sono prodotti naturali ricavati dalla distillazione di piante o parti di piante fresche che conservano intatte le loro proprietà e possono essere utilizzati a diverso scopo1. Essi sono comunemente considerati un sottoprodotto degli oli essenziali, ma in realtà, data la diversa composizione e la conseguente maggiore delicatezza, hanno un loro apprezzabile utilizzo, legato comunque al mantenimento delle proprietà delle piante da cui derivano. Nell’industria agrumaria gli idrolati sono il risultato della produzione degli oli essenziali attraverso spremitura a freddo delle bucce degli agrumi e sono considerati un rifiuto da smaltire. Lo studio della composizione chimica dell’idrolato è poco noto: gli idrolati sono comunque ricchi di composti prevalentemente ossigenati che oltre a conferire un gradevole profumo, possono donargli numerose proprietà biologiche. Abbiamo quindi deciso di intraprenderne uno studio di caratterizzazione LC-MS e GC-MS sull’idrolato dell’arancia di Ribera per identificare sostanze di interesse farmaceutico, cosmetico, ecc. e di possibile attività biologica. Questo potrebbe rappresentare per l’industria agrumaria un valore aggiunto nel valorizzare un prodotto attualmente di scarto. I risultati biologici preliminari hanno indicato che l’estratto organico inibisce la crescita di forme planctoniche di S. Aureus ATCC 25923 ad una concentrazione 5 mg/mL. Sono in corso ulteriori studi per valutare l’attività contro ceppi Gram-negativi e di inibizione di biofilm di Gram-positivi e Gram-negativi. Bibliografia 1 Lante, A.; Tinello, F. Innovative Food Science and Emerging Technologies, 2015, 27, 154–15