École normale supérieure de Cachan

About: École normale supérieure de Cachan is a education organization based out in Cachan, Île-de-France, France. It is known for research contribution in the topics: Decidability & Nonlinear system. The organization has 2717 authors who have published 5585 publications receiving 175925 citations.

Tool path deformation in 5-axis flank milling using envelope surface

Abstract: This paper deals with assessment and correction of tool path in 5-axis machining. The tool trajectory is described using two curves; each one corresponding to the trajectory of two particular points of the tool axis. The assessment of the tool path is performed via the calculation of the envelope surface, which is calculated using a kinematics approach. The geometrical deviations between the envelope surface and the nominal surface are calculated. When necessary, the tool path is corrected, so that the envelope surface fits the ideal surface as much as possible. This correction is carried out by the deformation of both curves that are representative of the tool trajectory. We choose to illustrate our purpose through flank milling of sculptured surfaces.

Pathogenesis, classification and treatment of mastocytosis: state of the art in 2010 and future perspectives.

Abstract: Mastocytosis is a myeloid neoplasm characterized by abnormal accumulation and frequent activation of mast cells (MCs) in various organs. Organ systems typically involved are the bone marrow, skin, liver and gastrointestinal tract. In most adult patients, the systemic form of mastocytosis (SM) is diagnosed, which includes an indolent subvariant, an aggressive subvariant and a leukemic subvariant, also termed MC leukemia. Whereas in pediatric mastocytosis, which is usually confined to the skin, a number of different KIT mutations and other defects may be detected, the KIT mutation D816V is detectable in most (adult) patients with SM. In a subset of these patients, additional oncogenic factors may lead to enhanced survival and growth of MCs and, thus, to advanced SM. Other factors may lead to MC activation, with consecutive anaphylactic reactions that can be severe or even fatal. Treatment of SM usually focuses on symptom relief by histamine receptor antagonists and other supportive therapy. However, in aggressive and leukemic variants, cytoreductive and targeted drugs must be applied. Unfortunately, the prognosis in these patients remains poor, even when treated with novel KIT- targeting agents, polychemotherapy or stem cell transplantation. This article provides a summary of our knowledge on the pathogenesis and on treatment options in SM.