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Biliary atresia (BA) is a progressive fibroinflammatory disease affecting both the extrahepatic and intrahepatic bile ducts, potentially leading to chronic cholestasis and biliary cirrhosis. Despite its prevalence, the exact mechanisms behind BA development remain incompletely understood. Recent research suggests that the gut microbiota and its metabolites may play significant roles in BA development. This paper offers a comprehensive review of the changing characteristics of gut microbiota and their metabolites at different stages of BA in children. It discusses their influence on the host's inflammatory response, immune system, and bile acid metabolism. The review also explores the potential of gut microbiota and metabolites as a therapeutic target for BA, with interventions like butyrate and gut microbiota preparations showing promise in alleviating BA symptoms. While progress has been made, further research is necessary to untangle the complex interactions between gut microbiota and BA, paving the way for more effective prevention and treatment strategies for this challenging condition.
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Ácidos e Sais Biliares , Atresia Biliar , Microbioma Gastrointestinal , Microbioma Gastrointestinal/fisiologia , Humanos , Atresia Biliar/microbiologia , Atresia Biliar/metabolismo , Ácidos e Sais Biliares/metabolismo , AnimaisRESUMO
Nobiletin (NOB) is a flavonoid derived from citrus peel that has potential as an alternative treatment for liver disease. Liver disease is a primary health concern globally, and there is an urgent need for effective drugs. This review summarizes the pharmacological characteristics of NOB and current in vitro and in vivo studies investigating the preventive and therapeutic effects of NOB on liver diseases and its potential mechanisms. The findings suggest that NOB has promising therapeutic potential in liver diseases. It improves liver function, reduces inflammation and oxidative stress, remodels gut microflora, ameliorates hepatocellular necrosis, steatosis, and insulin resistance, and modulates biorhythms. Nuclear factor erythroid 2-related factor 2 (Nrf2), nuclear transcription factor kappa (NF-κB), AMP-activated protein kinase (AMPK), peroxisome proliferator-activated receptor α(PPAR-α), extracellular signal-regulated kinase (ERK), protein kinase B (AKT), toll-like receptor 4 (TLR4) and transcription factor EB (TFEB) signaling pathways are important molecular targets for NOB to ameliorate liver diseases. In conclusion, NOB may be a promising drug candidate for treating liver disease and can accelerate its application from the laboratory to the clinic. However, more high-quality clinical trials are required to validate its efficacy and identify its molecular mechanisms and targets.
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Cerium-doped lutetium yttrium orthosilicate (LYSO:Ce) powder has been synthesized by the co-precipitation method. The influence of the Ce3+ doping concentration on the lattice structure and luminescence characteristics of LYSO:Ce powder was investigated by X-ray diffraction (XRD) and photoluminescence (PL). The XRD measurement indicates that the lattice structure of LYSO:Ce powder was not changed by doping ions. PL results show that LYSO:Ce powder has better luminescence performance when the Ce doping concentration is 0.3 mol%. In addition, the fluorescence lifetime of the samples was measured, and the results show that LYSO:Ce has a short decay time. The radiation dosimeter was prepared by LYSO:Ce powder with a Ce doping concentration of 0.3 mol%. Radioluminescence properties of the radiation dosimeter also were studied under X-ray irradiation at doses from 0.03 to 0.76 Gy, with dose rate from 0.09 to 2.284 Gy/min. The results show that the dosimeter has a certain linear relationship response and stability. The radiation responses of the dosimeter at different energies were obtained under X-ray irradiation with X-ray tube voltages ranging from 20 to 80 kV. The results show that the dosimeter has a certain linear relationship response in the low energy range of radiotherapy. These results indicate the potential application of LYSO:Ce powder dosimeters in remote radiotherapy and online radiation monitoring.
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OBJECTIVES: To ascertain the clinical characteristics of pediatric patients with contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis (AEs). METHODS: Two cases of CASPR2 antibody-associated AEs have been reported. In addition, a systematic search of literature published between January 2010 and March 2022 through six online databases was conducted to identify the pediatric patients with CASPR2 antibody-associated AEs. Data on demographics, clinical symptoms, laboratory examinations, imaging, treatment, and outcome were collected. RESULTS: Our updated literature search yielded 1,837 publications, of which 21 were selected, and 40 patients in this study met the diagnostic criteria for AE. There were 25 males and 15 females with a mean age of 9.2 years. The most common presenting symptoms are psychiatric symptoms (72.5%), sleep changes (62.5%), and movement disorders (60%). The psychiatric symptoms included mood changes (39.1%), behavior changes (25%), and hallucination (7.5%). In total, 23 cases (57.5%) combined with autonomic dysfunction, such as gastrointestinal dysmotility, cardiovascular-related symptoms, and sweating. No tumors were observed in children. Thirty-eight patients received first-line immunotherapy, and eight received first-line and second-line immunotherapy. All patients had a good clinical response to immune therapy. Mean mRS at onset was 3.4; It was 0.88 at the last follow-up. There was no recurrence during follow-up. CONCLUSION: Psychiatric symptoms, sleep disorders, movement disorders, and cardiovascular-related symptoms are the most common presentation in pediatric patients with CASPR2 antibody-associated AEs. Tumor, particularly with thymoma, is uncommon in children diagnosed with CASPR2 antibody-associated AEs. In addition, prompt diagnosis and immunotherapy can relieve symptoms and improve the prognosis.
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Doenças Autoimunes do Sistema Nervoso , Transtornos dos Movimentos , Neoplasias do Timo , Masculino , Feminino , Humanos , Criança , Autoanticorpos , Doenças Autoimunes do Sistema Nervoso/diagnóstico , ContactinasRESUMO
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most commonly identified cause of autoimmune encephalitis. Therapeutic plasma exchange has been increasingly employed to treat this disease. This expansion is a consequence of improved techniques and apheresis instruments, as well as the recognition of its applicability in neurological diseases. However, several aspects of treatment remain incompletely clarified, and treatment strategies are still heterogeneous, especially with regard to therapeutic plasma exchange in anti-NMDAR encephalitis. This review provides an overview of the use of therapeutic plasma exchange including the principle and mechanisms, the evidence, initial time, efficiency and complications in anti-NMDAR encephalitis.
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Encefalite Antirreceptor de N-Metil-D-Aspartato , Doença de Hashimoto , Humanos , Encefalite Antirreceptor de N-Metil-D-Aspartato/complicações , Encefalite Antirreceptor de N-Metil-D-Aspartato/terapia , Troca Plasmática/métodos , Receptores de N-Metil-D-Aspartato , Doença de Hashimoto/terapiaRESUMO
Primary cardiac tumors in children are exceedingly rare overall, which benign account for most part. The onset of the disease is occult, while the clinical manifestations are non-specific-patients may be asymptomatic or show a range of obstructive, arrhythmic, embolic or systemic symptoms. The clinical presentations generally depend on the tumors' size, localization, and pace of growth of the tumor. Moreover, the diagnosis needs comprehensive judgment based on imaging results and pathological examination. With advances in cardiac imagining and the introduction of cardiopulmonary support, the diagnosis and treatment of these rare tumors have improved the prognosis and outlook for benign tumors. To sum up the above, we sought to integrate articles from recent years for the latest comprehensive review of the clinical manifestations, imaging characteristics, clinic pathologic features and treatment of benign cardiac tumors in children to provide a broader idea for pediatricians to recognize and treat such diseases.
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Transition metal dichalcogenides have been the most attractive two-dimensional layered materials for electrocatalytic hydrogen evolution due to their unique structure and multi-phase electronic states. However, the enhancement of the WSe2 electrocatalytic hydrogen evolution reaction (HER) performance by bimetal co-doping has been rarely reported. Herein, the NiMo-WSe2 catalyst has been synthesized by a one-step hydrothermal reaction, with lower overpotentials of 177 and 188 mV at a current density of 10 mA cm-2 in 0.5 M H2SO4 and 1 M KOH, respectively. The large specific surface area and thinner edge morphology provide more active sites for hydrogen production, thereby significantly improving the charge transfer kinetics. Density functional theory calculation results show that under acidic conditions the ΔGH* values of NiMo-WSe2 with different structures and hydrogen adsorption sites are also different, when the hydrogen adsorption site was located at the top of the Se-Ni bond, the meta NiMo-WSe2 has a ΔGH* value (-0.04 eV) that is closest to 0. Meanwhile, NiMo-WSe2 (meta) also has a minimum of ΔGH* under alkaline conditions. DOS confirmed that Ni doping has a large impact on the electronic states at the WSe2 Fermi level, while NiMo co-doping greatly reduces the potential energy barrier of the HER reaction, jointly increasing the current density, and thus improving the HER performance.
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OBJECTIVES: 3ß-hydroxy-Δ5-C27-steroid dehydrogenase deficiency is a rare autosomal recessive condition. So far fewer than 100 cases have been reported and the factors affecting the prognosis are not yet established. The objective of this study is to explore a possible prediction of the outcome of this rare condition. METHODS: This review was undertaken and reported in accordance with the preferred reporting items for systematic review and meta-analyses guidelines. Demographics, clinical features, gene data, treatment strategies and prognoses at the last follow-up were extracted and summarized. Patients were divided into 2 groups (alive with native liver and liver transplantation/died). Risk factors for the different clinical features were identified. RESULTS: 87 patients that were taken from 7 case reports and 9 case series were included. 38 (38/63, 63.0%) of them presented initial symptoms when they were younger than 1 month and 55 (55/63, 87.3%) less than 1 year. There is a larger proportion of patients younger than 1 month or 1 year at the age of symptom onset in the liver transplantation /died group than patients in alive with the native liver group. The majority of patients (53/62, 85.5%) were diagnosed before the age of 5 year. In all cases, 65 (predicted) pathogenic variants have been identified. Over 70% of patients carried an HSD3B7 variant on exon 1, 4, 5 or 6. 71 (81.6%) were alive at the last follow-up, 16 (18.4%) underwent liver transplantation or died. No significance was found between the group alive with native liver and group liver transplantation /died. CONCLUSION: Age of onset of the symptoms may be a potential factor that determines the outcome of patients with 3ß-HSD deficiency, patients presented with symptoms and signs at an age younger than 1 month or even 1 year may have a worse prognosis. Since there is no difference between clinical outcome and zygosity of gene mutation, we recommend a further study about any possible relationship between mutation site and clinical characteristics or prognosis.