Brain and cerebellar hemidysplasia in a case with ipsilateral body dysplasia and suspicion of CHILD syndrome

A b s t r a c t CHILD syndrome is an acronym fo

Mutations in the von Hippel-Lindau Tumour Suppressor Gene in Central Nervous System Hemangioblastomas

Central nervous system hemangioblastomas (cHAB) are rare tumours which most commonly arise in the cerebellum. Most tumours are sporadic, but as many as one third of cHABs occur in the course of the hereditary disorder - von Hippel-Lindau disease (VHL). In order to diagnose new VHL families in Poland we performed sequencing of the entire VHL gene in archival material (paraffin embedded hemangioblastoma tissues) in a large series of 203 unselected patients with cHAB. VHL gene mutations were detected in 70 (41%) of 171 tumour samples from which DNA of relatively good quality was isolated. We were able to obtain blood samples from 19 of mutation positive cases. Eight (42%) of these harboured germline mutations in persons from distinct undiagnosed VHL families

Trwałość betonu w kontekście reaktywności alkalicznej kruszyw

Reaktywność kruszyw jest ciągle aktualnym problemem, który napotykają producenci betonu najczęściej okazjonalnie, ale który może spowodować znaczne problemy z trwałością betonu. W Polsce nie ma obecnie wypracowanego modelu postępowania z kruszywem w kontekście reaktywności alkalicznej, nie ma też zweryfikowanych, pewnych metod badawczych. Nie korzysta się także w wystarczającym stopniu z doświadczeń innych krajów i organizacji zajmujących się problematyką trwałości betonu. Celem niniejszego artykułu jest pokazanie schematów postępowania w aspekcie reaktywności alkalicznej kruszyw opracowanych w ostatnich latach przez komitet RILEM. Krótko zostały opisane także problemy z reaktywnością krajowych kruszyw oraz został przedstawiony obecnie funkcjonujący w Polsce system norm wykorzystywany w diagnozowaniu reaktywności alkalia-krzemionka.The reactivity of aggregates is a problem which faced concrete producers occasionally. It can cause considerable durability problems of concrete. In Poland there are no procedures regarding the reactivity of alkali. There are no validated test methods. The aim of this article is to show patterns of conduct developed in recent years by a committee RILEM. Briefly are presented problems of reactivity of aggregates in Poland. It presents actually used standards in Poland to study the reactivity of alkali-silica. This paper shows the ways of dealing with durability of concrete according to the guidelines of the RILEM committee

Effectiveness of Concrete Reinforcement with Recycled Tyre Steel Fibres

The role of searching for industrial waste management solutions in construction is key for environmental protection. Research in recent years has focused on solutions aimed at reducing the carbon footprint. This paper presents the results of tests conducted on concrete reinforced with treated recycled tyre steel fibres (RTSFs) compared to the same amount of manufactured steel fibres (MSFs). The effectiveness of concrete reinforcement with RTSFs was analysed using the fracture mechanics parameters of cementitious composites. Rheological tests, residual flexural tensile strength tests, work of fracture measurements, toughness indices, examinations of the fibre distribution in the concrete, and SEM observations of the concrete fractures with fibres were performed. Determining the work of fracture and toughness indices was an innovative aspect of this paper. As the amount of RTSFs increased, a decrease in the consistency was observed, although the distribution of fibres in the concrete was uniform, as proven by the results of computer tomography tests. Concrete reinforced with RTSFs that is purified and refined during the recycling process might have better properties than concrete reinforced with the same amount of MSFs. The application of RTSFs in construction has environmental and economic benefits in addition to the strengthening of cementitious composites

Influence of Catalytic Additive Application on the Wood-Based Waste Combustion Process

The furniture industry is one of the most dynamically developing sectors of the Polish economy. Unfortunately, due to national law, it involves producing a significant amount of wood-based waste, which can only be incinerated in installations that meet the requirements for waste incineration or co-incineration plants. This is due to the presence of various types of chemical additives in post-production residues, which increases the emission of hazardous compounds into the atmosphere during combustion. This article presents an analysis of the impact of the use of catalytic additives on the amount of emissions produced from the combustion of wood-based waste. For this purpose, the analyzed material was pelletized by mixing it with the DESONOX catalyst or by spraying the fuel with the DESONOX + H2O solution in a 1:1 ratio. Catalytic substances were introduced into the fuel until a concentration of 0.1% of its mass was obtained. The use of catalysts has significantly reduced CO and NOX emissions into the atmosphere. In the case of carbon monoxide, the most effective was Ad2 (reduction by 44%), and in the case of nitrogen oxides, Ad1 (reduction by 31%) achieved the best outcome. The results from this analysis may be an indication for Polish legislation encouraging furniture plants to burn this waste in their own units using catalytic substances

Not Only Trees Matter—Traffic-Related PM Accumulation by Vegetation of Urban Forests

In terms of the process of air purification, a lot of attention has been devoted to trees and shrubs. Little attention has been paid to herbaceous vegetation from the lower forest layers. Urban forests are often located on the outskirts of cities and surround exit roads where there is heavy traffic, generating particulate matter (PM) pollution. The aim of this study was to investigate the spread of PM from the road traffic in the air and to investigate how individual layers of urban forests accumulate PM. We conducted comparative analyses of PM accumulation on plants in five zones away from the road, into the forest, in the air, and in four vegetation layers: mosses, herbaceous plants, shrubs and trees. The results show that all forest layers accumulate PM. We show that PM is very efficiently accumulated by herbaceous plants growing along roadsides, and that the PM that was not deposited on herbaceous plants was accumulated by trees and shrubs. With increasing distance from the road into the forest, the PM content on herbaceous plants decreased and the accumulation on trees and shrubs increased. We estimated that PM concentration in the air dropped significantly in the front line of the trees, but it was still detectable up to 50 m into the forest. The results presented herein show that meadow vegetation and urban forests play a very important role in air purification. Our results provide a better understanding of the complexity of urban forest interactions and provide the basis for better planning of urban greenery