Bone dysplasia with optic atrophy, vascular malformation and seizures in a 14-year-old girl : a case report

Background: The heritable skeletal dysplasias or osteochondrodysplasias are a large heterogeneous group of disorders associated with abnormal shape, growth, or integrity of bones. Osteopetrosis is a collective term for a range of sclerosing bone diseases with various degree of defective remodeling. Increased bone density is the predominant radiologic feature. The skull is often involved with basal sclerosis and the sinuses are obliterated. The most serious consequences of the osteopetroses are seen in the nervous system. Because of perturbed remodeling of the skull bones, many aspects of the brain and cranial nerve function are endangered. Cranial nerves, blood vessels and the spinal cord may be compressed by progressive occlusion of cranial foramina. Carious, misplaced teeth, dysplastic fingernails, tendency to pathologic fractures are the other clinical manifestations. Case report: The authors present a 14-year-old girl with dysmorphic features, optic atrophy, CNS vessel malformation, pathologic fractures and seizures. The girl had a wide range of clinical and radiographic symptoms of bone dysplasias together with a giant left internal carotid artery aneurysm and epilepsy. Conclusions: On the basis of clinical and radiological features, a disease belonging to the group of skeletal dysplasias was recognized in our patient. The configuration of the presented symptoms does not allow at the moment strict classification to hitherto determined forms of dysplasia. This leads to the necessity of extending diagnostics, especially by molecular tests, and further long-lasting observations, which perhaps would allow classification of the presented syndrome to one of the known groups, or determination of a new clinical entity

Mikrostruktura wygrzewanych pokryć cynkowych typu galfan na podłożu stalowym

The commercially available Galfan coating containing 5-7wt.% of Al deposited on the low carbon steel substrate by hot dipping has been examined with respect to the microstructure of the coating/substrate interface area. The application of several experimental techniques (SEM/EDS, XRD, TEM/AEM/EDS/ED) allowed demonstrating the two-phase structure of the alloy coating in non-treated, commercially available Galfan samples: Zn-rich pre-eutectoid phase grains are surrounded by lamellar eutectics of -Al and -Zn. The transition layer between the alloy coating and steel substrate with the considerably higher Al content (SEM/EDS, TEM/EDS) has been found in both non-treated and annealed samples (600oC/5 minutes). Only the monoclinic FeAl3Znx phase however was revealed in the annealed sample (TEM/electron diffraction) remaining uncertain the presence of the orthorhombic Fe2Al5Znx phase, reported by several authors

Use of Humour in an Academic B-learning ESP Course

The subject of the study presented in this chapter concerns ESP presentations delivered by BA students of humanities which include examples of intentional high brow humour based on semantic incongruity and the use of poetic imagery. The skill of creating humorous messages is perceived in the context of the ability to think critically, as perceived by Don and Alleen Nilsen in the Linguistic Humor presentation. The appropriateness of their use is delineated by the boundaries set by Ronald A. Berk in Professors are from Mars and students are from snickers and Giselinde Kuipers in Humor and social boundaries. In principle, the humour used in presentations does not aim at entertainment. It helps student presenters strive to create and deliver original content according to course principles in order to 1. deepen their own and the listener’s understanding of the subject discussed, 2. involve them in individual and group language practice in class and on the e-learning platform. Action research carried out during classes included the collection of statistical data concerning evaluation of the presentation and of its delivery, and of the use of humour as well as the obtaining of additional information from students’ comments. Detailed analysis of these data shows that the ability to create and purposefully use humorous material is not connected with students’ language competence but rather with their ability to think critically and willingness to expend intellectual effort

The usefulness of folklore texts in the training of future teachers of Italian

This paper refers to the workshop “Laboratorio ludico” that took place at the Teachers Training College in Gliwice. Its aim was to help students, future teachers of Italian language, discover the rules of effective recreational teaching. The article is divided into three chapters. Chapter one analyses the structure and the contents of the course. Chapter two describes opportunities and cultural exploration found in the chosen examples of childrens’ folklore stories. Chapter three evaluates their impact on the perception of the Italian culture by the students and the usefulness of the project in the students’ learning process