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The photoelectrochemical performance of Ta₃N₅ photoanodes is strongly impacted by the presence of shallow and deep defects within the bandgap. However, the role of such states in defining the stability under operational conditions is not well understood. Here, we use a highly controllable synthesis approach to create homogenous Ta₃N₅ thin films with tailored defect concentrations to establish the relationship between atomic-scale point defects and macroscale stability. Reduced oxygen contents increase long-range structural order but lead to high concentrations of deep-level states, while higher oxygen contents result in reduced structural order but beneficially passivate deep-level defects. Despite the different defect properties, the synthesized photoelectrodes degrade similarly under water oxidation conditions due to formation of a surface oxide layer that blocks interfacial hole injection and accelerates charge recombination. In contrast, under ferrocyanide oxidation conditions, we find that Ta₃N₅ films with high oxygen concentrations exhibit long-term stability, whereas those possessing lower oxygen contents and higher deep-level defect concentrations rapidly degrade. These results indicate that deep-level defects result in rapid trapping of photocarriers and surface oxidation but that shallow oxygen donors can be introduced into Ta₃N₅ to enable kinetic stabilization of the interface.

Ta3N5 shows great potential as a semiconductor photoanode for solar water splitting. However, its performance is hindered by poor charge carrier transport and trapping due to a high density of defects that introduce electronic states deep within its bandgap. Here, we demonstrate that controlled Ti doping of Ta3N5 can dramatically reduce the concentration of deep-level defects and enhance its photoelectrochemical performance, yielding a sevenfold increase in photocurrent density and a 300 mV cathodic shift in photocurrent onset potential compared to undoped material. Comprehensive characterization reveals that Ti+4 ions substitute Ta+5 lattice sites, thereby introducing compensating acceptor states, reducing concentrations of nitrogen vacancies and reduced Ta+3 states, and thereby suppressing trapping and recombination. Importantly, Ti doping offers distinct advantages compared to Zr, an intensively investigated dopant of Ta3N5 in the same group of the periodic table. Specifically, Ti+4 and Ta+5 have more similar atomic radii, allowing for substitution without introducing lattice strain, and Ti exhibits a lower affinity for oxygen than Zr, enabling its incorporation without increasing the oxygen donor content. Consequently, we demonstrate that the electrical conductivity can be tuned by over seven orders of magnitude. Thus, Ti doping in Ta3N5 provides a powerful basis for precisely engineering the optoelectronic characteristics of Ta3N5 and to substantially improve its functional characteristics as an advanced photoelectrode for solar fuels applications.

Transition metal oxynitrides are a promising class of functional materials for photoelectrochemical (PEC) applications. Although these compounds are most commonly synthesized via ammonolysis of oxide precursors, such synthetic routes often lead to poorly controlled oxygen-to-nitrogen anion ratios, and the harsh nitridation conditions are incompatible with many substrates, including transparent conductive oxides. Here, we report direct reactive sputter deposition of a family of zirconium oxynitride thin films and the comprehensive characterization of their tunable structural, optical, and functional PEC properties. Systematic increases of the oxygen content in the reactive sputter gas mixture enable access to different crystalline structures within the zirconium oxynitride family. Increasing oxygen contents lead to a transition from metallic to semiconducting to insulating phases. In particular, crystalline Zr2ON2-like films have band gaps in the UV−visible range and are n-type semiconductors. These properties, together with a valence band maximum position located favorably relative to the water oxidation potential, make them viable photoanode candidates. Using chopped linear sweep voltammetry, we indeed confirm that our Zr2ON2 films are PEC-active for the oxygen evolution reaction in alkaline electrolytes. We further show that high-vacuum annealing boosts their PEC performance characteristics. Although the observed photocurrents are low compared to state-of-the-art photoanodes, these dense and planar thin films can offer a valuable platform for studying oxynitride photoelectrodes, as well as for future nanostructuring, band gap engineering, and defect engineering efforts.

2,7-Diazapyrenes are promising azaaromatic scaffolds with a unique structural geometry and supramolecular properties. This core moiety and its derivatives with some N-methyl cations like N-methyl-2,7,-diazapyrenium, and N,N′-dimethyl-2,7-diazapyrenium attract special attention due to their challenging photophysical properties, especially in the context of interactions with DNA and some of its mononucleotides. This review focuses on the analysis of the main synthetic approaches to 2,7-diazapyrene and its functional derivatives employing various strategies under different reaction conditions. The opportunities of applications of 2,7-diazapyrenes, including their remarkable photophysical and supramolecular properties, DNA-bindings, in sensors, molecular electronics, supramolecular systems, and related areas are also highlighted. © 2022 The Royal Society of Chemistry

A plethora of 6-(hetero)aryl C-C and C-N bonded tacrine analogues has been made accessible by employing palladium mediated (Suzuki-Miyaura, Heck, Sonogashira, Stille and Buchwald) cross-coupling reactions, starting from either halogenated or borylated residues. The successful use of Pd(dppf)Cl2·CH2Cl2 as a common catalytic system in realizing all these otherwise challenging transformations is the highlight of our optimized protocols. The analogues thus synthesized allow the available chemical space around the C-6 of this biologically relevant tacrine core to be explored. The in silico docking studies of the synthesized compounds were carried out against the acetylcholinesterase (AChE) enzyme. The hepatotoxicity studies of these compounds were done against complexes of CYP1A2 and CYP3A4 proteins with known inhibitors like 7,8-benzoflavone and ketoconazole, respectively. © 2022 The Royal Society of Chemistry.

Item does not contain fulltext, Tyrosinemia type 1 (TT1) and phenylketonuria (PKU) are both inborn errors of phenylalanine-tyrosine metabolism. Neurocognitive and behavioral outcomes have always featured in PKU research but received less attention in TT1 research. This study aimed to investigate and compare neurocognitive, behavioral, and social outcomes of treated TT1 and PKU patients. We included 33 TT1 patients (mean age 11.24 years; 16 male), 31 PKU patients (mean age 10.84; 14 male), and 58 age- and gender-matched healthy controls (mean age 10.82 years; 29 male). IQ (Wechsler-subtests), executive functioning (the Behavioral Rating Inventory of Executive Functioning), mental health (the Achenbach-scales), and social functioning (the Social Skills Rating System) were assessed. Results of TT1 patients, PKU patients, and healthy controls were compared using Kruskal-Wallis tests with post-hoc Mann-Whitney U tests. TT1 patients showed a lower IQ and poorer executive functioning, mental health, and social functioning compared to healthy controls and PKU patients. PKU patients did not differ from healthy controls regarding these outcome measures. Relatively poor outcomes for TT1 patients were particularly evident for verbal IQ, BRIEF dimensions "working memory", "plan and organize" and "monitor", ASEBA dimensions "social problems" and "attention problems", and for the SSRS "assertiveness" scale (all p values <0.001). To conclude, TT1 patients showed cognitive impairments on all domains studied, and appeared to be significantly more affected than PKU patients. More attention should be paid to investigating and monitoring neurocognitive outcome in TT1 and research should focus on explaining the underlying pathophysiological mechanism.

Contains fulltext : 286856.pdf (Publisher’s version ) (Open Access), Tyrosinemia type 1 (TT1) and phenylketonuria (PKU) are both inborn errors of phenylalanine-tyrosine metabolism. Neurocognitive and behavioral outcomes have always featured in PKU research but received less attention in TT1 research. This study aimed to investigate and compare neurocognitive, behavioral, and social outcomes of treated TT1 and PKU patients. We included 33 TT1 patients (mean age 11.24 years; 16 male), 31 PKU patients (mean age 10.84; 14 male), and 58 age- and gender-matched healthy controls (mean age 10.82 years; 29 male). IQ (Wechsler-subtests), executive functioning (the Behavioral Rating Inventory of Executive Functioning), mental health (the Achenbach-scales), and social functioning (the Social Skills Rating System) were assessed. Results of TT1 patients, PKU patients, and healthy controls were compared using Kruskal-Wallis tests with post-hoc Mann-Whitney U tests. TT1 patients showed a lower IQ and poorer executive functioning, mental health, and social functioning compared to healthy controls and PKU patients. PKU patients did not differ from healthy controls regarding these outcome measures. Relatively poor outcomes for TT1 patients were particularly evident for verbal IQ, BRIEF dimensions "working memory", "plan and organize" and "monitor", ASEBA dimensions "social problems" and "attention problems", and for the SSRS "assertiveness" scale (all p values <0.001). To conclude, TT1 patients showed cognitive impairments on all domains studied, and appeared to be significantly more affected than PKU patients. More attention should be paid to investigating and monitoring neurocognitive outcome in TT1 and research should focus on explaining the underlying pathophysiological mechanism.

This paper reviews the second NTIRE challenge on image dehazing (restoration of rich details in hazy image) with focus on proposed solutions and results. The training data consists from 55 hazy images (with dense haze generated in an indoor or outdoor environment) and their corresponding ground truth (haze-free) images of the same scene. The dense haze has been produced using a professional haze/fog generator that imitates the real conditions of haze scenes. The evaluation consists from the comparison of the dehazed images with the ground truth images. The dehazing process was learnable through provided pairs of haze-free and hazy train images. There were ~ 270 registered participants and 23 teams competed in the final testing phase. They gauge the state-of-the-art in image dehazing.

Computer vision (CV) algorithms are widely utilized in imaging processing for medical and personal electronics applications. In sensorics CV can provide a great potential to quantitate chemosensors' signals. Here we wish to describe a method for the CV-assisted spectrofluorometer-free detection of common nitro-explosive components,e.g.2,4-dinitrotoluene (DNT) and 2,4,6-trinitrotoluene (TNT), by using polyaromatic hydrocarbon (PAH, PAH = 1-pyrenyl or 9-anthracenyl) - basedbola-type chemosensors. The PAH components of these chemicalbolasare able to form stable, bright emissive in a visual wavelength region excimers, which allows their use as extended matrices of the RGB colors after imaging and digital processing. In non-polar solvents, the excimers have poor chemosensing properties, while in aqueous solutions, due to the possible micellar formation, these excimers provide “turn-off” fluorescence detection of DNT and TNT in the sub-nanomolar concentrations. A combination of these PAH-based fluorescent chemosensors with the proposed CV-assisted algorithm offers a fast and convenient approach for on-site, real-time, multi-thread analyte detection without the use of fluorometers. Although we focus on the analysis of nitro-explosives, the presented method is a conceptual work describing a general use of CV for quantitative fluorescence detection of various analytes as a simpler alternative to spectrofluorometer-assisted methods. © The Royal Society of Chemistry 2021.

A convenient synthetic approach to meso-substituted with 2,2’-bipyridine and 1-(pyridin-2‑yl)isoquinoline residues calix[4]arenes is reported. This approach involves the reaction of generated in situ 2‑lithio-calix[4]arene with 1,2,4‑triazine precursor with the following aromatization of the obtained adduct, and the aza-Diels- Alder reaction of the 1,2,4‑triazinyl-substituted calix[4]arene with 2,5‑norbornadien or in-situ generated 1,2‑dehydrobenzene. The UV/fluorescence response of thus obtained meso-pyridyl-substituted calix[4]arenes to metal cations is studied.

The derivatives of polycyclic aromatic hydrocarbon (PAH) display unique properties of fluorescence like excellent photostability, 1 lifetime of long fluorescence and including high quantum yield. Polycyclic aromatic hydrocarbon (PAH) could be used for organic light emitting iodes, biological fluorescent probe, press-sensitive materials etc.2 In the polymer materials area, here PAH is stablished into the polymer to label macromolecular chains, modify carbon nanotubes3 or improve polymer fluorescence as well. In this work we are glad to inform an efficient approach for the synthesis of few PAH (such as pyrene, anthracene and naphthalene) containing copolymers based on acrylic acid. First, for the synthesis of starting vinyl PAH, we have done vinylation of the PAH. Therefore, with respect to the starting vinyl PAH we used various proportion of acrylic acid.

The chemistry of hypervalent iodine(III) reagents have widely been explored in many organic transformations for the construction of C-C as well as C-N bonds especially for their environmentally benign behavior, low toxicity and milder reaction conditions. The synthetic usefulness of such reagents has been experienced in many ring transformations as well as arylation reactions due to their excellent yield of the products and selectivities.

During last two decades the mechnochemical synthesis became good alternative to the traditional organic synthesis. For the polymer chemistry mechanochemical polymerization is constituted as sustainable alternative to the solution polymerization.

Transition metal catalyzed carbonylation reactions have become most sought-after technology for the utilization of toxic gases such as carbon monoxide and carbon dioxide. These gases are important C1 building block for the development of industrial core technology through the conversion of bulk and fine chemicals into useful compounds.[1] However, the direct use of these gases has been restricted because of their inherent toxicity, need of sophisticated reactors and careful handling of such instruments as the ppm level of gas leakage may cause serious problems. The introduction of solid COsurrogates therefore highly desirable for the carbonylative transformation which must be safe to handle, stable at ambient temperature and cost effective.

An efficient synthetic approach towards 5‑arylethynyl-1,2,4‑triazines via direct C-H-functionalization of 5‑H-1,2,4‑triazines in reaction with lithium acetylenes is reported.

A simple approach for the synthesis of dihydropyrimidinones from aldehydes with β-ketoester and urea using BAILs as a catalyst at room temperature and solvent-free condition. This method shows high yields and simple separation techniques. © 2020 Author(s).

A simple, efficient, and green method for the one-pot synthesis of 4(3H)-quinazolinones by condensation of anthranilic acid, orthoesters, and amines in the presence of Brønsted Acidic ionic liquid has been developed. the advantages of the present protocol are this occurred at room temperature, under solvent-free conditions to afford the products in excellent yields, easy workup. © 2020 Author(s).

OBJECTIVE: To directly compare the efficacy of natalizumab and fingolimod in patients with active relapsing-remitting multiple sclerosis. METHODS: This phase 4, randomised, rater- and sponsor-blinded, prospective, parallel-group, clinic-based head-to-head study was conducted at 43 sites in nine countries. Patients were randomised (1:1) to intravenous natalizumab 300 mg every 4 weeks or oral fingolimod 0.5 mg once daily for ≤52 weeks. Enrolment-related early study termination precluded assessment of the primary endpoint (evolution of new on-treatment gadolinium-enhancing (Gd+) lesions to persistent black holes). Unplanned exploratory analyses of secondary endpoints evaluated the effects of treatment on the development of new T1 Gd+ lesions and new/newly enlarging T2 lesions, lesion volumes and relapse outcomes. RESULTS: The intent-to-treat population comprised 108 patients (natalizumab, n=54; fingolimod, n=54); 63 completed ≥24 weeks of treatment. Due to the limited numbers of events and patients at risk, MRI and relapse outcomes were reported over up to 24 and 36 weeks, respectively. The mean number of new T1 Gd+ lesions was numerically lower with natalizumab than with fingolimod by 4 weeks; accumulation rates were 0.02 and 0.09 per week, respectively, over 24 weeks (p=0.004). The cumulative probability of developing ≥1 lesion at 24 weeks was 40.7% with natalizumab versus 58.0% with fingolimod (HR=0.60; 95% CI 0.31-1.16; p=0.126); the corresponding probabilities for ≥2 lesions were 11.5% vs 48.5% (HR=0.25; 95% CI 0.09-0.68; p=0.007). No significant between-group differences were observed for the other MRI outcomes at 24 weeks. The cumulative probability of relapse over follow-up was 1.9% with natalizumab versus 22.3% with fingolimod (HR=0.08; 95% CI 0.01-0.64; p=0.017). Adverse events were consistent with known safety profiles. CONCLUSIONS: These results suggest that natalizumab is more efficacious than fingolimod in reducing multiple sclerosis relapses and T1 Gd+ l

5-Aryl-2,2′-bipyridine-6′-carboxylic acids with an extended conjugation system were prepared as ligands for lanthanide cations. © 2019 Author(s).

Beta-N-tosyloxazolidin-2-yl-substituted 2,2′-bipyridines and 2-((2,2′-bipyridin-5-yl)methylamino)ethanol were prepared and their reactivity was studied. © 2019 Author(s).

3-amino-5-(1H-indol-3-yl)-6-phenylpyrazine-2-carboxylic acid amides were obtained by the Taylor method, the SN H methodology, and further obtaining the amides through the corresponding acid chlorides. It is shown the formation of the unexpected product while using DCC. © 2019 Author(s).

The knowledge of the mechanism involved in the process of adsorption and desorption of arsenate and arsenite with ferric hydroxides is important to address the water toxicity problems and to tackle the adverse effect of these substances in nature. An essential outcome of previous studies on the kinetics of the arsenate adsorption on aluminum and iron oxide was that the adsorption is a two-phase (bi-phase) process. Quantum mechanical calculations using density functional theory were used to determine the thermodynamic variables governing the adsorption process to get an insight into the stability of the complexes formed. The previous investigation showed that the positively charged ferric hydroxide cluster had better stability at neutral pH. The chemisorbed charged monodentate complexes had Gibbs free energy of reaction –55.97 kcal/mol where the bidentate complex formation had Gibbs free energy of reaction –62.55 kcal/mol. The bidentate complex having a negative charge had more Gibbs free energy of reaction compared to uncharged one. The results of the study indicate that Gibbs free energy for the reaction has a significant role in controlling the kinetics of the adsorption and sorption process of arsenate on ferric hydroxide clusters.

A transition metal-free process has been reported for 1,2-dithiocyanation of alkynes in the presence of sodium persulfate and potassium thiocyanate reagent combination in a short reaction time under ambient air. Styrene derivatives are equally applicable under the same reaction conditions. Monothiocyanated vinyl derivatives were also synthesized from 2-ethynylpyridine and dimethyl acetylene dicarboxylate. The reaction proceeds by the radical/polar pathway as evidenced from our experiments and literature. After removal of the solvent from the reaction mixture by evaporation, the crude product was purified without conventional workup. © 2018 American Chemical Society.

New tripod-type 2,2′-bipyridine ligand consisting of a central polyaminocarboxylic moiety for the coordination to lanthanide cations and three appended 5-phenyl-2,2′-bipyridine fragments for the coordination to various transition metal cations have been prepared. A europium complex of this ligand was prepared, and its photophysical properties and a luminescent response towards transition metal salts (particularly, CdI2, Cd(OAc)2, Zn(ClO4)2, Cu(OAc)2, and HgCl2) have been studied. Europium cation luminescence quenching in the presence of transition metal salts in solution was observed in all cases. In addition, it was observed that the fluorescent response of the europium complex was quite individual depending on the type of the metal salt. The obtained data were compared with the earlier published data for some lanthanide complexes bearing additional sites for the chelation of transition metal cations. © 2018 Published by NRC Research Press.

Synthesis of oxazolidines has been reported from aziridines by the reaction with formic acid and formaldehyde in neat conditions. We have also observed a regio-selective nucleophilic ring opening of aziridines by HCOOH under same conditions in absence of formaldehyde where the HCOO− ion acts as a nucleophile. In this present method formic acid is not acting as reducing agent as classical organic reactions. The formic acid initially activates the aziridines whereas the formate group generated from formic acid acts as a nucleophile itself instead of the source of hydride ion affording the nucleophilic ring opening products. The synthesized formate ester was successfully hydrolyzed to the corresponding hydroxyl compound which is the key building block for the synthesis of heterocycles. Operational simplicity, mild and neat reaction conditions, broad substrates scope, good yields and uses of less expensive reagents are the notable advantages of the present procedures. © 2018 Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim

We report the first observation of the parity-violating 2.2 MeV gamma-ray asymmetry $A^{np}_\gamma$ in neutron-proton capture using polarized cold neutrons incident on a liquid parahydrogen target at the Spallation Neutron Source at Oak Ridge National Laboratory. $A^{np}_\gamma$ isolates the $\Delta I=1$, \mbox{$^{3}S_{1}\rightarrow {^{3}P_{1}}$} component of the weak nucleon-nucleon interaction, which is dominated by pion exchange and can be directly related to a single coupling constant in either the DDH meson exchange model or pionless EFT. We measured $A^{np}_\gamma = [-3.0 \pm 1.4 (stat) \pm 0.2 (sys)]\times 10^{-8}$, which implies a DDH weak $\pi NN$ coupling of $h_{\pi}^{1} = [2.6 \pm 1.2(stat) \pm 0.2(sys)] \times 10^{-7}$ and a pionless EFT constant of $C^{^{3}S_{1}\rightarrow ^{3}P_{1}}/C_{0}=[-7.4 \pm 3.5 (stat) \pm 0.5 (sys)] \times 10^{-11}$ MeV$^{-1}$. We describe the experiment, data analysis, systematic uncertainties, and the implications of the result., Comment: 6 pages, 5 figures

We report the first observation of the parity-violating 2.2 MeV gamma-ray asymmetry $A^{np}_\gamma$ in neutron-proton capture using polarized cold neutrons incident on a liquid parahydrogen target at the Spallation Neutron Source at Oak Ridge National Laboratory. $A^{np}_\gamma$ isolates the $\Delta I=1$, \mbox{$^{3}S_{1}\rightarrow {^{3}P_{1}}$} component of the weak nucleon-nucleon interaction, which is dominated by pion exchange and can be directly related to a single coupling constant in either the DDH meson exchange model or pionless EFT. We measured $A^{np}_\gamma = [-3.0 \pm 1.4 (stat) \pm 0.2 (sys)]\times 10^{-8}$, which implies a DDH weak $\pi NN$ coupling of $h_{\pi}^{1} = [2.6 \pm 1.2(stat) \pm 0.2(sys)] \times 10^{-7}$ and a pionless EFT constant of $C^{^{3}S_{1}\rightarrow ^{3}P_{1}}/C_{0}=[-7.4 \pm 3.5 (stat) \pm 0.5 (sys)] \times 10^{-11}$ MeV$^{-1}$. We describe the experiment, data analysis, systematic uncertainties, and the implications of the result., Comment: 6 pages, 5 figures

We have developed a new synthetic method for the synthesis of aziridines using Chloramine-T as an effective reagent in the presence of NH2OH center dot HCl and NaIO4. We found that the same combination of NH2OH center dot HCl and NaIO4 is also very effective for nucleophilic ring opening of aziridines.

We have developed a new synthetic method for the synthesis of aziridines using Chloramine-T as an effective reagent in the presence of NH 2OH·HCl and NaIO 4. We found that the same combination of NH 2OH·HCl and NaIO 4is also very effective for nucleophilic ring opening of aziridines. Copyright © 2017 Georg Thieme Verlag Stuttgart · New York.

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In this paper, comparison between G-N model of type II (without energy dissipation) and G-N model of type III (with energy dissipation) has been shown in a three dimensional thermoelastic half space with rotation subjected to time dependent heat source on the traction free boundary. Eigenvalue methodology has been adopted to solve the equations resulting from the application of the Normal mode analysis to the non-dimensional coupled equations. Variation of the numerically computed values of thermal stresses and temperature with and without rotation has been illustrated graphically.

Contains fulltext : 169680.pdf (publisher's version ) (Open Access), Cystathionine beta-synthase (CBS) deficiency is a rare inherited disorder in the methionine catabolic pathway, in which the impaired synthesis of cystathionine leads to accumulation of homocysteine. Patients can present to many different specialists and diagnosis is often delayed. Severely affected patients usually present in childhood with ectopia lentis, learning difficulties and skeletal abnormalities. These patients generally require treatment with a low-methionine diet and/or betaine. In contrast, mildly affected patients are likely to present as adults with thromboembolism and to respond to treatment with pyridoxine. In this article, we present recommendations for the diagnosis and management of CBS deficiency, based on a systematic review of the literature. Unfortunately, the quality of the evidence is poor, as it often is for rare diseases. We strongly recommend measuring the plasma total homocysteine concentrations in any patient whose clinical features suggest the diagnosis. Our recommendations may help to standardise testing for pyridoxine responsiveness. Current evidence suggests that patients are unlikely to develop complications if the plasma total homocysteine concentration is maintained below 120 mumol/L. Nevertheless, we recommend keeping the concentration below 100 mumol/L because levels fluctuate and the complications associated with high levels are so serious.

An efficient approach for the synthesis of 5,5″-or 5,6,5″-aryl substituted 2,2′:6′,2″-terpyridines, bearing an anneleted cyclopentene unit in one of the side-chain pyridine rings for the improved solubility in organic solvents, via their 1,2,4-triazine analogues has been developed. By using this approach, various aromatic substituents were introduced in the 2,2′:6′,2″-terpyridine core. Depending on the nature of the aromatic substituents, the obtained terpyridines exhibited an intense emission in a range of ca. 344-394 nm in acetonitrile solutions. For the most representative compounds, pronounced bathochromic shifts in both absorption and emission spectra were observed compared with previously reported substituted terpyridines. © 2017 Published by NRC Research Press.

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Contains fulltext : 169680.pdf (publisher's version ) (Open Access), Cystathionine beta-synthase (CBS) deficiency is a rare inherited disorder in the methionine catabolic pathway, in which the impaired synthesis of cystathionine leads to accumulation of homocysteine. Patients can present to many different specialists and diagnosis is often delayed. Severely affected patients usually present in childhood with ectopia lentis, learning difficulties and skeletal abnormalities. These patients generally require treatment with a low-methionine diet and/or betaine. In contrast, mildly affected patients are likely to present as adults with thromboembolism and to respond to treatment with pyridoxine. In this article, we present recommendations for the diagnosis and management of CBS deficiency, based on a systematic review of the literature. Unfortunately, the quality of the evidence is poor, as it often is for rare diseases. We strongly recommend measuring the plasma total homocysteine concentrations in any patient whose clinical features suggest the diagnosis. Our recommendations may help to standardise testing for pyridoxine responsiveness. Current evidence suggests that patients are unlikely to develop complications if the plasma total homocysteine concentration is maintained below 120 mumol/L. Nevertheless, we recommend keeping the concentration below 100 mumol/L because levels fluctuate and the complications associated with high levels are so serious.

Contains fulltext : 169680.pdf (publisher's version ) (Open Access), Cystathionine beta-synthase (CBS) deficiency is a rare inherited disorder in the methionine catabolic pathway, in which the impaired synthesis of cystathionine leads to accumulation of homocysteine. Patients can present to many different specialists and diagnosis is often delayed. Severely affected patients usually present in childhood with ectopia lentis, learning difficulties and skeletal abnormalities. These patients generally require treatment with a low-methionine diet and/or betaine. In contrast, mildly affected patients are likely to present as adults with thromboembolism and to respond to treatment with pyridoxine. In this article, we present recommendations for the diagnosis and management of CBS deficiency, based on a systematic review of the literature. Unfortunately, the quality of the evidence is poor, as it often is for rare diseases. We strongly recommend measuring the plasma total homocysteine concentrations in any patient whose clinical features suggest the diagnosis. Our recommendations may help to standardise testing for pyridoxine responsiveness. Current evidence suggests that patients are unlikely to develop complications if the plasma total homocysteine concentration is maintained below 120 mumol/L. Nevertheless, we recommend keeping the concentration below 100 mumol/L because levels fluctuate and the complications associated with high levels are so serious.

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In this paper, comparison between G-N model of type II (without energy dissipation) and G-N model of type III (with energy dissipation) has been shown in a three dimensional thermoelastic half space with rotation subjected to time dependent heat source on the traction free boundary. Eigenvalue methodology has been adopted to solve the equations resulting from the application of the Normal mode analysis to the non-dimensional coupled equations. Variation of the numerically computed values of thermal stresses and temperature with and without rotation has been illustrated graphically.

The synthesis of vicinal diiodo carbonyl compounds from α,β-unsaturated carbonyl compounds has been carried out for the first time using the combination of NH2OHHCl and NaIO4 under mild reaction conditions at room temperature. The present methodology is also applicable for the synthesis of vicinal diiodo derivatives of nitrostyrene. The remarkable advantages of the present protocol are room temperature reaction, easy operation, good yields, fast reaction, transition metal-free and neutral reaction conditions. The present methodology is applicable to gram scale synthesis. © 2016 Arkat. All rights reserved.

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