This report describes a patient with thalassemia intermedia-like phenotype born to normal parents in whom globin gene sequencing detected a novel abnormal hemoglobin (Hb) due to a T to A substitution at codon 60 of the beta-globin gene arising as a de novo mutation. Normal sequences were detected at the homologous beta-globin locus. This mutation results in the substitution of a polar (glutamic acid) for a nonpolar (valine) residue near the corner of the heme pocket of the beta-globin chain. The novel variant has been designated Hb Cagliari, from the place of birth of the propositus. Kinetics of globin synthesis performed following splenectomy suggest that this new Hb variant is synthesized at a near normal rate but undergoes rapid breakdown. The extreme lability of the variant explains the clinical and hematologic picture characterized by marked ineffective erythropoiesis, thalassemia-like bone changes, iron overload, high proportion of Hb F in the peripheral blood, reduced beta/alpha-globin chain synthesis ratio in peripheral blood reticulocytes, and absence of the abnormal Hb in peripheral blood at extensive protein structural analysis before splenectomy. This case indicates that a thalassemic hemoglobinopathy should be suspected in the presence of a patient with a thalassemia intermedia-like phenotype born to normal parents, even when protein structural analysis fails to detect an abnormal Hb. DNA sequencing may allow to define the mutation, thus making the proper diagnosis.
a b s t r a c tDesalinization may be a crucial component in solving the water resource development of coastal areas. The possibility to detect useful systems to correct and balance water quality needs, as necessary for the intended users, has been a fundamental target of recent desalination projects. Other sources may be locally available and can be retained as a complement to decrease the production cost and to correct the water quality parameters as per the WHO requirements. In coastal areas were groundwater of lower quality may be available, blending two or more sources are the winning solution. However, blending needs constant monitoring and continuous adjustments of proportions in the final mix. In order to meet this target, the operators need a decisional tool that enables the correct blending between different water sources. A simple but comprehensive blending tool, integrated by a hydrogeological dynamic verification tool for a better management of the overall system, was developed by Lotti Ingegneria, Italy. The blending tool, with methodological approach is reported herein, is an operational instrument aimed at calculating the chemical parameters concentration at blending reservoir level, on the basis of input and control data, by warming as well as in case of possible critical situations. The tool is based on a groundwater database spreadsheet described in terms of actual and target quality parameters and blending points (blending reservoirs) where the blending will be between desalinated water and other water sources. It was developed to be interfaced with supervisory control and data acquisition and allows easy monitoring and prompt evaluation of corrective actions as needed. Water obtained by a desalination process is extremely low in terms of salt concentration, for this reason it is necessary to pay attention to the aggressiveness and the taste of tap water. The solution to improve the flavor of tap water is blending desalinated water with available local water sources.
The study of some unpublished documents coming from several European and international libraries, after the publication of Anejos VIII, and the reconsideration of other primary sources that have already been studied, would seem to confirm most hypotheses raised in the book with a good margin of probability. The comparative analysis of these documents will allow us, as follows: a) to describe the historical background of the two letters, especially with regard to the rescriptum; b) to suggest a more precise dating of this rescriptum; and c) to outline the manuscript tradition of the two letters that, starting from the legal text in Koine Greek, are transformed into an apologetic literary text by means of interpolations that mostly affected the edict of tolerance. The preliminary study of some legal papyri, in comparison with the different versions of Ep. I, allowed us to propose a critical reconstruction of the text that has undergone numerous interpolations over the centuries. The first letter perfectly matches with both the structure and the legal terminology of the Hellenistic-Roman laws, in so fully mirroring the words used by Eusebius and Optatus to describe Maxentius' edict of tolerance. El estudio de algunos documentos inéditos procedentes de varias bibliotecas europeas e internacionales, tras la publicación de Anejos VIII, y la reconsideración de otras fuentes primarias ya estudiadas, parece confirmar la mayoría de las hipótesis planteadas en el libro con un buen margen de probabilidad. El análisis comparativo de estos documentos nos permitirá, de la siguiente manera: a) describir los antecedentes históricos de las dos epístolas, especialmente con referencia al rescriptum; b) hipotetizar una fecha más precisa de este rescriptum; y c) describir la tradición manuscrita de las dos epístolas que, a partir del texto legal en griego koiné, se transforman en un texto literario apologético mediante interpolaciones que afectan mayoritariamente el edicto de tolerancia. Además, el estudio preliminar de algunos papiros legales, en comparación con las diferentes versiones de Ep. 1, nos permitió proponer una reconstrucción crítica del texto que ha sufrido numerosas interpolaciones a lo largo de los siglos. La primera letra encaja perfectamente tanto con la estructura como con la terminología legal de las leyes helenístico-romanas, reflejando tan plenamente las palabras utilizadas por Eusebio y Optato para describir el edicto de tolerancia de Majencio.
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