The Challenge of Diagnosing Constitutional Mismatch Repair Deficiency Syndrome in Brain Malignancies from Young Individuals

Biallelic germline mismatch repair (MMR) gene (MLH1, MSH2, MSH6, and PMS2) mutations are an extremely rare event that causes constitutional mismatch repair deficiency (CMMRD) syndrome. CMMRD is underdiagnosed and often debuts with pediatric malignant brain tumors. A high degree of clinical awareness of the CMMRD phenotype is needed to identify new cases. Immunohistochemical (IHC) assessment of MMR protein expression and analysis of microsatellite instability (MSI) are the first tools with which to initiate the study of this syndrome in solid malignancies. MMR IHC shows a hallmark pattern with absence of staining in both neoplastic and non-neoplastic cells for the biallelic mutated gene. However, MSI often fails in brain malignancies. The aim of this report is to draw attention to the peculiar IHC profile that characterizes CMMRD syndrome and to review the difficulties in reaching an accurate diagnosis by describing the case of two siblings with biallelic MSH6 germline mutations and brain tumors. Given the difficulties involved in early diagnosis of CMMRD we propose the use of the IHC of MMR proteins in all malignant brain tumors diagnosed in individuals younger than 25 years-old to facilitate the diagnosis of CMMRD and to select those neoplasms that will benefit from immunotherapy treatment.     

Phenolic content and antioxidant activity of four date palm (Phoenix dactylifera L.) fruit varieties grown in Tunisia

Fruits of date palm ( Phoenix dactylifera L.) are consumed throughout the world and are a vital component of the diet in most Arabian countries. This study has been carried out to evaluate the total phenolic content and the antioxidant activity of four date palm fruit varieties grown in Tunisia. All measurements were made at the 'tamar' stage-the final stage of fruit ripeness. The date varieties were found to be rich in total phenolic ranging from 209.42 mg of equivalent gallic acid/100 g fresh weight in Kentichi to 447.73 mg equivalent gallic acid/100 g fresh weight in Allig. The date varieties studied were characterised by a high antioxidant activity ranging from 866.82 for the Deglet Nour to 1148.11 μmol Equivalent Trolox/100 g fresh weight for Khouet Kenta by the ABTS method. In fact, the DPPH method revealed that Allig having the lowest antioxidant activity (0.72) followed by Deglet Nour (1.53), Kentichi (1.68) and Khouet Kenta variety (1.96). This study suggest that Tunisian date palm fruit may serve as a good source of natural antioxidants and could potentially be considered as a functional food ingredient.     

Characterization of semi-solid slurry using a novel “Rheo-Characterizer” apparatus

The rheological properties of A357 semi-solid slurries, produced under different conditions using the SEED process, were analyzed with a novel “Rheo-Characterizer” apparatus. The TiB₂ grain refiner was also added to evaluate the impact on the microstructure and the cutting force. The α-Al particles and grain size were measured under different processing conditions. The effect of the solid fraction on the resulting cutting force curve was also investigated by altering the cutting temperature. The results show that the “Rheo-Characterizer” is capable of differentiating between the microstructural morphologies and the solid fraction present in the slug. A simple theoretical model was proposed to better understand the relationship between the microstructure and the cutting behaviour of the semi-solid slurry. The model, together with an analysis of the deformation phenomena observed on the wire and the slug, roughly predicts the principal characteristics of the experimental cutting curves.     

Concept Formation in WWTP by Means of Classification Techniques: A Compared Study

Although activated sludge process is a very widely used biologicalprocess in wastewater treatment plants (WWTP), and there areproperly functioning control loops such as that of dissolved oxygen,in practice, this type of plant requires a major time investment onthe part of the operator, involving many manual operations.Treatment plants work well most of the time, as long as there are not unforeseen occurrences. Normal operatingsituations (generally similar to design conditions) can be treatedmathematically by using efficient control algorithms. However, there aresituations in which the control system cannot properlymanage the plant, and in which the process can only be efficiently managedthanks to the operators experience. This is a case in which aknowledge-based system may be useful. One of the difficulties inherent tothe development of a knowledge-based system is to obtain the knowledge base(i.e., knowledge acquisition), specially whendealing with a wide, complicated and ill-structured)field.Among the aims of this work arethose to show how semi-automatic knowledge acquisition tools could helphuman experts to organize their knowledge about their domain and also, tocompare the power of different approaches of knowledge acquisition) to the same database.In this paper are presented the results obtained fromapplying two different classification techniques to the development of knowledge-bases for the management of an activated sludge process.     

Modelling the Doctor Blade-Roller Tribosystem for Improving the Cleaning Performance During Paper Production

Doctor blades are commonly used in paper machines to keep the surface of rollers clean. Due to higher demanding conditions, the requirements for doctor blades have steadily increased. The wear rates must remain low, while simultaneously their cleaning function has to be ensured. For this reason, the paper industry has developed a high degree of empirical knowledge concerning the cleaning of roller surfaces. However, up to now, no systematic approach has been successfully applied to optimize the cleaning performance of the doctor blade-roller tribosystem. This study presents an attempt to model the system based on the force equilibrium conditions at the blade tip between hydrodynamic and contact forces. The change of the blade geometry due to wear is also taken into account. By these means, a non-dimensional group involving the key parameters is obtained. This allows for a systematic improvement of the cleaning efficiency, by targeted changes of the process parameters.     

Enrichment of white flour with spices positively impacts safety and consumer acceptance of bread

Aiming at increasing the nutritional value and sensory quality of bread, we assessed the enrichment of white flour with different levels of powdered cinnamon and pomegranate peel, through rheological, nutritional, aromatic, textural and sensory analyses. These extra ingredients were chosen for their richness in bioactive compounds, and they differently affected bread quality. In relation to raw flour, introduced ingredients slightly decreased moisture and protein content, while increasing ash, fibre and radical scavenging activity, which can be attributed to the sharing of aromatic compounds by cinnamon and pomegranate peel during the bread making process. When cinnamon was incorporated at 1%, the content of all furan derivatives was reduced up to 1/4 of initial value, 2-pentyl furan totally disappeared, and furfuryl alcohol was reduced to 2.1%. Despite enriched bread’s texture was slightly worse, it was still acceptable in sensory tests, as 150 interviewed naive consumers preferred the new product over the control.     

Monitoring of the microcapsule/liposome application on textile fabrics

In recent years, new technologies have led to the production of biofunctional textiles. These biofunctional textiles contain microscopic capsules of ingredients that break as the fabric rubs the skin, releasing the active agents. Absorption and desorption behaviour of active agents embedded into the different biofunctional textiles should be taken into account when determining the amount of active agents incorporated into these textiles and when following the delivery mechanism as the fabric comes in contact with the skin. In this work, an encapsulated active agent (a sun filter, ethyl hexyl methoxycinnamate [EHMC] into microcapsules or liposomes) was applied by foulard onto different fabrics. The amount of capsules and active agents embedded into the fibres were quantified by (1) weight difference between untreated and treated fabrics, (2) extraction with isopropanol in an ultrasound bath, or (3) extraction with isopropanol/water 50/50 in a soxhlet device. Sun filter detection of the extraction baths was followed by HPLC and by UV spectrophotometry. The results show that the real amount of the EHMC present in different textile substrates depends on the way that the active agent is trapped, the ionic character of the fibres and on the vehicles used.