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Clinical and molecular characterization of 10 Chinese children with congenital adrenal hyperplasia due to 11beta‑hydroxylase deficiency

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World Journal of Pediatrics 2024年第4期20卷 422-433页

作者： wen‑li lu Xiao‑Yu Ma Jiao Zhang Jun‑Qi Wang Ting‑Ting Zhang Lei Ye Yuan Xiao Zhi‑Ya Dong Wei Wang Shou‑Yue Sun Chuan‑Yin li Rong‑Gui Hu Guang Ning li‑Dan Zhang Department of Pediatrics Ruijin HospitalShanghai Jiao Tong University School of MedicineNo.197Ruijin 2nd RdHuangpu DistrictShanghai 200025China Department of Pediatric Genetic and Metabolic Endocrinology West China Second University HospitalSichuan UniversityNo.20Section 3Renmin South RoadSichuan 610041China Department of Endocrine and Metabolic Diseases Shanghai Clinical Center for Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Cancer Center School of MedicineShanghai Tenth People’s HospitalTongji UniversityYanchang Zhong Lu 301St RdJing’an DistrictShanghai 200031China

Background The clinical manifestations of nonclassical 11beta-hydroxylase deficiency are very similar to those of nonclassical 21-hydroxylase deficiency.For this study,we investigated the relationship between the clinical and molecular features of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency and reviewed the related literature,which are expected to provide assistance for the clinical diagnosis and analysis of congenital adrenal hyperplasia.Methods Clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively analyzed.We examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of proteins.Results We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were reported.Female patients received treatment at an early stage,with an average age of 2.08±1.66 years,whereas male patients received treatment significantly later,at an average age of 9.77±3.62 years.The most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>T.All mutations lead to spatial conformational changes that affect protein stability.Conclusions Our study found that there was no significant correlation between each specific mutation and the severity of clinical manifestations.Different patients with the same gene pathogenic variant may have mild or severe clinical manifestations.The correlation between genotype and phenotype needs further study.Three-dimensional protein simulations may provide additional support for the physiopathological mechanism of genetic mutations.

关键词： 11β-hydroxylase deficiency CYP11B1 Enzymatic activity Phenotype-genotype correlation Threedimensional protein simulations

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Platycodin D inhibits angiogenic vascular mimicry in NSCLC by regulating the eIF4E-mediated RNA methylome

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Journal of Pharmaceutical Analysis 2024年第1期14卷 152-155页

作者： Shuyu Zheng Yanlin Xin Jiamin lin Zejuan Xie Keyu Cheng Shanshan Wang wenli lu Hao Yang Tianming lu Jun li Ruogu Qi Yuanyuan Guo Department of Biochemistry and Molecular Biology School of Medicine&Holistic Integrative MedicineNanjing University of Chinese MedicineNanjing210023China Department of Neurosurgery The Second Affiliated HospitalZhejiang UniversityHangzhou310009China

The treatment of non-small cell lung cancer(NSCLC)remains a challenge due to tumor evolution during anti-angiogenesis therapies,in which the mechanism of vascular mimicry(VM)is believed to result in ineffective treatment[1].To conquer this challenge,substantial effort has recently been devoted to seeking out natural compounds on account of their multitarget actions.As a traditional herbal medicine,platycodin D(PD)is the major bioactive monomer derived from Platycodon grandiflorum(P.grandiflorum)and is used as an expectorant for pulmonary disease in Asia[2].

关键词： NSCLC eIF4E treatment

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Evaluation of G3BP1 in the prognosis of acute and acute-on-chronic liver failure after the treatment of artificial liver support system

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World Journal of Hepatology 2024年第2期16卷 251-263页

作者： wen-Yuan li lu-wen Wang Jin Dong Yao Wang Department of Anesthesiology Renmin Hospital of Wuhan UniversityWuhan 430060Hubei ProvinceChina Department of Infectious Diseases Renmin Hospital of Wuhan UniversityWuhan 430060Hubei ProvinceChina Department of Nephrology Renmin Hospital of Wuhan UniversityWuhan 430060Hubei ProvinceChina

BACKGROUND The increased expression of G3BP1 was positively correlated with the prognosis of liver failure.AIM To investigate the effect of G3BP1 on the prognosis of acute liver failure(ALF)and acute-on-chronic liver failure(ACLF)after the treatment of artificial liver support system(ALSS).METHODS A total of 244 patients with ALF and ACLF were enrolled in this study.The levels of G3BP1 on admission and at discharge were detected.The validation set of 514 patients was collected to verify the predicted effect of G3BP1 and the viability of prognosis.RESULTS This study was shown that lactate dehydrogenase(LDH),alpha-fetoprotein(AFP)and prothrombin time were closely related to the prognosis of patients.After the ALSS treatment,the patient’amount of decreased G3BP1 index in difference of G3BP1 between the value of discharge and admission(difG3BP1)liver disease high-risk or low-risk group.At the same time,compared with the inflam matory marks[tumor necrosis factor-α,interleukin(IL)-1βand IL-18],G3BP1 had higher discrimination and was more stable in the model analysis and validation set.When combined with AFP and LDH,concordance index was respectively 0.84 and 0.8 in training and validation cohorts.CONCluSION This study indicated that G3BP1 could predict the prognosis of ALF or ACLF patients treated with ALSS.The combination of G3BP1,AFP and LDH could accurately evaluate the disease condition and predict the clinical endpoint of patients.

关键词： G3BP1 Prognosis Acute liver failure Acute-on-chronic liver failure Artificial liver support system

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Metallothionein 1E Alleviates Cadmium-induced Renal Cytotoxicity through Promoting Mitochondrial Functional Recovery

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Biomedical and Environmental Sciences 2024年第1期37卷 117-121页

作者： wen Si Hui li lu Bei HUANG Hui Dan XIE Ying luO Lei Key Laboratory of Molecular Epidemiology of Hunan Province School of MedicineHunan Normal UniversityChangsha 410081HunanChina Department of Occupational Health Changsha Centre for Disease Control and PreventionChangsha 410004HunanChina The Key Laboratory of Model Animals and Stem Cell Biology in Hunan Province Hunan Normal University School of MedicineChangsha 410013HunanChina

The pervasive utilization of industrial substances has escalated human exposure to cadmium(Cd),a metal associated with long-term negative health outcomes such as renal dysfunction,neurological disorders,and various cancers^([1]).Once ingested by humans,Cd interacts with cysteine-rich metallothioneins(MTs)which have metal-binding and antioxidant properties and is subsequently transported to the kidney^([2]).

关键词： Renal kidney cadmium

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Upadacitinib for refractory ulcerative colitis with primary nonresponse to infliximab and vedolizumab:A case report

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World Journal of Clinical Cases 2024年第9期12卷 1685-1690页

作者： Xuan Xu Jing-wen Jiang Bing-Yun lu Xia-Xi li Department of Gastroenterology Shenzhen HospitalSouthern Medical UniversityShenzhen 518100Guangdong ProvinceChina The First Clinical Medical School Southern Medical UniversityGuangzhou 510515Guangdong ProvinceChina

BACKGROUND Many patients with ulcerative colitis(UC)do not respond well to,or tolerate conventional and biological therapies.There is currently no consensus on the treatment of refractory UC.Studies have demonstrated that the selective Janus kinase 1 inhibitor upadacitinib,a small-molecule drug,is effective and safe for treating UC.However,no studies have revealed that upadacitinib is effective in treating refractory UC with primary nonresponse to infliximab and vedolizumab.CASE SUMMARY We report the case of a 44-year-old male patient with a chief complaint of bloody diarrhoea with mucus and pus,in addition to dizziness.The patient had recurrent disease after receiving mesalazine,prednisone,azathioprine,infliximab and vedolizumab over four years.Based on the endoscopic findings and pathological biopsy,the patient was diagnosed with refractory UC.In particular,the patient showed primary nonresponse to infliximab and vedolizumab.Based on the patient’s history and recurrent disease,we decided to administer upadacitinib.During hospitalisation,the patient was received upadacitinib under our guidance.Eight weeks after the initiation of upadacitinib treatment,the patient’s symptoms and endoscopic findings improved significantly.No notable adverse reactions have been reported to date.CONCluSION Our case report suggests that upadacitinib may represent a valuable strategy for treating refractory UC with primary nonresponse.

关键词： Upadacitinib Refractory ulcerative colitis Primary nonresponse Infliximab Vedolizumab Case report

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Pt nanoclusters modified porous g-C_(3)N_(4)nanosheets to significantly enhance hydrogen production by photocatalytic water reforming of methanol

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Chinese Journal of Chemical Engineering 2024年第2期66卷 40-50页

作者： Yi-Fei liang Jin-Rong lu Shang-Kun Tian wen-Quan Cui li liu Hebei Provincial Key Laboratory of Environmental Photocatalytic and Electrocatalytic Materials College of Chemical EngineeringNorth China University of Science and TechnologyTangshan 063210China

For the use of green hydrogen energy,it is crucial to have efficient photocatalytic activity for hydrogen generation by water reforming of methanol under mild conditions.Much attention has been paid to gC_(3)N_(4)as a promising photocatalyst for the generation of hydrogen.To improve the separation of photogenerated charge,porous nanosheet g-C_(3)N_(4)was modified with Pt nanoclusters(Pt/g-C_(3)N_(4))through impregnation and following photo-induced reduction.This catalyst showed excellent photocatalytic activity of water reforming of methanol fo r hydrogen production with a 17.12 mmol·g^(-1)·h^(-1)rate at room temperature,which was 311 times higher than that of the unmodified g-C_(3)N_(4).The strong interactions of Pt-N in Pt/g-C_(3)N_(4)constructed effective electron transfer channels to promote the separation of photogenerated electrons and holes effectively.In addition,in-situ infrared spectroscopy was used to investigate the intermediates of the hydrogen production reaction,which proved that methanol and water eventually turn into H_(2)and CO_(2)via formaldehyde and formate.This study provides insights for understanding the photocatalytic hydrogen production in the water reforming of methanol.

关键词： Water reforming of methanol Photocatalysis g-C_(3)N_(4) Pt nanoclusters Hydrogen production

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Ni-Catalyzed Enantioselective Difunctionalization of Alkynes to Azepine Derivatives Bearing a Quaternary Center and an Unprotected Imine

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Chinese Journal of Chemistry 2024年第8期42卷 873-878页

作者： Jian Long Zhiwu lu Xiao-lin li Peng Xue wen-Bo liu Engineering Research Center of Organosilicon Compounds&Materials(Ministry of Education)and College of Chemistry and Molecular Sciences Wuhan UniversityWuhanHubei430072 China

Comprehensive Summary,The azepine ring is a prominent structural scaffold in biologically significant molecules. In this study, we present a Ni(II)-catalyzed asymmetric difunctionalization of alkynes, involving intermolecular regioselective arylation and intramolecular nitrile addition, enabling the synthesis of enantioenriched azepine derivatives. This reaction simultaneously installs an all-carbon quaternary stereocenter and introduces an unprotected imine functionality, showing great promise for subsequent transformations. The reaction exhibits good tolerance toward various functional groups, resulting in high yields and enantioselectivities. The synthetic utility of this methodology is further demonstrated through gram-scale synthesis and product derivatization. This research offers an efficient approach to the synthesis of seven-membered nitrogen heterocycles.

关键词： Nickel Asymmetric catalysis N-Heterocycles Alkyne functionalization Quaternary centers

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Nano polystyrene microplastics could accumulate in Nile tilapia(Oreochromis niloticus):Negatively impacts on the intestinal and liver health through water exposure

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Journal of Environmental Sciences 2024年第3期137卷 604-614页

作者： Di Wu Xing lu li-Xue Dong Juan Tian Jin Deng Lei Wei Hua wen Shan Zhong Ming Jiang School of Basic Medical Sciences Wuhan UniversityWuhan 430071China Key Laboratory of Freshwater Biodiversity Conservation Ministry of Agriculture and Rural AffairsYangtze River Fisheries Research InstituteChinese Academy of Fishery SciencesWuhan 430223China Hubei Province Key Laboratory of Allergy and Immunology Wuhan 430071China

Microplastics(MPs)have become a significant concern for their potential toxicity.However,the correlation between the size of plastic particles and their toxicity remains inconclusive.Here,we investigate the toxic effects of different sizes(80 nm,800 nm,8μm and 80μm)polystyrene MPs(PS-MPs)on the model organism Nile tilapia(Oreochromis niloticus).The results of bioluminescent imaging indicate that the 80 nm PS-MPs are more likely to invade the body.H&E staining shows severe damage on the intestinal villi and distinct hepatic steatosis in the 80 nm group.Ed U labeling shows that the proliferation activity of intestinal and liver cells reduces significantly in the 80 nm group.The gut microbiome analysis shows a severe imbalance of gut microbiota homeostasis in the 80 nm group.The analysis of liver transcriptomics and metabolomics shows that the liver lipid metabolism is disordered in the 80 nm group.In conclusion,this study confirms that the 80 nm PS-MPs are more likely to induce intestinal and liver toxicity.All the above lay the foundation for further study on the pathological damage of MPs to other organisms.

关键词： Microplastics(MPs) Intestinal toxicity liver toxicity Gut microbiome lipid metabolism

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Energy beam-based direct and assisted polishing techniques for diamond:A review

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International Journal of Extreme Manufacturing 2024年第1期6卷 93-124页

作者： Zhuo li Feng Jiang Zhengyi Jiang Zige Tian Tian Qiu Tao Zhang Qiuling wen Xizhao lu Jing lu Hui Huang Institute of Manufacturing Engineering Huaqiao UniversityXiamen 361021People’s Republic of China School of Mechanical MaterialsMechatronic and Biomedical EngineeringUniversity of WollongongWollongongNSW 2522Australia College of Mechanical Engineering and Automation Huaqiao UniversityXiamen 361021People’s Republic of China State Key Laboratory of High Performance Tools Huaqiao UniversityXiamen 361021People’s Republic of China

Diamond is a highly valuable material with diverse industrial applications,particularly in the fields of semiconductor,optics,and high-power electronics.However,its high hardness and chemical stability make it difficult to realize high-efficiency and ultra-low damage machining of diamond.To address these challenges,several polishing methods have been developed for both single crystal diamond(SCD)and polycrystalline diamond(PCD),including mechanical,chemical,laser,and ion beam processing methods.In this review,the characteristics and application scope of various polishing technologies for SCD and PCD are highlighted.Specifically,various energy beam-based direct and assisted polishing technologies,such as laser polishing,ion beam polishing,plasma-assisted polishing,and laser-assisted polishing,are summarized.The current research progress,material removal mechanism,and infuencing factors of each polishing technology are analyzed.Although some of these methods can achieve high material removal rates or reduce surface roughness,no single method can meet all the requirements.Finally,the future development prospects and application directions of different polishing technologies are presented.

关键词： single crystal diamond polycrystalline diamond energy beam polishing technology material removal mechanism influencing factors

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Early systemic anticoagulation reduces hospital readmission in acute necrotizing pancreatitis patients:A retrospective cohort study

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Hepatobiliary & Pancreatic Diseases International 2024年第1期23卷 77-82页

作者： wen-Jian Mao Jing Zhou Guo-Fu Zhang Fa-Xi Chen Jing-Zhu Zhang Bai-Qiang li lu Ke Wei-Qin li Department of Critical Care Medicine Jinling HospitalNanjing Medical UniversityNanjing 210002China Department of Critical Care Medicine Jinling HospitalMedical School of Nanjing UniversityNanjing 210002China National Institute of Healthcare Data Science Nanjing UniversityNanjing 210010China

Background:Early systemic anticoagulation(SAC)is a common practice in acute necrotizing pancreatitis(ANP),and its impact on in-hospital clinical outcomes had been assessed.However,whether it affects long-term outcomes is unknown.This study aimed to evaluate the effect of SAC on 90-day readmission and other long-term outcomes in ANP patients.Methods:During January 2013 and December 2018,ANP patients admitted within 7 days from the onset of abdominal pain were screened.The primary outcome was 90-day readmission after discharge.Cox proportional-hazards regression model and mediation analysis were used to define the relationship between early SAC and 90-day readmission.Results:A total of 241 ANP patients were enrolled,of whom 143 received early SAC during their hospitalization and 98 did not.Patients who received early SAC experienced a lower incidence of splanchnic venous thrombosis(SVT)[risk ratio(RR)=0.40,95%CI:0.26-0.60,Plity of life,patients who received early SAC had a significantly higher score in the subscale of vitality(P=0.03)while the other subscales were all comparable between the two groups.Multivariable Cox regression model showed that early SAC was an independent protective factor for 90-day readmission after adjusting for potential confounders with a hazard ratio of 0.57(95%CI:0.34-0.96,P=0.04).Mediation analysis showed that SVT mediated 37.0%of the early SAC-90-day readmission causality.Conclusions:The application of early SAC may reduce the risk of 90-day readmission in the survivors of ANP patients,and reduced SVT incidence might be the primary contributor.

关键词： Anticoagulation Splanchnic venous thrombosis Acute necrotizing pancreatitis Readmission Long-term outcomes

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