Glypican-3–Deficient Mice Exhibit Developmental Overgrowth and Some of the Abnormalities Typical of Simpson-Golabi-Behmel Syndrome

Glypicans are a family of heparan sulfate proteoglycans that are linked to the cell surface through a glycosyl–phosphatidylinositol anchor. One member of this family, glypican-3 (Gpc3), is mutated in patients with the Simpson-Golabi-Behmel syndrome (SGBS). These patients display pre- and postnatal overgrowth, and a varying range of dysmorphisms. The clinical features of SGBS are very similar to the more extensively studied Beckwith-Wiedemann syndrome (BWS). Since BWS has been associated with biallelic expression of insulin-like growth factor II (IGF-II), it has been proposed that GPC3 is a negative regulator of IGF-II. However, there is still no biochemical evidence indicating that GPC3 plays such a role

Pseudoacromegaly

© 2018 Elsevier Inc. Individuals with acromegaloid physical appearance or tall stature may be referred to endocrinologists to exclude growth hormone (GH) excess. While some of these subjects could be healthy individuals with normal variants of growth or physical traits, others will have acromegaly or pituitary gigantism, which are, in general, straightforward diagnoses upon assessment of the GH/IGF-1 axis. However, some patients with physical features resembling acromegaly – usually affecting the face and extremities –, or gigantism – accelerated growth/tall stature – will have no abnormalities in the GH axis. This scenario is termed pseudoacromegaly, and its correct diagnosis can be challenging due to the rarity and variability of these conditions, as well as due to significant overlap in their characteristics. In this review we aim to provide a comprehensive overview of pseudoacromegaly conditions, highlighting their similarities and differences with acromegaly and pituitary gigantism, to aid physicians with the diagnosis of patients with pseudoacromegaly.PM is supported by a clinical fellowship by Barts and the London Charity. Our studies on pituitary adenomas and related conditions received support from the Medical Research Council, Rosetrees Trust and the Wellcome Trust

[DEMO] MRI Design Review System

Experience and control your design using natural interfaces! Most of todays conventional design review systems require special programming skills for preparation and high-capacity hard- and software for demonstration. Interacting with 3D data sometimes can be complicated. Today we face five major problem fields using design review systems: Interaction with 3D data, navigation in 3D space, controlling design alternatives, design presentation using less extensive hardware, content development without special software and programming skills

Intelligent decision support system to optimize, manage and plan water quality monitoring programs based on a participative approach

Tableau d'honneur de la Faculté des études supérieures et postdoctorales, 2017-2018Cette thèse s’intéresse au développement d’un système intelligent d’aide à la décision (SIAD) destiné à la conception, à la gestion et à l’optimisation des programmes de suivi de la qualité de l’eau (PSQE). Toute son originalité repose sur le fait qu’elle aborde la question dans une perspective holistique qui se traduit par le développement d’une approche participative dans le but de déterminer les besoins en connaissances sur la qualité et la quantité de l’eau sur lesquels se base le SIAD pour assister les gestionnaires de PSQE à toutes les étapes de la planification, de la gestion et de l’optimisation d’un PSQE. ...This thesis focuses on the development of an intelligent decision-support system (IDSS) to plan, manage and optimize water quality monitoring programs (WQMPs). The main originality of this thesis is to have approached thequestion of planning, managing and optimizing WQMPs in a holistic manner. The holistic approach transcends into the developing of a participative approach to identify knowledge needs on water quality and quantity to feed an IDSS which assists WQMP managers in every aspect of planning, managing and optimizing WQMPs. ..