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I. INTRODUCTION Despite the broad use of glucocorticoids in inflammatory diseases (Table 1), a major question remains unanswered: why some patients with inflammatory diseases respond well to glucocorticoids while others are refractory or need chronic glucocorticoid therapy to maintain their disease in remission. Indeed, some patients are steroid-sensitive (50-75%), while others are steroid-dependent (30-35%) or resistant (10-15%). Most steroid-dependent or steroid-resistant patients develop a cushingoid appearance or metabolic dysfunction, which suggests that their poor glucocorticoid sensitivity is limited to the immune system rather than involving nonimmune tissues. Recent studies have revealed that both the process of inflammation or the disease itself and the genetic and constitutional background of the patient may influence his or her variable response to glucocorticoids [1-4].
The adrenal glands became known as gladulae renis incumbentes through the work of Bartolommeo Eustachio, nearly five centuries ago. These anatomic structures, however, did not enter the realm of clinical medicine until the middle of the 19th century, when T. Addison described the first patients with adrenal insufficiency. In the years that followed, the three zonae of the adrenal cortex were defined and several clinical and pathologic abnormalities were described. Recently, a number of adrenocortical disorders have had their pathophysiology elucidated at the physiologic and molecular levels. This chapter reviews diseases associated with adrenocortical dysfunction, whose molecular pathogenesis is either known or under investigation. These include primary glucocorticoid and corticotropin resistance (hereditary isolated glucocorticoid deficiency, triple A syndrome), adrenal hypoplasia congenita, pseudohypoaldosteronism, adrenoleukodystrophy and adrenomyeloneuropathy, the apparent mineralocorticoid excess syndrome, and Carney complex. Adrenocortical cancer is discussed separately, since in its inherited forms (Li-Fraumeni and Beckwith-Wiedemann syndromes) the molecular pathogenesis has been elucidated to some extent, whereas little is known about the molecular mechanisms leading to sporadic carcinomas. The congenital adrenal hyperplasia syndromes and Liddle syndrome and other causes of hypertension are described elsewhere.
Following the international trends, extensive research on seismic resistant structures has been carried out in Europe during the last decade, with the introduction of several systems with innovative steel‐based elements, as the result of European and national research projects. However, these systems have not claimed a fair share of the steel construction market, as provisions for their design have not been included in the Eurocodes and only a few designers are confident enough to employ them. The INNOSEIS project, which has received funding from the Research Fund for Coal and Steel (RFCS) with the participation of 11 partners, aims to deal with this shortcoming. In this paper, the valorisation actions for 12 such innovative anti‐seismic devices are presented. Information documents for all dissipative systems have been produced and combined in a single volume, translated in several European languages, for the dissemination to all partners of the construction sector such as architects, structural engineers, construction companies, steel producers and all potential decision makers of the construction sector. Criteria are proposed as to determine which of the systems are characterised as devices and are subject to CE marking in accordance with EN 15129, and which may be considered as innovative systems that require a code approval in EN 1998–1. For the latter, pre‐normative design recommendations are drafted that will allow them to receive the status of code‐approved systems. A reliability‐based methodological procedure to define values of behaviour factors (q‐factors) for building structures is proposed, which will be in turn applied to determine q‐factors for structural systems with the anticipated systems. A number of case studies with application examples of realistic steel buildings, in which the systems are employed, are presented. Dissemination of the project includes seminars and workshops in several European and Mediterranean countries, as well as the development of online, printed and electronic material, which is free for all people involved in the construction sector, in order to achieve the wide application of innovative seismic resistance systems in practical design.
Since their release in 2012, the FEMA P-58 guidelines for seismic performance assessment of buildings have been regarded as the state-of-the-art paradigm for buildingspecific risk assessment and loss estimation.The latter is carried out through a rigorous component-by-component procedure that requires a complete component inventory, along with fragility and repair cost information.A fully compatible story-based approach is investigated herein as a simplified alternative that can potentially reduce the required input data with only a minor drop in accuracy.As an example, a set of story loss functions relating story repair cost with story-level engineering demand parameters are derived for standard inventory makeups of low/midrise steel office buildings.Preliminary results for a 4-story steel building show a promising balance between accuracy and simplicity.
