Central nervous system anomalies in 41 Chinese children incontinentia pigmenti.

INTRODUCTION: Incontinentia pigmenti (IP) is a rare neuroectodermal dysplasia caused by a defect in the IKBKG gene. The pathogenesis of central nervous system injury is believed to be related to microvascular ischemia. Currently, few treatment strategies are available for the inflammatory phase. MATERIALS AND METHODS: This retrospective descriptive analysis included the clinical data of 41 children with IP collected from 2007 to 2021 in Xi'an, China, comprising clinical characteristics, imaging findings, blood cell analysis, skin histopathology, and genetic data. RESULTS: Fourteen children (34%) aged 4 days to 5 months exhibited clinical signs and symptoms, including convulsions, delayed psychomotor development following neurological damage, and revealed significant MRI abnormalities, including ischemia, hypoxia, cerebral hypoperfusion, hemorrhage, encephalomalacia, and cerebral atrophy. Eight of the 24 patients (33%) presented with retinal vascular tortuosity and telangiectasis, accompanied by neovascularization and hemorrhage. Thirty-eight children (93%) had elevated eosinophils (mean: 3.63 ± 4.46 × 109), and 28 children (68%) had significantly elevated platelets (mean: 420.16 ± 179.43 × 109). Histopathology of skin revealed microvascular extravasation and vasodilation with perivascular and intravascular eosinophilic infiltration. CONCLUSION: Brain injury in IP occurs during infancy until 5 months of age, which is also the acute dermatitis phase accompanied by eosinophilia and an increased platelet count. This study provides evidence of microvascular damage to the skin and fundus during the inflammatory phase. The mechanism of microvascular damage may be similar to that in the brain.

Precursor-Directed Biosynthesis of Neoantimycin Derivatives with Selective Cytotoxicity.

A precursor-directed biosynthesis approach led to the accumulation of seven new neoantimycin derivatives (1-7) from Streptomyces conglobatus RJ2. Structure elucidation was conducted using NMR and HRESIMS analysis, and the absolute configuration was determined by advanced Marfey's method, Mosher's analysis, and ECD analysis. The obtained compounds revealed selective and significant cytotoxicity, specifically against colorectal cancer cells bearing the K-ras mutation, with IC50 values ranging from 40 nM to 3.5 µM.

Cooperative Copper Single-Atom Catalyst in 2D Carbon Nitride for Enhanced CO2 Electrolysis to Methane.

Renewable-electricity-powered carbon dioxide (CO2) reduction (eCO2R) to high-value fuels like methane (CH4) holds the potential to close the carbon cycle at meaningful scales. However, this kinetically staggered 8-electron multistep reduction suffers from inadequate catalytic efficiency and current density. Atomic Cu-structures can boost eCO2R-to-CH4 selectivity due to enhanced intermediate binding energies (BEs) resulting from favorably shifted d-band centers. In this work, 2D carbon nitride (CN) matrices, viz. Na-polyheptazine (PHI) and Li-polytriazine imides (PTI), are exploited to host Cu-N2 type single-atom sites with high density (≈1.5 at%), via a facile metal-ion exchange process. Optimized Cu loading in nanocrystalline Cu-PTI maximizes eCO2R-to-CH4 performance with Faradaic efficiency (FECH4) of ≈68% and a high partial current density of 348 mA cm-2 at -0.84 V vs reversible hydrogen electrode (RHE), surpassing the state-of-the-art catalysts. Multi-Cu substituted N-appended nanopores in the CN frameworks yield thermodynamically stable quasi-dual/triple sites with large interatomic distances dictated by the pore dimensions. First-principles calculations elucidate the relative Cu-CN cooperative effects between the matrices and how the Cu local environment dictates the adsorbate BEs, density of states, and CO2-to-CH4 energy profile landscape. The 9N pores in Cu-PTI yield cooperative Cu-Cu sites that synergistically enhance the kinetics of the rate-limiting steps in the eCO2R-to-CH4 pathway.

Combination of AS101 and Mefloquine Inhibits Carbapenem-Resistant Pseudomonas aeruginosa in vitro and in vivo.

Background: In recent years, carbapenem-resistant Pseudomonas aeruginosa (CRPA) has spread around the world, leading to a high mortality and close attention of medical community. In this study, we aim to find a new strategy of treatment for CRPA infections. Methods: Eight strains of CRPA were collected, and PCR detected the multi-locus sequence typing (MLST). The antimicrobial susceptibility test was conducted using the VITEK@2 compact system. The minimum inhibitory concentration (MIC) for AS101 and mefloquine was determined using the broth dilution method. Antibacterial activity was tested in vitro and in vivo through the chessboard assay, time killing assay, and a mouse model. The mechanism of AS101 combined with mefloquine against CRPA was assessed through the biofilm formation inhibition assay, electron microscopy, and detection of reactive oxygen species (ROS). Results: The results demonstrated that all tested CRPA strains exhibited multidrug resistance. Moreover, our investigation revealed a substantial synergistic antibacterial effect of AS101-mefloquine in vitro. The assay for inhibiting biofilm formation indicated that AS101-mefloquine effectively suppressed the biofilm formation of CRPA-5 and CRPA-6. Furthermore, AS101-mefloquine were observed to disrupt the bacterial cell wall and enhance the permeability of the cell membrane. This effect was achieved by stimulating the production of ROS, which in turn hindered the growth of CRPA-3. To evaluate the therapeutic potential, a murine model of CRPA-3 peritoneal infection was established. Notably, AS101-mefloquine administration resulted in a significant reduction in bacterial load within the liver, kidney, and spleen of mice after 72 hours of treatment. Conclusion: The present study showed that the combination of AS101 and mefloquine yielded a notable synergistic bacteriostatic effect both in vitro and in vivo, suggesting a potential clinical application of this combination in the treatment of CRPA.

An Investigation Into Whether the Facial Nerve and Auditory Nerve can be Protected by Removal of the Posterior Wall of the Internal Auditory Canal Under 30° Neuroendoscopy During Vestibular Schwannoma Surgery.

The aim of this study was to evaluate the surgical technique of microresection of vestibular schwannoma by removing the posterior wall of the internal auditory canal (IAC) under neuroelectrophysiological monitoring and 30° neuroendoscopy, with respect to the protection of facial and auditory nerve function. Forty-five cases of microscopic resection of auditory neuromas were performed through a posterior approach to the inferior occipital sigmoid sinus using a 30° neuroendoscope to assist in the removal of the posterior wall of the IAC during surgery. Patients underwent cranial enhancement magnetic resonance imaging examination and functional assessment of the facial and auditory nerves before and after surgery, and clinical data were collected for retrospective analysis. All tumors were removed in 41 patients, and most of the tumors were removed in 4 patients. The facial nerve was anatomically preserved in 43 patients (95.6%), and the percentage of facial nerve function preservation (House-Brackmann grade I-II) was 84.4%. Forty patients (88.9%) had anatomical preservation of the auditory nerve, with a 66.7% functional preservation rate. At 3 to 39 months of follow-up, 45 patients were reviewed with 3.0 T-enhanced magnetic resonance imaging, and no tumor recurrence was observed in any of the patients. Microscopic resection of auditory neuroma through the posterior approach of the inferior occipital sigmoid sinus with intraoperative use of 30° neuroendoscopic assistance to abrade the posterior wall of the IAC can eliminate dead space in certain anatomical areas during surgery and minimize surgical damage to the facial and auditory nerves, which is the basis for preservation of facial and auditory nerve function.

18-Residue Peptaibols Produced by the Sponge-Derived Trichoderma sp. GXIMD 01001.

Seven new 18-residue peptaibols, trichorzins A-G (1-7), were isolated from the sponge-derived fungus Trichoderma sp. GXIMD 01001. Their structures and configurations were characterized by a comprehensive interpretation of the NMR spectroscopic data, MS/MS fragmentation, Marfey's method, and ECD analysis. The general sequences of trichorzins A-G are as follows: Ac-Aib1-Ala/Ser2-Ala3-Aib/Iva4-Iva5-Gln6-Aib/Iva7-Val/allo-Ile8-Aib9-Gly10-Leu11-Aib12-Pro13-Leu14-Aib15-Aib16-Gln17-Trpol/Pheol18. The obtained compounds were assessed for their potential antiproliferative and antimicrobial activities. All obtained compounds did not show potent antibacterial activity but exhibited significant cytotoxicity, with the lowest IC50 values at 0.46-4.7 µM against four human carcinoma cell lines.

Multi-Objective Optimization of Sugarcane Milling System Operations Based on a Deep Data-Driven Model.

The extraction of sugarcane juice is the first step of sugar production. The optimal values of process indicators and the set values of operating parameters in this process are still determined by workers' experience, preventing adaptive adjustment of the production process. To address this issue, a multi-objective optimization framework based on a deep data-driven model is proposed to optimize the operation of sugarcane milling systems. First, the sugarcane milling process is abstracted as the interaction of material flow, energy flow, and information flow (MF-EF-IF) by introducing synergetic theory, and each flow's order parameters and state parameters are obtained. Subsequently, the state parameters of the subsystems are taken as inputs, and the order parameters-including the grinding capacity, electric consumption per ton of sugarcane, and sucrose extraction-are produced as outputs. A collaborative optimization model of the MF-EF-IF of the milling system is established by using a deep kernel extreme learning machine (DK-ELM). The established milling system model is applied for an improved multi-objective chicken swarm optimization (IMOCSO) algorithm to obtain the optimal values of the order parameters. Finally, the milling process is described as a Markov decision process (MDP) with the optimal values of the order parameters as the control objectives, and an improved deep deterministic policy gradient (DDPG) algorithm is employed to achieve the adaptive optimization of the operating parameters under different working conditions of the milling system. Computational experiments indicate that enhanced performance is achieved, with an increase of 3.2 t per hour in grinding capacity, a reduction of 660 W per ton in sugarcane electric consumption, and an increase of 0.03% in the sucrose extraction.

Design of an ultra-broadband optical filter based on a local micro-structured long period fiber grating near PMTP.

This paper presents a local micro-structured long period fiber grating (LMS-LPFG) ultra-broadband optical filter based on the wide bandwidth near the phase-matching turning point (PMTP). The structure of LMS-LPFG is obtained by dividing a LPFG into two parts of equal length and reducing the cladding radius of the second LPFG. At this time, the LMS-LPFG can be regarded as a cascade of two equal-length LPFGs with different resonance wavelengths. The cladding mode and grating period are determined to make the first LPFG work in the double-peak resonance state, and the second LPFG operates near PMTP. It is found that the transmission spectra of the two LPFGs can be superimposed to form a wide loss bandwidth. Then the cladding radii of the second LPFG and grating structure parameters are designed based on coupled-mode theory. First, the grating period corresponding to the operating wavelength is determined from the phase-matching curve of LMS-LPFG. Then, the radius of the second LPFG with a designated grating period is selected to make LPFG 2 work in PMTP by reducing its cladding radius. In addition, the grating lengths of the two LPFGs are determined by maximizing the loss of the LMS-LPFG's transmission spectrum. Finally, the two LPFGs are cascaded into a LMS-LPFG, and the optical transmission spectrum of the LMS-LPFG is calculated by the transfer matrix method. Simulation results show that the bandwidth of the transmission spectrum can reach 380 nm. In addition, the flexibility of design for the operating wave band is discussed and confirmed, and can meet different actual requirements of optical communication.

Grain Boundary-Derived Cu+ /Cu0 Interfaces in CuO Nanosheets for Low Overpotential Carbon Dioxide Electroreduction to Ethylene.

Electrochemical CO2 reduction reaction can be used to produce value-added hydrocarbon fuels and chemicals by coupling with clean electrical energy. However, highly active, selective, and energy-efficient CO2 conversion to multicarbon hydrocarbons, such as C2 H4 , remains challenging because of the lack of efficient catalysts. Herein, an ultrasonication-assisted electrodeposition strategy to synthesize CuO nanosheets for low-overpotential CO2 electroreduction to C2 H4 is reported. A high C2 H4 Faradaic efficiency of 62.5% is achieved over the CuO nanosheets at a small potential of -0.52 V versus a reversible hydrogen electrode, corresponding to a record high half-cell cathodic energy efficiency of 41%. The selectivity toward C2 H4 is maintained for over 60 h of continuous operation. The CuO nanosheets are prone to in situ restructuring during CO2 reduction, forming abundant grain boundaries (GBs). Stable Cu+ /Cu0 interfaces are derived from the low-coordinated Cu atoms in the reconstructed GB regions and act as highly active sites for CO2 reduction at low overpotentials. In situ Raman spectroscopic analysis and density functional theory computation reveal that the Cu+ /Cu0 interfaces offer high *CO surface coverage and lower the activation energy barrier for *CO dimerization, which, in synergy, facilitates CO2 reduction to C2 H4 at low overpotentials.

Impact of Cluster Nursing on Nursing on VAS Score and Urinary Function of Patients after Percutaneous Nephrolithotomy with Pneumatic Lithotripsy (PCNL).

Objective: To explore the effects of cluster nursing on VAS score and urinary system function of patients after percutaneous nephrolithotomy with pneumatic lithotripsy (PCNL). Methods: November 2019-January 2019, 114 patients with PCNL who received treatment in our hospital were selected and randomly divided into two groups: the control group and the study group. The study group received cluster nursing, and the control group received routine nursing. Compare the Barthel index (BI), between two groups, SAS score, complications rate, surgical outcomes, pain scores, quality of life scores, knowledge awareness rate, and satisfaction rate were compared. Results: There were no significant variations in Bi and SAS scores before the nursing (P > 0.05). The BI ratings were clearly higher after nursing, whereas the SAS and pain levels were considerably lower, however, the research group altered more dramatically (P0.05). The study group's incidence of complications was lower (P0.05) than the control group's; the study group's hospitalization cost was lower, and the hospitalization and lower bed activity were shorter (P0.05); after the nursing, the organised quality score was significantly higher in both groups, but the research group changed more dramatically (P0.05). Compared with the control group, the knowledge of the research team was higher (P < 0.05); after the nursing, both group satisfaction scores were obviously high, but the study group changes more significantly (P < 0.05). Conclusion: After PCNL treatment, the patient receives cluster therapy, improving the patient's anxiety, reducing the degree of pain, and improving the quality of life in patients, and the patient satisfaction is high. Therefore, cluster nursing is worthy of extensive application in the postoperative care of patients with PCNL.

Amine-Functionalized Carbon Nanodot Electrocatalysts Converting Carbon Dioxide to Methane.

The electrochemical conversion of carbon dioxide (CO2 ) to methane (CH4 ), which can be used not only as fuel but also as a hydrogen carrier, has drawn great attention for use in supporting carbon capture and utilization. The design of active and selective electrocatalysts with exceptional CO2 -to-CH4 conversion efficiency is highly desirable; however, it remains a challenge. Here a molecular tuning strategy-in situ amine functionalization of nitrogen-doped graphene quantum dots (GQDs) for highly efficient CO2 -to-CH4 conversion is presented. Amine functionalized nitrogen-doped GQDs achieve a CH4 Faradic efficiency (FE) of 63% and 46%, respectively, at CH4 partial current densities of 170 and 258 mA cm-2 , approximating to or even outperforming state-of-the-art Cu-based electrocatalysts. These GQDs also convert CO2 to C2 products mainly including C2 H4 and C2 H5 OH with a maximum FE of ≈10%. A systematic analysis reveals that the CH4 yield varies linearly with amine group content, whereas the C2 production rate is positively dependent on pyridinic N dopant content. This work provides insight into the rational design of carbon catalysts with CO2 -to-CH4 conversion efficiency at the industrially relevant level.

Regulation of functional groups on graphene quantum dots directs selective CO2 to CH4 conversion.

A catalyst system with dedicated selectivity toward a single hydrocarbon or oxygenate product is essential to enable the industrial application of electrochemical conversion of CO2 to high-value chemicals. Cu is the only known metal catalyst that can convert CO2 to high-order hydrocarbons and oxygenates. However, the Cu-based catalysts suffer from diverse selectivity. Here, we report that the functionalized graphene quantum dots can direct CO2 to CH4 conversion with simultaneous high selectivity and production rate. The electron-donating groups facilitate the yield of CH4 from CO2 electro-reduction while electron-withdrawing groups suppress CO2 electro-reduction. The yield of CH4 on electron-donating group functionalized graphene quantum dots is positively correlated to the electron-donating ability and content of electron-donating group. The graphene quantum dots functionalized by either -OH or -NH2 functional group could achieve Faradaic efficiency of 70.0% for CH4 at -200 mA cm-2 partial current density of CH4. The superior yield of CH4 on electron-donating group- over the electron-withdrawing group-functionalized graphene quantum dots possibly originates from the maintenance of higher charge density of potential active sites (neighboring C or N) and the interaction between the electron-donating group and key intermediates. This work provides insight into the design of active carbon catalysts at the molecular scale for the CO2 electro-reduction.

Reconstructing two-dimensional defects in CuO nanowires for efficient CO2 electroreduction to ethylene.

Here we report that in situ reconstructed Cu two-dimensional (2D) defects in CuO nanowires during CO2RR lead to significantly enhanced activity and selectivity of C2H4 compared to the CuO nanoplatelets. Specifically, the CuO nanowires achieve high faradaic efficiency of 62% for C2H4 and a partial current density of 324 mA cm-2 yet at a low potential of -0.56 V versus a reversible hydrogen electrode. Structural evolution characterization and in situ Raman spectra reveal that the high yield of C2H4 on CuO nanowires is attributed to the in situ reduction of CuO to Cu followed by structural reconstruction to form 2D defects, e.g., stacking faults and twin boundaries, which improve the CO production rate and *CO adsorption strength. This finding may provide a paradigm for the rational design of nanostructured catalysts for efficient CO2 electroreduction to C2H4.

Unravelling the Role of Strong Metal-Support Interactions in Boosting the Activity toward Hydrogen Evolution Reaction on Ir Nanoparticle/N-Doped Carbon Nanosheet Catalysts.

Pt-based catalysts are commercial electrocatalysts for the hydrogen evolution reaction (HER), but their shortcomings of expensive and imperfect efficiency hinder their large-scale application. Here, we report an Ir-based HER catalyst supported by N-doped carbon nanosheets (Ir-NCNSs). The NCNSs, with a high surface area and unique atomic composition, enable Ir nanoparticles (NPs) to disperse at 2-3 nm and strongly coordinate to the Ir through Ir-N bonds, which exposes many active sites and strengthens their durability. The catalyst displays a low overpotential and a small Tafel slope of 46.3 mV at 10 mA cm-2 and 52 mV dec-1 in 0.5 M H2SO4, respectively. When used in 1.0 M KOH, Ir-NCNSs also show excellent electrocatalytic activity with a low overpotential of 125 mV at 10 mA cm-2. The calculated results further suggest that Ir NPs and NCNSs have excellent selectivity for strong metal-support interactions, corresponding to a significant and stable HER characteristic. Our findings provide insight into the design of high-efficiency Ir-based HER catalysts.

High-Temperature Oxidation Properties and Microstructural Evolution of Nanostructure Fe-Cr-Al ODS Alloys.

The oxidation behavior and microstructural evolution of the nanostructure of Fe-Cr-Al oxide dispersion strengthened (ODS) alloys prepared by spark plasma sintering were investigated by high-temperature oxidation experiments in air at 1200 °C for 100 h. The formation of Al2O3 scale was observed by X-ray diffraction (XRD) and scanning electron microscopy (SEM) with energy-dispersive X-ray spectroscopy (EDS) line scans. The oxidation rate of Fe-Cr-Al ODS alloys is lower than that of conventional Fe-Cr-Al alloys, and the oxide layer formed on the Fe-Cr-Al alloy appeared loose and cracked, whereas the oxide layer formed on the Fe-Cr-Al ODS alloys was adherent and flat. This is due to the high density of dispersed nano-oxides hindering the diffusion of Al element and the formation of vacancies caused by them. In addition, the nano-oxides could also adhere to the oxide layer. Besides, the microstructure of the Fe-Cr-Al ODS alloy had excellent stability during high-temperature oxidation.

Production of 3-desmethyl protostreptovaricin I from the genetically engineered Streptomyces spectabilis CCTCC M2017417.

A new streptovaricin analogue, namely 3-desmethyl protostreptovaricin I (1), was isolated from the culture of the genetically engineered strain ΔstvM2 derived from Streptomyces spectabilis CCTCC M2017417. Its structure was elucidated on the basis of extensive spectroscopic analyses, including 1D and 2D NMR tests, and high resolution mass spectrometry analysis. Compound 1 is the first example of 3-desmethyl streptovaricin analogues reported so far, however, it showed no antibacterial activities against Staphylococcus aureus ATCC 29213.

Uncovering the cytochrome P450-catalyzed methylenedioxy bridge formation in streptovaricins biosynthesis.

Streptovaricin C is a naphthalenic ansamycin antibiotic structurally similar to rifamycins with potential anti-MRSA bioactivities. However, the formation mechanism of the most fascinating and bioactivity-related methylenedioxy bridge (MDB) moiety in streptovaricins is unclear. Based on genetic and biochemical evidences, we herein clarify that the P450 enzyme StvP2 catalyzes the MDB formation in streptovaricins, with an atypical substrate inhibition kinetics. Furthermore, X-ray crystal structures in complex with substrate and structure-based mutagenesis reveal the intrinsic details of the enzymatic reaction. The mechanism of MDB formation is proposed to be an intramolecular nucleophilic substitution resulting from the hydroxylation by the heme core and the keto-enol tautomerization via a crucial catalytic triad (Asp89-His92-Arg72) in StvP2. In addition, in vitro reconstitution uncovers that C6-O-methylation and C4-O-acetylation of streptovaricins are necessary prerequisites for the MDB formation. This work provides insight for the MDB formation and adds evidence in support of the functional versatility of P450 enzymes.

Effect of Hot Rolling on the Microstructure and Properties of Nanostructured 15Cr ODS Alloys with Al and Zr Addition.

Oxide dispersion strengthened (ODS) alloys with Al and Zr addition have excellent radiation tolerance, high-temperature strength, and corrosion resistance. The 15Cr-Al-Zr-ODS alloys are processed by mechanical alloying (MA), hot isostatic pressing (HIP), subsequent hot rolling to large strains of 70%, and further annealing. The effect of hot rolling on the microstructure, and the properties of nanostructured 15Cr ODS alloys with Al and Zr addition, were investigated. The microstructure after hot rolling and annealing showed obvious anisotropy. The cubic texture (φ1 = 0°, Φ = 0°, φ2 = 0°) {0 0 1} <1 0 0> and brass-R texture (φ1 = 0°, Φ = 55°, φ2 = 45°) {1 1 1} <1 1 0> were observed. The similar size distribution of precipitates was obtained for the comparison of the hot rolling samples with the hot isostatic pressed samples, which can be attributed to excellent thermal stability. After hot rolling, the alloy showed higher yield strength but did not lose too much plasticity.

Ansavaricin J, a New Heterocyclic Ring-Fused Streptovaricin from Gene stvP5-Deleted Mutant of Streptomyces spectabilis CCTCC M2017417.

A new ring-fused streptovaricin analogue, named ansavaricin J, was unprecedently isolated from the culture of the genetically modified strains ΔstvP5 which derived from Streptomyces spectabilis CCTCC M2017417. Its structure was elucidated via comprehensive spectroscopic analyses, including 1D- and 2D-NMR tests, and HR-ESI-MS data analysis. Notably, ansavaricin J and E represent the only two reported examples of heterocyclic ring-fused streptovaricins thus far, however, it only showed insignificant antibacterial activities against Staphylococcus aureus.

Microstructure and Low-Cycle Fatigue Behavior of Al-9Si-4Cu-0.4Mg-0.3Sc Alloy with Different Casting States.

The low-cycle fatigue behavior of Al-9Si-4Cu-0.4Mg-0.3Sc alloy with different casting states was investigated by performing low-cycle fatigue tests and by means of observations and analysis with a scanning electron microscope (SEM) and a transmission electron microscope (TEM). It was found that the metal-mold cast and die-cast Al-9Si-4Cu-0.4Mg-0.3Sc alloys exhibited the cyclic stress response of strain hardening under all imposed total strain amplitudes. The cyclic deformation resistance and fatigue life of the metal-mold cast Al-9Si-4Cu-0.4Mg-0.3Sc alloy were lower than those of the die-cast Al-9Si-4Cu-0.4Mg-0.3Sc alloy. The plastic strain and elastic strain amplitudes of the metal-mold cast and die-cast Al-9Si-4Cu-0.4Mg-0.3Sc alloys were linearly related to the number of reversals to failure, which obeyed the Coffin-Manson and Basquin formulas, respectively. The results of TEM observation revealed that at all imposed total strain amplitudes, the cyclic deformation mechanisms of the metal-mold cast and die-cast Al-9Si-4Cu-0.4Mg-0.3Sc alloys were planar slip and wavy slip at the lower and higher strain amplitudes, respectively.