Effect of the addition of a mental health specialist for evaluation of undiagnosed patients in centres for rare diseases (ZSE-DUO): a prospective, controlled trial with a two-phase cohort design.

Background: People with complex symptomatology but unclear diagnosis presenting to a centre for rare diseases (CRD) may present with mental (co-)morbidity. We hypothesised that combining an expert in somatic medicine with a mental health specialist working in tandem will improve the diagnostic outcome.

Methods: Patients aged 12 years and older who presented to one of the 11 participating German CRDs with an unknown diagnosis were recruited into this prospective cohort trial with a two-phase cohort design.

[Quality of Life and Experienced Distress of Patients Suspected of having a Rare (Chronic) Health Condition - Initial Findings from the ZSE-DUO Study].

Objective: Patients suspected of having a rare (chronic) health condition have often gone a long way within the healthcare system. To date, little is known about the health-related quality of life of this group of patients. The study aims to describe the health-related quality of life and the perceived distress of patients suspected of having a rare (chronic) health condition and compare the results with standard values of the German population.

Resolution Limit in Infrared Chemical Imaging.

Chemical imaging combines the spatial specificity of optical microscopy with the spectral selectivity of vibrational spectroscopy. Mid-infrared (IR) absorption imaging instruments are now able to capture high-quality spectra with microscopic spatial detail, but the limits of their ability to resolve spatial and spectral objects remain less understood. In particular, the sensitivity of measurements to chemical and spatial changes and rules for optical design have been presented, but the influence of spectral information on spatial sensitivity is as yet relatively unexplored.

Dual guidance structure for evaluation of patients with unclear diagnosis in centers for rare diseases (ZSE-DUO): study protocol for a controlled multi-center cohort study.

Background: In individuals suffering from a rare disease the diagnostic process and the confirmation of a final diagnosis often extends over many years. Factors contributing to delayed diagnosis include health care professionals' limited knowledge of rare diseases and frequent (co-)occurrence of mental disorders that may complicate and delay the diagnostic process. The ZSE-DUO study aims to assess the benefits of a combination of a physician focusing on somatic aspects with a mental health expert working side by side as a tandem in the diagnostic process.

Temporal and spatial variability of terrestrial diatoms at the catchment scale: controls on productivity and comparison with other soil algae.

Terrestrial diatoms are an integral component of the soil microbial community. However, their productivity and how it compares to other algal groups remains poorly known. This lack of knowledge hampers their potential use as environmental markers in various applications.

Temporal and spatial variability of terrestrial diatoms at the catchment scale: controls on communities.

Diatoms are generally regarded as inhabitants of water bodies. However, numerous taxa are able to survive and reproduce in a variety of non-aquatic ecosystems. Although terrestrial diatoms are discussed extensively in the literature, most of those studies covered floristic aspects and few information exists on their ecology.

Water Vapor, Clouds, and Saturation in the Tropical Tropopause Layer.

The goal of this investigation is to understand the mechanism behind the observed high relative humidity with respect to ice (RHi) in the tropical region between ~14 km (150 hPa) and the tropopause, often referred to as the tropical tropopause layer (TTL). As shown by satellite, aircraft and balloon observations, high (>80%) RHi regions are widespread within the TTL. Regions with the highest RHi are co-located with extensive cirrus.

A global assessment of freshwater mollusk shell oxygen isotope signatures and their relation to precipitation and stream water.

Records of δO in stream flow are critical for understanding and modeling hydrological, ecological, biogeochemical and atmospheric processes. However, the number of such records are extremely limited globally and the length of such time series are usually less than a decade. This situation severely handicaps their use in model testing and evaluation.

Emotions experienced during the shedding of the first primary tooth.

Aim: To examine the different emotions expressed by children exfoliating their first primary tooth, evaluate their proportional distribution, and reveal possible influences of child- and parent-related variables on the reported emotions.

Design: A cross-sectional questionnaire was directed to parents of children who had recently shed at least one primary tooth. Primary outcome variables were the child's emotions at the time of exfoliation.

Microphysical Properties of Tropical Tropopause Layer Cirrus.

Pervasive cirrus clouds in the tropical tropopause layer (TTL) play an important role in determining the composition of stratospheric air through dehydration of tropospheric air entering the stratosphere. This dehydration affects Earth's energy budget and climate, yet uncertainties remain regarding the microphysical processes that govern TTL cirrus. TTL cirrus were sampled with the NASA Global Hawk UAV for over 30 hr in the Western Pacific in 2014 during the Airborne Tropical TRopopause EXperiment.

Mycobacterium leprae genomes from naturally infected nonhuman primates.

Leprosy is caused by the bacterial pathogens Mycobacterium leprae and Mycobacterium lepromatosis. Apart from humans, animals such as nine-banded armadillos in the Americas and red squirrels in the British Isles are naturally infected with M. leprae.

Physical Processes Controlling the Vertical and Longitudinal Distributions of Relative Humidity in the Tropical Tropopause Layer Over the Pacific.

The vertical distribution of relative humidity with respect to ice (RHI) in the Boreal wintertime Tropical Tropopause Layer (TTL, ≃14-18 km) over the Pacific is examined with the extensive dataset of measurements from the NASA Airborne Tropical TRopopause EXperiment (ATTREX). Multiple deployments of the Global Hawk during ATTREX provided hundreds of vertical profiles spanning the longitudinal extent of the Pacific with accurate measurements of temperature, pressure, water vapor concentration, ozone concentration, and cloud properties. We also compare the measured RHI distributions with results from a transport and microphysical model driven by meteorological analysis fields.

Origin and Dynamics of Rare Earth Elements during Flood Events in Contaminated River Basins: Sr-Nd-Pb Isotopic Evidence.

In order to precisely quantify the contribution of anthropogenic activities and geogenic sources to the dissolved and suspended loads of rivers we have combined for the first time Rare Earth Element (REE) concentrations with Sr-Nd-Pb isotope ratios. We observed enrichments in Anthropogenic Rare Earth Elements (AREE) for dissolved (Gd) and suspended (Ce and Nd) loads of river water. During flood events, AREE anomalies progressively disappeared and gave way to the geogenic chemical signature of the basin in both dissolved and suspended loads.

A pervasive role for biomass burning in tropical high ozone/low water structures.

Air parcels with mixing ratios of high O3 and low H2O (HOLW) are common features in the tropical western Pacific (TWP) mid-troposphere (300-700 hPa). Here, using data collected during aircraft sampling of the TWP in winter 2014, we find strong, positive correlations of O3 with multiple biomass burning tracers in these HOLW structures. Ozone levels in these structures are about a factor of three larger than background.

Ice nucleation and dehydration in the Tropical Tropopause Layer.

Optically thin cirrus near the tropical tropopause regulate the humidity of air entering the stratosphere, which in turn has a strong influence on the Earth's radiation budget and climate. Recent high-altitude, unmanned aircraft measurements provide evidence for two distinct classes of cirrus formed in the tropical tropopause region: (i) vertically extensive cirrus with low ice number concentrations, low extinctions, and large supersaturations (up to ∼70%) with respect to ice; and (ii) vertically thin cirrus layers with much higher ice concentrations that effectively deplete the vapor in excess of saturation. The persistent supersaturation in the former class of cirrus is consistent with the long time-scales (several hours or longer) for quenching of vapor in excess of saturation given the low ice concentrations and cold tropical tropopause temperatures.

Solid phase extraction coupled to liquid chromatography-tandem mass spectrometry analysis of sulfonamides, tetracyclines, analgesics and hormones in surface water and wastewater in Luxembourg.

In the early 1990s different studies highlighted the relationship between pharmaceuticals, human health and the environment. Among the emerging contaminants, antibiotics are obviously of high concern, because of their potential for inducing antibiotic resistance. In addition, natural and synthetic hormones are relevant because of their potential endocrine-disrupting effects on wildlife.

Controlled nerve growth factor release from multi-ply alginate/chitosan-based nerve conduits.

The delivery kinetics of growth factors has been suggested to play an important role in the regeneration of peripheral nerves following axotomy. In this context, we designed a nerve conduit (NC) with adjustable release kinetics of nerve growth factor (NGF). A multi-ply system was designed where NC consisting of a polyelectrolyte alginate/chitosan complex was coated with layers of poly(lactide-co-glycolide) (PLGA) to control the release of embedded NGF.

Variations in glial cell line-derived neurotrophic factor release from biodegradable nerve conduits modify the rate of functional motor recovery after rat primary nerve repairs.

Accelerating axonal regeneration to shorten the delay of reinnervation and improve functional recovery after a peripheral nerve lesion is a clinical demand and an experimental challenge. We developed a resorbable nerve conduit (NC) for controlled release of glial cell line-derived neurotrophic factor (GDNF) with the aim of assessing motor functional recovery according to the release kinetics of this factor in a short gap model. Different types of resorbable NCs were manufactured from a collagen tube and multiple coating layers of poly(lactide-coglycolide), varying in poly(lactide-coglycolide) type and coating thickness to afford three distinct release kinetics of the neurotrophic factor.

Nerve conduits and growth factor delivery in peripheral nerve repair.

Peripheral nerves possess the capacity of self-regeneration after traumatic injury. Transected peripheral nerves can be bridged by direct surgical coaptation of the two nerve stumps or by interposing autografts or biological (veins) or synthetic nerve conduits (NC). NC are tubular structures that guide the regenerating axons to the distal nerve stump.

Hydrogel nerve conduits produced from alginate/chitosan complexes.

Nerve conduits (NCs) represent a promising alternative to conventional treatments for peripheral nerve repair. Materials for NC production should be biodegradable, possess adequate mechanical properties, and allow for exchange of nutrients. To this aim, we developed biodegradable NC made of a hydrogel that consisted of the oppositely charged polysaccharides alginate and chitosan.

Genetically engineered herpes simplex viruses that express IL-12 or GM-CSF as vaccine candidates.

We are using genetically modified, conditionally replicating herpes simplex virus (HSV) that express either interleukin (IL)-12 or granulocyte macrophage-colony stimulating factor (GM-CSF) as live, attenuated vaccine candidates for protection against HSV infection and/or disease. We report the following: (1) animals previously vaccinated with these candidate vaccines exhibited dose-dependent protection after intranasal, intraperitoneal or intracranial challenge with the highly virulent E377-MB wild-type HSV-1; (2) the IL-12 expressing virus (M002) consistently conferred protection at lower immunization doses than GM-CSF expressing virus (M004); (3) between 80 and 100% protection from E377-MB challenge was conferred after intramuscular immunization of mice with any of the three Deltagamma1 34.5 HSV, as opposed to 50% protection elicited after immunization with wild-type HSV-1 (F); and (4) latent virus was not detected at a higher rate in animals immunized and subsequently challenged with E377-MB than in immunized animals alone.

Climatic change and the effect on hydrology and water management in the Rhine basin.

There has been a marked increase in recent years in the resources dedicated to investigating the problems arising from climate change with respect to hydrology and to some extent to water resource management. Many of these studies are concentrated on the river Rhine basin. In order to review the actual state of scientific findings a workshop, organised by the International Commission for the Hydrology of the Rhine (CHR), was held on 24 and 25 June 2003.

Spatial variability of trends in hydrological extremes induced by orographically enhanced rainfall events due to westerly atmospheric circulations.

Since the mid 1970s, the number of days with westerly atmospheric circulations has strongly increased during winter months. As a consequence, rainfall totals, rainfall event duration and intensity have been subject to significant positive trends throughout the Mosel river basin. However, the trends identified through the non-parametrical test named Kendall's tau have shown to be spatially varying.