HB Setif [A94(Gi)Asp+Tyr] in Malta

Since testing of newborn infants for abnormal hemoglobins (Hbs) was reinitiated in Malta in 1989, about 20,000 cord blood samples have been studied with an isoelectrofocusing (IEF) technique (1). Among these, 2% have an abnormal Hb, i.e. 1.8% have a y-globin variant, and 0.2% have an a-globin variant (2). P-Globin variants have been noted only sporadically. In a further survey for hemoglobinopathies in elderly members of the Maltese population, the presence of two a-globin variants that differed in amount and electrophoretic mobility, was noted (Fig. 1). One had a pl and peptide map consistent with that of Hb St. Luke’s [a95(G2)Pro+Arg] which occurs at levels of around 10% of total Hb in heterozygotes from Malta (3,4). The other had a slower electrophoretic mobility and was more abundant in the red cell lysate. It was present in the proband, a healthy 88-year-old male, and in his healthy 91 -year-old sister.peer-reviewe

A Geomatic Approach to the Preservation and 3D Communication of Urban Cultural Heritage for the History of the City: The Journey of Napoleon in Venice

The use of historical maps in a digital environment can give considerable support to the study of the history of cities. It allows you to combine information from different sources, processed according to different geomatic techniques, to provide a reconstruction of urban configurations of the past and their comparison with iconographic and textual documentation of the same period. The aim of the research is to try to make the knowledge of a historical event easily accessible by converging within a simple model the various sources on which the reconstruction itself is based. This paper deals with the reconstruction of the ephemeral architecture created for Napoleon’s visit to Venice through the generation of 3D virtual models. The reconstruction was approached through a rigorous method, inserting these models into the context for which they were conceived. The generation of the historical city model, taking advantage of the algorithms of structure from motion applied to photogrammetry, made it possible to compare it with what was shown by the old paintings depicting the event. Virtual models processed within the GIS environment have been uploaded online thanks to the use of WebGIS. We chose to share the research results on the internet to allow users to avail themselves of a space that no longer exists from within it, going beyond the pictorial images of the past, overcoming communication through rendering and videos. The simultaneous application of methods and techniques related to the various components of geomatics within the digital environment has enabled the operation of a faithful reconstruction of reality, bringing to light past urban scenarios that no longer exist and are only known through paintings

Formulation of Green Particulate Composites from PLA and PBS Matrix and Wastes Deriving from the Coffee Production

Particulate composites based on poly(lactic acid) and poly(butylene succinate) biopolymers have been formulated. Silver skin, the by-product derived from the roasting of coffee beans, has been used as a filler up to a 30 wt% of loading. The microstructure, crystallinity, thermal stability, mechanical properties and water absorption of the derived composites have been investigated. Data so far collected underline that a trade-off of the mechanical properties can be obtained by adding the filler, while the overall amount of crystallinity remains constant. Up to the highest filler content, moisture uptake follows a Fichian behaviour while the value of the contact angle is slightly increased by modification. Thus, silver skin, which actually is used for fuel or soil fertilization, finds here a different environmentally friendly valorization into the field of biocomposites

Mutation pattern in the Bruton's tyrosine kinase gene in 26 unrelated patients with X linked Agammaglobulinemia

Mutation pattern was characterized in the Bruton's tyrosine kinase gene (BTK) in 26 patients with X-linked agammaglobulinemia, the first described immunoglobulin deficiency, and was related to BTK expression. A total of 24 different mutations were identified. Most BTK mutations were found to result in premature termination of the translation product. Mutations were detected in most BTK exons with a predominance of frameshift and nonsense mutations in the 5' end of the gene and missense mutations in its 3' part, corresponding to the catalytic domain of the enzyme. Nonsense and frameshift mutations were associated with diminished levels of BTK mRNA expression, except for a frameshift mutation in exon 17 and two nonsense mutations in exon 2, indicating that these cases are not confined to penultimate exons. One amino acid substitution (R28H) was found in the pleckstrin homology domain's residue, which is mutated in mice bearing the X-linked immunodeficiency phenotype; another substitution (R307G) was identified in the src homology domain 2. All remaining amino acid substitutions were found in the catalytic domain of Bt