Phelan-McDermid syndrome: clinical report of a 70-year-old woman

Item does not contain fulltextPhelan-McDermid or 22q13.3 deletion syndrome is characterized by global intellectual disability, childhood hypotonia, severely delayed or absent speech, features of autism spectrum disorder, without any major dysmorphisms or somatic anomalies. It is typically diagnosed before adolescence and data about adult patients are virtually absent. The expression of its phenotypical characteristics appears to be linearly related to the deletion size. Here, an intellectually disabled geriatric female patient is described with a long history of challenging behaviors in whom Phelan-McDermid syndrome was demonstrated. Detailed analysis of the patient's history and functioning resulted in a psychiatric diagnosis of atypical bipolar disorder and her behavior significantly improved upon maintenance treatment with a mood stabilizing agent. The present article confirms recent findings that atypical bipolar disorder may be part of the psychopathological phenotype of Phelan-McDermid syndrome, reason why careful etiological search is warranted, also in the geriatric population

An indicatorsystem for natural purification in surface waters. Background report

This report contains the literature reviews that have been performed as the basis for a proposal for an indicator system for the life support function 'natural purification of surface waters'. The proposal for the indicator system is given in RIVM report 607605001.In dit rapport zijn een aantal literatuurstudies weergegeven, die als achtergrond gediend hebben bij het voorstel van een graadmeter voor de regulatie functie (life support functie) 'zelfreinigend vermogen van oppervlaktewater'. Het voorstel voor deze indicator is weergegeven in RIVM rapport 60760500

An indicatorsystem for natural purification in surface waters. Background report

In dit rapport zijn een aantal literatuurstudies weergegeven, die als achtergrond gediend hebben bij het voorstel van een graadmeter voor de regulatie functie (life support functie) 'zelfreinigend vermogen van oppervlaktewater'. Het voorstel voor deze indicator is weergegeven in RIVM rapport 607605001This report contains the literature reviews that have been performed as the basis for a proposal for an indicator system for the life support function 'natural purification of surface waters'. The proposal for the indicator system is given in RIVM report 607605001.AlterraRIZAUniversiteit van AmsterdamUniversiteit van Utrech

Limits and Borders: Stages of Transboundary Water Management

Contains fulltext : 111706.pdf (publisher's version ) (Closed access)16 p

Revisiting port pricing; a proposal for seven port pricing principles

A review of seven large landlord port authorities around the world reveals a notable diversity of pricing structures. While port authorities increasingly act as commercial undertakings, port pricing often seems to be not driven by commercial considerations. In this paper, we argue that ports can be regarded as “business ecosystems” with multiple users. This provides a valuable perspective on pricing and raises the question of whether ports can be regarded as two-sided markets. We argue this is not the case. The business ecosystem perspective provides a basis for deducing seven pricing principles for port authorities that are detailed in the paper and illustrated with cases these principles. These pricing principles are broadly follow a direct user pays approach; capture value from “non-core” tenants; price aggressively for activities with a high strategic value; differentiate pricing based on price elasticity and connectivity improvements; maximize revenue from long-term lease agreements, price port dues competitively; critically consider differentiation of charges based on environmental performance; and finally use incentives to align interests of terminal operators and shipping lines. We conclude that the ecosystem perspective is central to the understanding of pricing decisions of port authorities and that various pricing issues deserve more attention