To date, over 40 different mutations in transthyretin (TTR) have been associated with amyloid deposition. The major unresolved problem is the correlation between the clinical heterogeneity and the genetic heterogeneity. For instance, whereas some mutations produce neuropathy and some give rise to cardiomyopathy, others produce vitreous opacities, the vast majority being neuropathic. Moreover, some mutations are not amyloidogenic but are responsible to hyperthyroxinemias (by virtue of the protein function in thyroid transport), whereas others are apparently nonpathogenic. The study of TTR variants is very important to the understanding of the amyloid formation process and to establish a relationship between the structure and function of the molecule. The results of current TTR mutation screening programs and their characterization are summarized.

Introduction: Chronic obstructive pulmonary disease (COPD) is a major and increasing global health problem with enormous amount of expenditure of direct/indirect health-care costs [1]. Quality of life (QOL) is an important domain for measuring the impact of chronic disease. The importance of measurement of quality of life (QOL) in COPD subjects is because no single measurement of lung function can satisfactorily identify the various disturbances that may cause S220 ABSTRACTS

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Hydro generators installed in Itaipu Binacional power plant with 824/737 MVA rated output power (50/60 Hz) belong to the largest ones in the world. Among many unique features the generators are equipped with the largest hydrodynamic thrust bearings ever built (external diameter 5200 mm, axial load equals approximately 3600 t). This paper is an attempt to propose a new thrust bearing design with the use of the stateof-the-art technologies and simulation techniques that demonstrate a reduction of friction power losses generated by the thrust bearing. This paper is divided into two parts. Within the first one the original thrust bearing design which was implemented in the generators is described. Related calculation results based on a thermo-elastohydrodynamic (TEHD) calculation software used by Alstom will be presented. A comparison between measurement results gathered in the 1980's is given. In the second part a potential solution of a more beneficial bearing design is described. The proposed thrust bearing design modification is an implementation of Alstom's Polypad TM coating. This modern polymer (PEEK) coating material has already been used by Alstom in projects around the world for many years. This coating allows pushing the operating parameters limits toward higher temperatures and lower oil film thicknesses far beyond the limits known for the conventional bearing materials.