Clinical and laboratory profile and outcome in children with Wilson disease: an observational study in South India.

BACKGROUND: Wilson disease is an autosomal recessive disorder owing to defective copper metabolism which causes abnormal accumulation of copper and damage to the liver, brain, kidneys and other organs. AIM: To describe the clinical features, laboratory investigations and outcome of Wilson disease in children. METHODS: A retrospective observational study was conducted in the paediatric department of a tertiary- care hospital in South India by reviewing medical records between January 2018 and March 2023. The diagnosis of Wilson disease was confirmed by the presence of low serum ceruloplasmin and/or high urine copper excretion in combination with clinical and ophthalmological features. RESULTS: A total of 32 cases were analysed. The mean (SD) age at presentation was 110 (36) months with a M:F ratio of 1.6:1. Isolated hepatic involvement was seen in 19 (60%) patients while 13 (40%) patients had a neurological presentation, either as an isolated entity or in combination with hepatic manifestations. Low serum ceruloplasmin levels were detected in 31 (96%) patients. Urine copper levels were elevated in all patients. Twenty-one patients were commenced on D penicillamine while 11 patients were treated with a combination chelation therapy with zinc. Eighteen patients (56%) were on regular follow-up. CONCLUSION: The clinical presentation of Wilson disease in children is diverse, varying from the more common hepatic or neurological manifestations to the less common atypical forms of the disease. Diagnosis is based on clinical and ophthalmological features in combination with biochemical abnormalities in the form of low ceruloplasmin and high urinary copper. The majority of patients can be medically managed with chelation therapy.

Recent developments in two-dimensional molybdenum disulfide-based multimodal cancer theranostics.

Recent advancements in cancer research have led to the generation of innovative nanomaterials for improved diagnostic and therapeutic strategies. Despite the proven potential of two-dimensional (2D) molybdenum disulfide (MoS2) as a versatile platform in biomedical applications, few review articles have focused on MoS2-based platforms for cancer theranostics. This review aims to fill this gap by providing a comprehensive overview of the latest developments in 2D MoS2 cancer theranostics and emerging strategies in this field. This review highlights the potential applications of 2D MoS2 in single-model imaging and therapy, including fluorescence imaging, photoacoustic imaging, photothermal therapy, and catalytic therapy. This review further classifies the potential of 2D MoS2 in multimodal imaging for diagnostic and synergistic theranostic platforms. In particular, this review underscores the progress of 2D MoS2 as an integrated drug delivery system, covering a broad spectrum of therapeutic strategies from chemotherapy and gene therapy to immunotherapy and photodynamic therapy. Finally, this review discusses the current challenges and future perspectives in meeting the diverse demands of advanced cancer diagnostic and theranostic applications.

Low defect density in MoS2 monolayers grown on Au(111) by metal-organic chemical vapor deposition.

Monolayers of transition metal dichalcogenides (TMDs) possess high potential for applications in novel electronic and optoelectronic devices and therefore the development of methods for their scalable growth is of high importance. Among different suggested approaches, metal-organic chemical vapor deposition (MOCVD) is the most promising one for technological applications because of its lower growth temperature compared to the most other methods, e.g., conventional chemical vapor or atomic layer deposition (CVD, ALD). Here we demonstrate for the first time the epitaxial growth of MoS2 monolayers on Au(111) by MOCVD at 450 °C. We confirm the high quality of the grown TMD monolayers down to the atomic scale using several complementary methods. These include Raman spectroscopy, non-contact atomic force microscopy (nc-AFM), X-ray photoelectron spectroscopy and scanning tunneling microscopy (STM). The topographic corrugation of the MoS2 monolayer on Au(111), revealed in a moiré structure, was measured as ≈20 pm by nc-AFM. The estimated defect density calculated from STM images of the as-grown MoS2 monolayers is in the order of 1012 vacancies/cm2. The defects are mainly caused by single sulfur vacancies. Our approach is a step forward towards the technologically relevant growth of high-quality, large-area TMD monolayers.

Few-cycle laser pulse characterization on-target using high-harmonic generation from nano-scale solids.

We demonstrate high-harmonic generation for the time-domain observation of the electric field (HHG-TOE) and use it to measure the waveform of ultrashort mid-infrared (MIR) laser pulses interacting with ZnO thin-films or WS2 monolayers. The working principle relies on perturbing HHG in solids with a weak replica of the pump pulse. We measure the duration of few-cycle pulses at 3200 nm, in reasonable agreement with the results of established pulse characterization techniques. Our method provides a straightforward approach to accurately characterize femtosecond laser pulses used for HHG experiments right at the point of interaction.

Bowel Obstruction From Biliary Stent Migration: An Unusual Case of Abdominal Pain.

Endoscopic biliary stent placement is an important procedure that is commonly done in patients with malignant obstruction of the biliary tree. However, it can also be done to relieve non-maligant obstructions short term until more curative surgical interventions can be performed. There are two main types of stents used for these procedures: self-expanding metal stents (SEMSs) and plastic stents. Each of these stent types has different indications, and determining the correct stent for each individual patient is important. Here, we present a case of a 73-year-old female who presented with abdominal pain due to small bowel obstruction caused by a dislodged biliary duct stent. We hope to promote more focus on selecting the right stent type for each patient and encouraging follow-up visits after placement, especially for those with a history of medical noncompliance.

Structural and electronic properties of MoS2 and MoSe2 monolayers grown by chemical vapor deposition on Au(111).

The exceptional electronic and photonic properties of the monolayers of transition metal dichalcogenides including the spin-orbit splitting of the valence and conduction bands at the K points of the Brillouin zone make them promising for novel applications in electronics, photonics and optoelectronics. Scalable growth of these materials and understanding of their interaction with the substrate is crucial for these applications. Here we report the growth of MoS2 and MoSe2 monolayers on Au(111) by chemical vapor deposition at ambient pressure as well as the analysis of their structural and electronic properties down to the atomic scale. To this aim, we apply ultrahigh vacuum surface sensitive techniques including scanning tunneling microscopy and spectroscopy, low-energy electron diffraction, X-ray and angle-resolved ultraviolet photoelectron spectroscopy in combination with Raman spectroscopy at ambient conditions. We demonstrate the growth of high-quality epitaxial single crystalline MoS2 and MoSe2 monolayers on Au(111) and show the impact of annealing on the monolayer/substrate interaction. Thus, as-grown and moderately annealed (<100 °C) MoSe2 monolayers are decoupled from the substrate by excess Se atoms, whereas annealing at higher temperatures (>250 °C) results in their strong coupling with the substrate caused by desorption of the excess Se. The MoS2 monolayers are strongly coupled to the substrate and the interaction remains almost unchanged even after annealing up to 450 °C.

Tumour Microenvironment as a Potential Immune Therapeutic Target for Tongue Cancer Management.

Immunotherapy is a promising approach in the management of human cancers and has been proven to provide a durable response in many cancers. It is helpful as an adjuvant therapy for cancers and at present is considered as a fourth pillar supporting surgery, chemotherapy and radiotherapy. In the treatment of oral cancer, immunotherapy is approved in late-stage diseases where surgical resection cannot be carried out or fails, leading to recurrences and metastasis. Evidences suggest that when given as a first-line treatment, it can elicit an immune response that shrinks tumours, which could provide long-term benefit for patients. But unlike the traditional approach which follows the uniform protocol for all oral cancer patients, effective immunotherapy requires a more site-specific personalized approach. The aim of this paper is to review the various immune evasive mechanisms adopted by tumour cells and their relevance as potential targets for immunotherapy in oral tongue squamous cell carcinoma.

Kapitza-resistance-like exciton dynamics in atomically flat MoSe2-WSe2 lateral heterojunction.

Being able to control the neutral excitonic flux is a mandatory step for the development of future room-temperature two-dimensional excitonic devices. Semiconducting Monolayer Transition Metal Dichalcogenides (TMD-ML) with extremely robust and mobile excitons are highly attractive in this regard. However, generating an efficient and controlled exciton transport over long distances is a very challenging task. Here we demonstrate that an atomically sharp TMD-ML lateral heterostructure (MoSe2-WSe2) transforms the isotropic exciton diffusion into a unidirectional excitonic flow through the junction. Using tip-enhanced photoluminescence spectroscopy (TEPL) and a modified exciton transfer model, we show a discontinuity of the exciton density distribution on each side of the interface. We introduce the concept of exciton Kapitza resistance, by analogy with the interfacial thermal resistance referred to as Kapitza resistance. By comparing different heterostructures with or without top hexagonal boron nitride (hBN) layer, we deduce that the transport properties can be controlled, over distances far greater than the junction width, by the exciton density through near-field engineering and/or laser power density. This work provides a new approach for controlling the neutral exciton flow, which is key toward the conception of excitonic devices.

Regulating Li-Ion Transport through Ultrathin Molecular Membrane to Enable High-Performance All-Solid-State-Battery.

Solid-state lithium metal batteries with garnet-type electrolyte provide several advantages over conventional lithium-ion batteries, especially for safety and energy density. However, a few grand challenges such as the propagation of Li dendrites, poor interfacial contact between the solid electrolyte and the electrodes, and formation of lithium carbonate during ambient exposure over the solid-state electrolyte prevent the viability of such batteries. Herein, an ultrathin sub-nanometer porous carbon nanomembrane (CNM) is employed on the surface of solid-state electrolyte (SSE) that increases the adhesion of SSE with electrodes, prevents lithium carbonate formation over the surface, regulates the flow of Li-ions, and blocks any electronic leakage. The sub-nanometer scale pores in CNM allow rapid permeation of Li-ions across the electrode-electrolyte interface without the presence of any liquid medium. Additionally, CNM suppresses the propagation of Li dendrites by over sevenfold up to a current density of 0.7 mA cm-2 and enables the cycling of all-solid-state batteries at low stack pressure of 2 MPa using LiFePO4 cathode and Li metal anode. The CNM provides chemical stability to the solid electrolyte for over 4 weeks of ambient exposure with less than a 4% increase in surface impurities.

Interface engineering of charge-transfer excitons in 2D lateral heterostructures.

The existence of bound charge transfer (CT) excitons at the interface of monolayer lateral heterojunctions has been debated in literature, but contrary to the case of interlayer excitons in vertical heterostructure their observation still has to be confirmed. Here, we present a microscopic study investigating signatures of bound CT excitons in photoluminescence spectra at the interface of hBN-encapsulated lateral MoSe2-WSe2 heterostructures. Based on a fully microscopic and material-specific theory, we reveal the many-particle processes behind the formation of CT excitons and how they can be tuned via interface- and dielectric engineering. For junction widths smaller than the Coulomb-induced Bohr radius we predict the appearance of a low-energy CT exciton. The theoretical prediction is compared with experimental low-temperature photoluminescence measurements showing emission in the bound CT excitons energy range. We show that for hBN-encapsulated heterostructures, CT excitons exhibit small binding energies of just a few tens meV and at the same time large dipole moments, making them promising materials for optoelectronic applications (benefiting from an efficient exciton dissociation and fast dipole-driven exciton propagation). Our joint theory-experiment study presents a significant step towards a microscopic understanding of optical properties of technologically promising 2D lateral heterostructures.

CRAB score for prediction of colectomy within 2 years following admission for acute severe ulcerative colitis.

Background: The Oxford and Swedish indexes were developed to predict in-hospital colectomy in acute severe ulcerative colitis (ASUC), but not long-term prediction, and all these indexes were based on Western data. Our study aimed to analyze the predictors of colectomy within 3 years of ASUC in an Indian cohort and derive a simple predictive score. Methods: A prospective observational study was conducted in a tertiary health care center in South India over a period of 5 years. All patients admitted with ASUC were followed up for a period of 24 months after the index admission, to look for progression to colectomy. Results: A total of 81 (47 male) patients were included in the derivation cohort. Fifteen (18.5%) patients required colectomy during a follow-up period of 24 months. On regression analysis, C-reactive protein (CRP) and serum albumin were independent predictors of 24-month colectomy. The CRAB (CRP + AlBumin) score was obtained by multiplying coefficient of beta to albumin and CRP (CRAB score = CRP x 0.2 - Albumin x 0.26). The CRAB score demonstrated an AUROC of 0.923 and a score of >0.4 with a sensitivity of 82% and specificity of 92% for the prediction of 2-year colectomy following ASUC. The score was validated in a validation cohort of 31 patients, and at >0.4, the score had a sensitivity of 83% and a specificity of 96% in predicting colectomy. Conclusion: CRAB score is a simple prognostic score that can predict 2-year colectomy in ASUC patients with high sensitivity and specificity.

Nanoscale friction on MoS2/graphene heterostructures.

Stacked hetero-structures of two-dimensional materials allow for a design of interactions with corresponding electronic and mechanical properties. We report structure, work function, and frictional properties of 1 to 4 layers of MoS2 grown by chemical vapor deposition on epitaxial graphene on SiC(0001). Experiments were performed by atomic force microscopy in ultra-high vacuum. Friction is dominated by adhesion which is mediated by a deformation of the layers to adapt the shape of the tip apex. Friction decreases with increasing number of MoS2 layers as the bending rigidity leads to less deformation. The dependence of friction on applied load and bias voltage can be attributed to variations in the atomic potential corrugation of the interface, which is enhanced by both load and applied bias. Minimal friction is obtained when work function differences are compensated.

Charge-State-Enhanced Ion Sputtering of Metallic Gold Nanoislands.

Experimental results on the charge-state-dependent sputtering of metallic gold nanoislands are presented. Irradiations with slow highly charged ions of metallic targets were previously considered to show no charge state dependent effects on ion-induced material modification, since these materials possess enough free electrons to dissipate the deposited potential energy before electron-phonon coupling can set in. By reducing the size of the target material down to the nanometer regime and thus enabling a geometric energy confinement, a possibility is demonstrated to erode metallic surfaces by charge state related effects in contrast to regular kinetic sputtering.

Reproducibility of the computational fluid dynamic analysis of a cerebral aneurysm monitored over a decade.

Computational fluid dynamics (CFD) simulations are increasingly utilised to evaluate intracranial aneurysm (IA) haemodynamics to aid in the prediction of morphological changes and rupture risk. However, these models vary and differences in published results warrant the investigation of IA-CFD reproducibility. This study aims to explore sources of intra-team variability and determine its impact on the aneurysm morphology and CFD parameters. A team of four operators were given six sets of magnetic resonance angiography data spanning a decade from one patient with a middle cerebral aneurysm. All operators were given the same protocol and software for model reconstruction and numerical analysis. The morphology and haemodynamics of the operator models were then compared. The segmentation, smoothing factor, inlet and outflow branch lengths were found to cause intra-team variability. There was 80% reproducibility in the time-averaged wall shear stress distribution among operators with the major difference attributed to the level of smoothing. Based on these findings, it was concluded that the clinical applicability of CFD simulations may be feasible if a standardised segmentation protocol is developed. Moreover, when analysing the aneurysm shape change over a decade, it was noted that the co-existence of positive and negative values of the wall shear stress divergence (WSSD) contributed to the growth of a daughter sac.

Signatures of Multiband Effects in High-Harmonic Generation in Monolayer MoS_{2}.

High-harmonic generation (HHG) in solids has been touted as a way to probe ultrafast dynamics and crystal symmetries in condensed matter systems. Here, we investigate the polarization properties of high-order harmonics generated in monolayer MoS_{2}, as a function of crystal orientation relative to the mid-infrared laser field polarization. At several different laser wavelengths we experimentally observe a prominent angular shift of the parallel-polarized odd harmonics for energies above approximately 3.5 eV, and our calculations indicate that this shift originates in subtle differences in the recombination dipole strengths involving multiple conduction bands. This observation is material specific and is in addition to the angular dependence imposed by the dynamical symmetry properties of the crystal interacting with the laser field, and may pave the way for probing the vectorial character of multiband recombination dipoles.

Ion-Induced Surface Charge Dynamics in Freestanding Monolayers of Graphene and MoS_{2} Probed by the Emission of Electrons.

We compare the ion-induced electron emission from freestanding monolayers of graphene and MoS_{2} to find a sixfold higher number of emitted electrons for graphene even though both materials have similar work functions. An effective single-band Hubbard model explains this finding by a charge-up in MoS_{2} that prevents low energy electrons from escaping the surface within a period of a few femtoseconds after ion impact. We support these results by measuring the electron energy distribution for correlated pairs of electrons and transmitted ions. The majority of emitted primary electrons have an energy below 10 eV and are therefore subject to the dynamic charge-up effects at surfaces.

Exciton Dynamics in MoS2-Pentacene and WSe2-Pentacene Heterojunctions.

We measured the exciton dynamics in van der Waals heterojunctions of transition metal dichalcogenides (TMDCs) and organic semiconductors (OSs). TMDCs and OSs are semiconducting materials with rich and highly diverse optical and electronic properties. Their heterostructures, exhibiting van der Waals bonding at their interfaces, can be utilized in the field of optoelectronics and photovoltaics. Two types of heterojunctions, MoS2-pentacene and WSe2-pentacene, were prepared by layer transfer of 20 nm pentacene thin films as well as MoS2 and WSe2 monolayer crystals onto Au surfaces. The samples were studied by means of transient absorption spectroscopy in the reflectance mode. We found that A-exciton decay by hole transfer from MoS2 to pentacene occurs with a characteristic time of 21 ± 3 ps. This is slow compared to previously reported hole transfer times of 6.7 ps in MoS2-pentacene junctions formed by vapor deposition of pentacene molecules onto MoS2 on SiO2. The B-exciton decay in WSe2 shows faster hole transfer rates for WSe2-pentacene heterojunctions, with a characteristic time of 7 ± 1 ps. The A-exciton in WSe2 also decays faster due to the presence of a pentacene overlayer; however, fitting the decay traces did not allow for the unambiguous assignment of the associated decay time. Our work provides important insights into excitonic dynamics in the growing field of TMDC-OS heterojunctions.

Patterned Growth of Transition Metal Dichalcogenide Monolayers and Multilayers for Electronic and Optoelectronic Device Applications.

A simple, large area, and cost-effective soft lithographic method is presented for the patterned growth of high-quality 2D transition metal dichalcogenides (TMDs). Initially, a liquid precursor (Na2 MoO4 in an aqueous solution) is patterned on the growth substrate using the micromolding in capillaries technique. Subsequently, a chemical vapor deposition step is employed to convert the precursor patterns to monolayer, few layers, or bulk TMDs, depending on the precursor concentration. The grown patterns are characterized using optical microscopy, atomic force microscopy, Raman spectroscopy, X-ray photoelectron spectroscopy, scanning electron microscopy, and photoluminescence spectroscopy to reveal their morphological, chemical, and optical characteristics. Additionally, electronic and optoelectronic devices are realized using the patterned TMDs and tested for their applicability in field effect transistors and photodetectors. The photodetectors made of MoS2 line patterns show a very high responsivity of 7674 A W-1 and external quantum efficiency of 1.49 × 106 %. Furthermore, the multiple grain boundaries present in patterned TMDs enable the fabrication of memtransistor devices. The patterning technique presented here may be applied to many other TMDs and related heterostructures, potentially advancing the fabrication of TMDs-based device arrays.

Chemical Vapor Deposition of High-Optical-Quality Large-Area Monolayer Janus Transition Metal Dichalcogenides.

One-pot chemical vapor deposition (CVD) growth of large-area Janus SeMoS monolayers is reported, with the asymmetric top (Se) and bottom (S) chalcogen atomic planes with respect to the central transition metal (Mo) atoms. The formation of these 2D semiconductor monolayers takes place upon the thermodynamic-equilibrium-driven exchange of the bottom Se atoms of the initially grown MoSe2 single crystals on gold foils with S atoms. The growth process is characterized by complementary experimental techniques including Raman and X-ray photoelectron spectroscopy, transmission electron microscopy, and the growth mechanisms are rationalized by first principle calculations. The remarkably high optical quality of the synthesized Janus monolayers is demonstrated by optical and magneto-optical measurements which reveal the strong exciton-phonon coupling and enable an exciton g-factor of -3.3.