Perinatal-lethal Gaucher disease presenting as hydrops fetalis

Perinatal-lethal Gaucher disease is very rare and is considered a variant of type 2 Gaucher disease that occurs in the neonatal period. The most distinct features of perinatal-lethal Gaucher disease are non-immune hydrops fetalis. Less common signs of the disease are hepatosplenomegaly, ichthyosis and arthrogryposis. We report a case of Gaucher's disease (type 2) diagnosed in a newborn who presented with Hydrops Fetalis

A highly efficient green synthesis of 1, 8-dioxo-octahydroxanthenes

SmCl3 (20 mol%) has been used as an efficient catalyst for reaction between aromatic aldehydes and 5,5-dimethyl-1,3-cyclohexanedione at 120°C to give 1,8-dioxo-octahydroxanthene derivatives in high yield. The same reaction in water, at room temperature gave only the open chain analogue of 1,8-dioxo-octahydroxanthene. Use of eco-friendly green Lewis acid, readily available catalyst and easy isolation of the product makes this a convenient method for the synthesis of either of the products

Towards an integrated workflow for cooperative process management

International audienc

A Process Traceability Methodology to Support Conflict Management

International audienc

A Process Model Based Methodology to Support Conflict Management

International audienc

Vers une structuration de la collaboration au sein du processus de conception

National audienc

Comprehensive biochemistry

ISO 55000 puts ‘value’ at the core of asset management. This paper provides a framework to help production companies implement value-based Asset Management (AM) in a way that it contributes to operational excellence. Value-based AM is achieved when the value delivered by assets is used by the organization as the key decision criterion to choose between different AM options (both at tactical and operational level). Given this perspective, it is vital that organizations are able to quantify the value delivered by their assets and manage that value through informed and coherent decision-making. Value-based AM is still a concept more quoted in theory than described in practical terms. A clear understanding about the main elements that are needed to enable it is still missing in industrial practice. The framework presented in this paper provides the key elements needed for successful integration of a value quantification model with the AM system to ensure the effective implementation of value-based approach in AM