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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.

Pacing in vasovagal syncope : physiology, pacemaker sensors and recent clinical trials. Precise patient selection and measurable benefit

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

Highly viscoelastic films at the water/air interface : α-Cyclodextrin with anionic surfactants

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

Gender equality in the German Geophysical Society

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

Trajectories of maternal symptoms of posttraumatic stress disorder predict long-term mental health of children following the Wenchuan earthquake in China : A 10-year follow-up study

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

A clinical diagnostic algorithm for early onset cerebellar ataxia

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

Identification of human D lactate dehydrogenase deficiency

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

Movement disorders and nonmotor neuropsychological symptoms in children and adults with classical galactosemia

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

A Comparative Study of Four Metaheuristic Algorithms, AMOSA, MOABC, MSPSO, and NSGA-II for Evacuation Planning

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

Application of remote sensing techniques and machine learning algorithms in dust source detection and dust source susceptibility mapping

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

Recommendations for patient screening in ultra-rare inherited metabolic diseases : What have we learned from Niemann-Pick disease type C?

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

Eye movement disorders and neurological symptoms in late-onset inborn errors of metabolism

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

Progressive myoclonus ataxia : Time for a new definition?

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

Reversal of status dystonicus after relocation of pallidal electrodes in DYT6 generalized dystonia

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

Expanding the ADCY5 phenotype toward spastic paraparesis : Amutation in the M2 domain

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

A preliminary study of telemedicine for patients with hepatic glycogen storage disease and their healthcare providers : from bedside to home site monitoring

Background: The purpose of this project was to develop a telemedicine platform that supports home site monitoring and integrates biochemical, physiological, and dietary parameters for individual patients with hepatic glycogen storage disease (GSD). Methods and results: The GSD communication platform (GCP) was designed with input from software developers, GSD patients, researchers, and healthcare p

Treatable inherited rare movement disorders

There are many rare movement disorders, and new ones are described every year. Because they are not well recognized, they often go undiagnosed for long periods of time. However, early diagnosis is becoming increasingly important. Rapid advances in our understanding of the biological mechanisms responsible for many rare disorders have enabled the development of specific treatments for some of them.