Cryopyrin-associated periodic syndromes and autoinflammation
ABSTRACT Autoinflammatory syndromes are a distinct class of inherited diseases of cytokine dysregulation with important cutaneous features. Several disorders, including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome and neonatal onset multisystem inflammatory disorder (NOMID), are associated with mutations in a common gene, CIAS-1. These disorders are now believed to represent related conditions along a spectrum of disease severity, in which FCAS is the mildest and NOMID is the most severe phenotype. Patients typically present with lifelong atypical urticaria with systemic symptoms, with potential for developing end-organ damage due to chronic inflammation. Advances in the understanding of the genetic basis of these syndromes have also revealed cytokine signalling molecules that are critical to normal regulation of inflammatory pathways. The dramatic response of these syndromes to anakinra, an interleukin (IL)-1 antagonist, highlights the important role of IL-1 cytokine signalling in the pathogenesis of this rare but fascinating class of diseases.
Article: Monogenic Autoinflammatory Syndromes[Show abstract] [Hide abstract]
ABSTRACT: “Autoinflammatory syndromes” is the broad name given to a group of heritable conditions that initially consisted of a group of diseases termed hereditary periodic fever syndromes. Autoinflammatory syndrome is the preferred name for these conditions, because not all of the disorders that have been added to this class of inflammatory disorde rs present with periodic febrile episodes. In contrast to autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis, autoanti-bodies and antigen-specific T cells do not play a role in the pathogenesis of the autoinflammatory syndromes. Although the term autoinflammatory syndrome is now commonly used to include polygenic diseases such as Behcet's syndrome, Still's disease, and even Type 2 diabetes, this chapter addresses the monogenic autoin-flammatory syndromes. These syndromes demonstrate a Mendelian inheritance pattern, and their genetic causes have been identified. The disorders typically present in childhood. The disorders are divided into different clinical syndromes that include mixtures of clinical manifestations in the joints, the bones, the skin, the abdomen, and the lymph nodes.
Article: You are not expected to read all, this would be impossible: it is just a suggestion for updating your knowledge or satisfying your curiosity. If you feel a lack of knowledge, some of these publications may help. As an allergologists/immunologist, you are expected to have a basic understanding of immunology and its implication in the daily clinical practice
Conference Paper: Sensor frequency allocation issues for WRC-2000[Show abstract] [Hide abstract]
ABSTRACT: The 2000 World Radiocommunication Conference (WRC-2000), which was to be held in Istanbul, Turkey in May of 2000, has two major topics that concern passive sensing on its agenda. Agenda item 1.17 deals with the possible upgrade to a worldwide primary allocation for the Earth exploration satellite (passive) service in the 18.6-18.8 GHz band. This band is an essential component to the suite of frequencies used for global observation of the Earth's environment. Agenda item 1.16 deals with passive allocations above 71 GHz and encompasses a number of important passive sensing bands such as 86-92 GHz, 100-102 GHz, around 118 GHz, around 150 and 167 GHz, around 183 GHz, 200-209 GHz, 226-238 GHz, and around 250 GHz. Agenda item 1.4 deals with the use of high density applications of the fixed service (HDFS) above 30 GHz, and this may affect passive sensors using channels in the band 55.78-56.26 GHz. Limits on the HDFS in the form of a power density limit, an emission mask, or both are likely needed to ensure the protection of this important band for atmospheric measurements used in numerical weather prediction models. These items are discussed in the context of the Conference Preparatory Meeting process. The results of the actual World Radiocommunication Conference are also presentedGeoscience and Remote Sensing Symposium, 2000. Proceedings. IGARSS 2000. IEEE 2000 International; 02/2000