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Phenylketonuria (PKU), a genetic metabolic disorder that is characterized by the inability to convert phenylalanine to tyrosine, leads to severe intellectual disability and other cerebral complications if left untreated. Dietary treatment, initiated soon after birth, prevents most brain-related complications. A leading hypothesis postulates that a shortage of brain monoamines may be associated with neurocognitive deficits that are observable even in early-treated PKU. However, there is a paucity of evidence as yet for this hypothesis.
Methods
We therefore assessed in vivo striatal dopamine D2/3 receptor (D2/3R) availability and plasma monoamine metabolite levels together with measures of impulsivity and executive functioning in 18 adults with PKU and average intellect (31.2 ± 7.4 years, nine females), most of whom were early and continuously treated. Comparison data from 12 healthy controls that did not differ in gender and age were available.
Results
Mean D2/3R availability was significantly higher (13%; p = 0.032) in the PKU group (n = 15) than in the controls, which may reflect reduced synaptic brain dopamine levels in PKU. The PKU group had lower plasma levels of homovanillic acid (p < 0.001) and 3-methoxy-4-hydroxy-phenylglycol (p < 0.0001), the predominant metabolites of dopamine and norepinephrine, respectively. Self-reported impulsivity levels were significantly higher in the PKU group compared with healthy controls (p = 0.033). Within the PKU group, D2/3R availability showed a positive correlation with both impulsivity (r = 0.72, p = 0.003) and the error rate during a cognitive flexibility task (r = 0.59, p = 0.020).
Conclusions
These findings provide further support for the hypothesis that executive functioning deficits in treated adult PKU may be associated with cerebral dopamine deficiency.
Hepatitis C virus (HCV) is a major cause of chronic liver disease worldwide. A patient was recently found to be HCV seropositive during hemodialysis follow-up.
OBJECTIVE
To determine whether nosocomial transmission had occurred and which viral populations were transmitted.
DESIGN
HCV transmission case.
SETTING
A dialysis unit in a French hospital.
METHODS
Molecular and epidemiologic investigations were conducted to determine whether 2 cases were related. Risk analysis and auditing procedures were performed to determine the transmission pathway(s).
RESULTS
Sequence analyses of the NS5b region revealed a 5a genotype in the newly infected patient. Epidemiologic investigations suggested that a highly viremic genotype 5a HCV-infected patient who underwent dialysis in the same unit was the source of the infection. Phylogenetic analysis of NS5b and hypervariable region-1 sequences revealed a genetically related virus (>99.9% nucleotide identity). Deep sequencing of hypervariable region-1 indicated that HCV quasispecies were found in the source whereas a single hypervariable region-1 HCV variant was found in the newly infected patient, and that this was identical to the major variant identified in the source patient. Risk analysis and auditing procedures were performed to determine the transmission pathway(s). Nosocomial patient-to-patient transmission via healthcare workers’ hands was the most likely explanation. In our dialysis unit, this unique incident led to the adjustment of infection control policy.
CONCLUSIONS
The data support transmission of a unique variant from a source with a high viral load and genetic diversity. This investigation also underlines the need to periodically evaluate prevention and control practices.
Infect. Control Hosp. Epidemiol. 2016;37(2):134–139
Several ways using surface modification are commonly used to enhance thermal
stability of polymer matrices. In this study, Continuous-Wave carbon dioxide
(CW CO2) laser irradiation and atmospheric pressure non-thermal He
plasma treatment on microporous poly(vinyl chlo-ride)/Silica composite have
been investigated and compared. On one hand the alternative was based on the
efficiency of the thermal energy afforded by CW CO2 laser irradiation
and induced photodegradation processes to release HCl and form polyene
sequences under well-controlled condition. On the other hand atmospheric
plasma treatment involved surface modification by formation of unstable
radicals, inducing crosslinking and dehydrochlorination. In both cases a
global thermal stabilization of the composite was noticed by partial
dehydrochlorination of PVC, even for short exposure time. The main effects
observed after laser irradiation were related to the formation of a very
dense structure on surface with very low chlorine content and an important
chlorine atoms in-depth gradient on the cross section up to 150 μm in
thickness; whereas atmospheric He plasma treatment led to a homogeneous
decrease of the chlorine in-depth content due to the plasma interpenetration
in the porous microstructure.
Structural and chemical modifications both on extreme surface and in the
thickness have been investigated respectively by Environmental Scanning
Electron Microscope (ESEM) coupled with an Energy Dispersive X-ray analysis
(EDX) and X-ray Photoelectron Spectroscopy (XPS). Composites thermal
stability and investigation on chlorine release have been evaluated by
Thermo Gravimetric Analysis (TGA) coupled with Mass Spectrometry (MS).
Experimental and theoretical evidence is presented for oxygen DX centers in AlxGa1−xN. As the aluminum content increases, Hall effect measurements reveal an increase in the electron activation energy, consistent with the emergence of a deep DX level from the conduction band. Persistent photoconductivity is observed in Al0 39Ga0. 61N:O at temperatures below 150 K after exposure to light, with an optical threshold energy of 1.3 eV, in excellent agreement with first-principles calculations. Unlike oxygen, silicon does not exhibit DX-like behavior, in agreement with previous theoretical predictions.
The structural quality of GaN films grown by hydride vapor phase epitaxy (HVPE) was characterized by transmission electron microscopy (TEM), x-ray diffraction (XRD), and atomic force microscopy (AFM). Films were grown up to 40μm on sapphire with either a GaC1 pretreatment prior to growth or on a ZnO buffer layer. Dislocation densities were found to decrease with increasing film thickness. This is attributed to the mixed nature of the defects present in the film which enabled dislocation annihilation. The thickest film had a defect density of 5×107 dislocations/cm2.
Gax In1 − x Pepilayers grown under a range of growth conditions by organometallic vapor phase epitaxy (OMVPE) on GaAs substrates have been studied in the electron microscope. The results show the presence of an ordering of the group III sublattice parallel to some of the {111} planes. Dark-field images directly reveal ordered domains of different orientations that appear not to be perfect, but contain many planar defects parallel to the growth surface.
Ordered GaxIn1-xP epilayers grown on GaAs substrates have been studied in the electron microscope. Dark-field images and high-resolution micrographs have directly revealed the presence of an ordering of the Ga and In (111) planes. Several different planar defects have been identified in the ordered structure. There is a very high density of faults parallel to the (001) growth surface. Models for these faults have been constructed in terms of stacking faults and twin boundaries in the ordered structure. Other flat planar defects which occur approximately parallel to (111) and (111) planes can also be described in these terms.
The microstructure of the interfacial zone in a SCS6 filaments/Ti-6A1–4V matrix composite was determined using TEM and HREM. The interfacial zone (e ∼5 μm) consists of microstructurally distinct layers. The fiber/matrix interaction resulted in two layers respectively crystallized in grains of about 20 nm and 100 nm. The reaction zones are mainly made of brittle TiC. They are separated from the SiC reinforcement by five layers which form the coating of the SCS6 filament. These five layers may be described as SiC-C composites with either an equiaxe fine grain structure or a distorted lamellar structure. The structure of these layers can explain the toughning of the alloy by the SCS6 filaments despite of the brittle F/M reaction products formed during the processing.
GaxIn(1−x)P epilayers grown on GaAs substrates by MOVPE, for different growth temperatures and values of x∼0.5, have been studied by electron microscopy. The results indicate that under certain conditions the ternary epilayer is ordered parallel to the (111) plane. Dark-field images obtained using the superlattice reflections reveal ordered domains of different orientations. High-resolution images have been obtained from the ordered domains. The structure of these domains is not perfect but contains many planar faults parallel to the growth surface.
The National Enforcement Investigations Center of the EPA provides support services for the enforcement activities of the Agency. Recently, we have analyzed hazardous wastes as part of efforts to enforce the Resource Conservation and Recovery Act and the Superfund Act. Sample preparation for inorganic elemental analysis is a difficult and time-consuming step. Thus, it would be desirable to be able to use x-ray fluorescence methods which require relatively little sample preparation for the analysis of solid hazardous wastes. A major problem to be overcome is the need to calibrate for a large variety of samples. However, a compensating factor is that the error will be largely determined by the sampling error and the measurement accuracy is not quite so critical.
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