Abstract
Using the PION ICRH modelling code and comparisons against JET tokamak experiments, the effect of including pitch angle dependence within the RF diffusion operator on the fast ion particle ...distribution functions is quantified. It is found to be of greatest importance in cases of higher harmonic heating and lower heating ion mass, resulting in faster drop-off of the distribution’s high energy tail. We see differences of several orders of magnitude in the high-energy range and significant non-linear alterations by several tens of percent to ion species power partition. ITER scenario operational parameters are also considered, and this improved treatment is shown to benefit anticipated ITER scenarios with second harmonic hydrogen heating, according to our predictions. PION’s combination of benchmarked simplified wave physics and Fokker-Planck treatment offers modelling advantages. Since including the pitch angle dependence in the RF diffusion operator has not led to a significant increase in the required computing time when modelling different ICRF schemes in JET discharges, it has been made available within the production code.
Abstract
Achieving high-performance conditions and maximizing the fusion yield of plasma discharges have been one of the main goals of recent Joint European Torus (JET) campaigns in preparation for ...its second deuterium–tritium (D–T) campaign. The simulations shown in this work delve into the role of external heating using neutral beam injection (NBI) and radio-frequency waves in the ion cyclotron range of frequencies (ICRF) in order to optimise high fusion performance in the JET tokamak. A baseline discharge with record neutron rate is used as a reference in order to perform a D–T prediction, which considers the NBI+RF synergy. In this work, our focus is on JET’s two main minority schemes, H and
3
He. This study tackles the heating mechanisms by which these schemes reach high-performance conditions. The H scheme typically boosts the ICRF fusion enhancement through the second D harmonic heating, whereas
3
He minority is characterised by its strong bulk ion heating. Both features are beneficial for increasing the fusion yield. Nevertheless, the minority concentration is a relevant parameter, which needs to be assessed to understand in which concentration ranges the benefits of each particular minority scheme are met. Therefore, the main objective of this work is to assess in which concentration range the ICRF fusion enhancement and bulk ion heating are optimal, for H and
3
He, respectively. Under these conditions, our prediction suggests
3
He concentration should stay above 1.2% and H should remain below 2.2%.
Dedicated studies performed for toroidal Alfvén eigenmodes (TAEs) in ASDEX-Upgrade (AUG) discharges with monotonic q-profiles have shown that electron cyclotron resonance heating (ECRH) can make TAEs ...more unstable. In these AUG discharges, energetic ions driving TAEs were obtained by ion cyclotron resonance heating (ICRH). It was found that off-axis ECRH facilitated TAE instability, with TAEs appearing and disappearing on timescales of a few milliseconds when the ECRH power was switched on and off. On-axis ECRH had a much weaker effect on TAEs, and in AUG discharges performed with co- and counter-current electron cyclotron current drive (ECCD), the effects of ECCD were found to be similar to those of ECRH. Fast ion distributions produced by ICRH were computed with the PION and SELFO codes. A significant increase in Te caused by ECRH applied off-axis is found to increase the fast ion slowing-down time and fast ion pressure causing a significant increase in the TAE drive by ICRH-accelerated ions. TAE stability calculations show that the rise in Te causes also an increase in TAE radiative damping and thermal ion Landau damping, but to a lesser extent than the fast ion drive. As a result of the competition between larger drive and damping effects caused by ECRH, TAEs become more unstable. It is concluded, that although ECRH effects on AE stability in present-day experiments may be quite significant, they are determined by the changes in the plasma profiles and are not particularly ECRH specific.
Weiss-Kruszka syndrome (WSKA) is a rare disorder caused by mutations in the
gene or deletion of 9p31.2 chromosome region, involving
. The prevalence of WSKA is unknown as only 24 affected individuals ...have been described. This syndrome should be suspected in individuals presenting mild global developmental delay and common craniofacial abnormalities.
We presented a case of an infant, 3 years and 4-month life who presented pondostatural and psychomotor retardation, generalized hypotonia with hypermobility, bilateral palpebral ptosis, epicanthal folds, and poorly expressive facies as the main clinical features. These characteristics lead to the realization of genetics studies that resulted in the identification of a novel mutation c.3306dup; p.(Gln1103Thrfs*10) in
.
WSKA should be suspected in individuals presenting mild global developmental delay, ptosis, downslanting palpebral fissures, exaggerated Cupid's Bow, arched eyebrows, epicanthal folds and short upturned nose with a bulbous tip. Hypertrophy of the ventricular septum and severe OSA were described in our patient and should be considered in future reviews of the disease. This case is added to the reduced number of publications previously reported regarding WSKA and contributes to understanding the genetic characteristics, clinical features, and diagnosis of this syndrome.
WSKA: Weiss-Kruszka syndrome; CP: craniofacial perimeter; WES: whole-exome sequencing; RSV: respiratory syncytial virus; OSA: obstructive sleep apnoea; ACMG: American College of Medical Genetics and Genomics.
INTRODUCTIONNeurofibromatosis type 1 (NF1) is a progressive multisystem disorder following an autosomal dominant inheritance pattern that presents with multiple neurological manifestations.METHODSWe ...reviewed medical histories of patients with NF1 followed up at our hospital's paediatric neurology department from May 1990 to 31 December 2018. We collected data on neurological symptoms.RESULTSA total of 128 patients with NF1 were identified. Mean age (SD) at NF1 diagnosis was 4.43 (3.38) years (range, 0.5-14.5 years). There was a slight female predominance (53.1%). Macrocephaly (head circumference over 2 SDs above average for age) was present in 37.5% of cases. Attention-deficit/hyperactivity disorder was recorded in 28.9% of patients (37): combined type in 20 patients, predominantly inattentive in 15, and predominantly impulsive/hyperactive in 2. Other manifestations included headache (18.6%), cognitive impairment (7.8%), motor deficit (6.2%), and epilepsy (4.68%). Brain MRI was performed in 85 patients, revealing T2-weighted hyperintensities in the basal ganglia and/or cerebellum in 60 patients (70.5%), Chiari malformation type 1 in 4 cases, and arachnoid cysts in 3. Optic nerve gliomas were identified by MRI in 22 patients (25.8%). Other MRI findings included plexiform neurofibromas (9.3%) and central nervous system gliomas (3.1%).CONCLUSIONSThe neurological manifestations identified in our sample are consistent with those reported in the literature. Effective transfer strategies from paediatric neurology departments and subsequent clinical follow-up by adult neurology departments are needed to prevent loss to follow-up in adulthood.
Abstract
The required heating power,
P
L
H
, to access the high confinement regime (H-mode) in tritium containing plasmas is investigated in JET with ITER-like wall at a toroidal magnetic field of
B
...t
=
1.8
T and a plasma current of
I
p
=
1.7
MA.
P
L
H
, also referred to as the L-H power threshold, is determined in plasmas of pure tritium as well as mixtures of hydrogen with tritium (H-T) and mixtures of deuterium with tritium (D-T), and is compared to the L-H power threshold in plasmas of pure hydrogen and pure deuterium. It is found that, for otherwise constant parameters,
P
L
H
is not the same in plasmas with the same effective isotope mass,
A
e
f
f
, when they differ in their isotope composition. Thus,
A
e
f
f
is not sufficient to describe the isotope effect of
P
L
H
in a consistent manner for all considered isotopes and isotope mixtures. The electron temperature profiles measured at the L-H transition in the outer half of the radius are very similar for all isotopes and isotope mixtures, despite the fact that the L-H power threshold varies by a factor of about six. This finding, together with the observation of an offset linear relation between the L-H power threshold,
P
L
H
, and an effective heat diffusivity,
χ
e
f
f
, indicates that the composition-dependent heat transport in the low confinement mode (L-mode) determines, how much power is needed to reach the necessary electron temperatures at the edge, and hence
P
L
H
.
An infrared spectroscopy (IR) based methodology has been developed to determine γ-butyrolactone (GBL) in adulterated beverages. The proposed method permits the direct screening of GBL in beverages ...and involves a minimum sample treatment requiring less than 2 min for quantitative determination of GBL. Sensitivity of IR method was improved by using liquid-liquid extraction (LLE) providing detection limits of 0.023 mg g−1. Accuracy of the proposed methodology was evaluated through the analysis of soft beverages and alcoholic cocktails spiked with GBL at concentration levels ranging from 0.075 to 10 mg g−1 providing recovery values from 91 to 100%. GBL was determined in twelve blind-spiked beverages, including from mineral water to wine and cocktails. Results obtained were statistically comparable to those provided by a liquid chromatography (LC) reference methodology and consistent with the spiked values. Therefore, the use of LLE-FTIR allowed a simple, sensitive and quantitative determination of GBL in soft beverages and alcoholic cocktails, thus evidencing its use for sex submission intention.
Display omitted
•Fast and simple determination of GBL in beverages.•Method based on one step liquid extraction and direct transmission IR measurements.•Forensic applications with conventional instrumentation.
•Evaluation of five analytical methodologies for MDMA determination.•Greenness assessment by modified Eco-scale, GAPI and AGREE criteria.•Comparison of green scores obtained for the MDMA ...determination procedures.•Direct IR spectroscopy methods provides the greenest evaluations.
The determination of 3,4-methylenedioxymethamphetamine (MDMA) in seized samples, by employing five different analytical procedures, has been used to illustrate the differences in their greenness classification depending on the selected evaluation criteria. A set of seventeen MDMA seized samples were analysed by: a) liquid chromatography with diode array detection (LC-DAD), b) gas chromatography with flame ionization detection (GC-FID), c) ion mobility spectrometry (IMS), d) total attenuated reflectance mid-infrared spectroscopy (ATR-FTIR) and e) diffuse reflectance near infrared spectroscopy (DR-NIR). The studied analytical procedures provided statistically comparable MDMA concentration values. However, the greenness scores of analytical procedures using the three criteria of Green Analytical Eco-Scale, the Green Analytical Procedure Index (GAPI) and the Analytical GREEnness metric approach (AGREE) provided significant differences but in all cases ATR-FTIR and DR-NIR were the greenest methods followed by IMS, GC and LC. The aforementioned greenness aspects are discussed in this study.
Display omitted
The use of ion mobility spectrometry (IMS) has been evaluated as analytical methodology to detect and evaluate the occupational exposure to pesticides. The developed IMS methodology was used, in ...positive and negative modes, to determine the presence of pesticides in air and to evaluate possible inhalation exposures of workers and users based on active sampling on Teflon membranes and direct thermal desorption IMS. The negative IMS mode was used to determine bensulfuron, clorpyrifos, diniconazole, diuron, flutolanil and imidacloprid, while the positive mode was employed to evaluate formetanate, metalaxyl, metamitrone, metribuzin, paclobutrazol and pirimicarb. The IMS measurements provided limits of detection from 8pg to 600pg. Indoor air samples, from phytosanitary plants, and outdoor samples, obtained from pesticide treatments in a local farm, were analysed providing pesticide air concentrations in the range of 0.04 to>0.25mgm−3. Occupational exposure of workers and pesticide users were evaluated and compared with values recommended by the authorities, providing useful information to improve the prevention programs in the phytosanitary field.
Display omitted
•IMS to evaluate occupational exposure in the pesticide industry.•Detection limits of the order of pg of pesticides per membrane.•Active sampling through PTFE filters as sampling method.•Qualitative and quantitative information can be obtained.
The exposure to smoking related products has been evaluated through urine illness risk marker determination through the analysis of urine samples of smokers and vapers. Biomarkers and their ...metabolites such as
N
-acetyl-
S
-(2-cyanoethyl)-L-cysteine (CEMA),
N
-acetyl-
S
-(3,4-dihydroxybutyl)-L-cysteine (DHBMA),
N
-acetyl-
S
-1-(hydroxymethyl)-2-propen-1-yl)-L-cysteine (MHBMA),
N
-acetyl-
S
-(3-hydroxypropyl)-L-cysteine (3HPMA), 2
R
-
N
-acetyl-
S
-(4-hydroxybutan-2-yl)-L-cysteine (HMPMA), and
N
-acetyl-
S
-(3-carboxy-2-propyl)-L-cysteine (CMEMA) together with nicotine and cotinine were identified and quantified by LC-HRMS and LC-MS/MS, and data found normalized to the creatinine level. One hundred two urine samples were collected from smokers, non-smokers, and vapers, spanning an age range from 16 to 79 years. Results obtained showed that CEMA was only detected in urine samples from smokers and MHBMA was in the same order of magnitude in all the urine samples analyzed. HMPMA was found in the urine of vapers at the same order of concentration as in non-smokers. 3HPMA in vapers was lower than in the urine of smokers, presenting an intermediate situation between smokers and non-smokers. On the other hand, DHBMA in vapers can reach similar values to those found for smokers, while CMEMA shows concentrations in the urine of vapers higher than in the case of non-smokers and traditional smokers, requiring new research to link this metabolite to the use of electronic cigarettes and possible alternative metabolomic routes. In general, this study seems to verify that traditional smoking practice constitutes a major source of carcinogenic chemicals compared with substitutive practices, although those practices are not free of potential harm.
Graphical abstract
PDF
