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In the present study, a morphological model based on the main governing processes of inlet channels was developed for application in long-term simulations, requiring minimum data on forcing and only key information on the inlet geometry. The model considers the balance between the inlet sand transport due to tides and river flows and the longshore transport due to breaking waves. The resulting equ

It has been suggested that non-aqueous solvents containing amines may provide alternatives to aqueous alkanolamine solvents due to the potentially lower energy requirement for the regeneration of the amine. This paper presents experimental data on the solubility of CO2 and heat of absorption in the organic solvent N-methyl-2-pyrrolidone (NMP) and mixtures of 2-amino-2-methyl-1-propanol (AMP) in NM

In the recent Regards Photographiques judgement, the European Court of Justice held that member states cannot limit the reduced value added tax (VAT) rate to artistic photographs. In this article the judgement is analysed and discussed, also in connection with previous case law on Customs law. Also, it is discussed whether this judgement would oblige the Netherlands to apply the reduced VAT rate t

The compliance policy of the Dutch tax administration does not seem to include charities. In the article it is discussed how charities could be fitted into this compliance policy making use of the compliance pyramid of the Australian tax administration.

The role of pacing in vasovagal syncope (VVS) is considered from a physiological basis. Most VVS patients lose consciousness due to hypotension before severe bradycardia/asystole occurs. Patients that benefit from dual-chamber pacing are typically older with highly symptomatic, late-onset, frequent and severe syncope with short/no prodrome and documented severe cardioinhibition. Tilt-testing is of

This work showcases the remarkable viscoelasticity of films consisting of α-cyclodextrin (α-CD) and anionic surfactants (S) at the water/air interface, the magnitude of which has not been observed in similar systems. The anionic surfactants employed are sodium salts of a homologous series of n-alkylsulfates (n = 8–14) and of dodecylsulfonate. Our hypothesis was that the very high viscoelasticity c

The German Geophysical Society (Deutsche Geophysikalische Gesellschaft, DGG) was founded in 1922 in Leipzig, Germany, on the initiative of the famous German seismologist Emil Wiechert (1861–1928), known for his fundamental work to record earthquake waves to study the earth's interior. Facing the German historical background of the early 20th century, the 24 founding members wanted to lead German g

Background: Maternal psychopathology can be an important factor associated with psychological adjustment of children. However, there is limited research on long-term impacts of maternal posttraumatic stress disorder (PTSD) on children's mental health. This study examined how PTSD trajectories of women exposed to the 2008 Wenchuan earthquake in China predicted their children's mental health symptom

Early onset cerebellar Ataxia (EOAc) comprises a large group of rare heterogeneous disorders. Determination of the underlying etiology can be difficult given the broad differential diagnosis and the complexity of the genotype–phenotype relationships. This may change the diagnostic work-up into a time-consuming, costly and not always rewarding task. In this overview, the Childhood Ataxia and Cerebe

Phenotypic and biochemical categorization of humans with detrimental variants can provide valuable information on gene function. We illustrate this with the identification of two different homozygous variants resulting in enzymatic loss-of-function in LDHD, encoding lactate dehydrogenase D, in two unrelated patients with elevated D-lactate urinary excretion and plasma concentrations. We establish

Although movement disorders (MDs) are known complications, the exact frequency and severity remains uncertain in patients with classical galactosemia, especially in children. We determined the frequency, classification and severity of MDs in a cohort of pediatric and adult galactosemia patients, and assessed the association with nonmotor neuropsychological symptoms and daily functioning. Patients

Evacuation planning is an important activity in disaster management to reduce the effects of disasters on urban communities. It is regarded as a multi-objective optimization problem that involves conflicting spatial objectives and constraints in a decision-making process. Such problems are difficult to solve by traditional methods. However, metaheuristics methods have been shown to be proper solut

The aim of this research was to develop a method to produce a Dust Source Susceptibility Map (DSSM). For this purpose, we applied remote sensing and statistical-based machine learning algorithms for experimental dust storm studies in the Khorasan Razavi Province, in north-eastern Iran. We identified dust sources in the study area using MODIS satellite images during the 2005–2016 period. For dust s

Background: Rare and ultra-rare diseases (URDs) are often chronic and life-threatening conditions that have a profound impact on sufferers and their families, but many are notoriously difficult to detect. Niemann-Pick disease type C (NP-C) serves to illustrate the challenges, benefits and pitfalls associated with screening for ultra-rare inborn errors of metabolism (IEMs). A comprehensive, non-sys

Inborn errors of metabolism in adults are still largely unexplored. Despite the fact that adult-onset phenotypes have been known for many years, little attention is given to these disorders in neurological practice. The adult-onset presentation differs from childhood-onset phenotypes, often leading to considerable diagnostic delay. The identification of these patients at the earliest stage of dise

Background: The clinical demarcation of the syndrome progressive myoclonus ataxia is unclear, leading to a lack of recognition and difficult differentiation from other neurological syndromes. Objectives: The objective of this study was to apply a refined definition of progressive myoclonus ataxia and describe the clinical characteristics in patients with progressive myoclonus ataxia and with isola

Background: DYT6 dystonia can have an unpredictable clinical course and the result of deep brain stimulation (DBS) of the internal part of the globus pallidus (GPi) is known to be less robust than in other forms of autosomal dominant dystonia. Patients who had previous stereotactic surgery with insufficient clinical benefit form a particular challenge with very limited other treatment options avai

Patients with an ADCY5 gene mutation reveal a heterogenous clinical presentation including axial hypotonia, motor milestone delay, fluctuating dyskinesias, dystonia, and/or myoclonus with episodic exacerbations during drowsiness and sleep.1,2 Phenotype-genotype correlations and somatic mosaicism are suggested to explain the wide phenotypic spectrum.1 The ADCY5 gene encodes 1 of 9 membrane-bound ad