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Current understanding of ELF4 deficiency:a novel inborn error of immunity

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World Journal of Pediatrics 2024年第5期20卷 444-450页

作者： Hong-Qiang du xiao-Dong Zhao Department of Rheumatology&Immunology National Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of Child Rare Diseases in Infection and ImmunityChildren’s Hospital of Chongqing Medical UniversityZhongshan Er Road 136Yuzhong DistrictChongqingChina

Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this condition,reported by our team and others,are very limited worldwide.As such,our current knowledge of this new disease remains preliminary.This review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel IEI.Data sources A comprehensive review was conducted after an extensive literature search in the PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with ELF4 deficiency.Our search strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of the time of manuscript submission.Results The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric males.In some cases,immunodeficiency and autoimmunity can also be prominent.Targeted Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 gene.Western blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the variant.To fully confirm the pathogenicity of the variant,further functional test is strongly advised.Glucocorticoid and biologics are the mainstream management of ELF4 deficiency disorder.Conclusions Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of DEX.When atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 function.Experience of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and further mechanistic study.

关键词： ELF4 transcription factor Immune dysregulation Inborn errors of immunity Mechanism Recurrent infections

Toeplitz Operators and Carleson Measures for Weighted Bergman Spaces Induced by Regular Weights

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Acta Mathematica Sinica,English Series 2024年第5期40卷 1345-1359页

作者： Jun Tao du Song xiao Hasi WULAN Department of Mathematics Guangdong University of Petrochemical TechnologyMaoming 525000P.R.China Department of Mathematics Shantou UniversityShantou 515063P.R.China

In this paper,we give a universal description of the boundedness and compactness of Toeplitz operator T_(μ)^(ω)between Bergman spaces A_(η)^(p)and A_(υ)^(q)whenμis a positive Borel measure,1 详细信息

关键词： Bergman space Carleson measure Toeplitz operator regular weight

Stress-corrosion coupled damage localization induced by secondary phases in bio-degradable Mg alloys:phase-field modeling

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Journal of Magnesium and Alloys 2024年第1期12卷 361-383页

作者： Chao Xie Shijie Bai xiao Liu Minghua Zhang Jianke du The Faculty of Mechanical Engineering and Mechanics Ningbo UniversityNingbo 315211P.R.China Key Laboratory of High Temperature Wear Resistant Materials Preparation Technology of Hunan Province Hunan University of Science and TechnologyXiangtan 411201P.R.China

In this study,a phase-field scheme that rigorously obeys conservation laws and irreversible thermodynamics is developed for modeling stress-corrosion coupled damage(SCCD).The coupling constitutive relationships of the deformation,phase-field damage,mass transfer,and electrostatic field are derived from the entropy inequality.The SCCD localization induced by secondary phases in Mg is numerically simulated using the implicit iterative algorithm of the self-defined finite elements.The quantitative evaluation of the SCCD of a C-ring is in good agreement with the experimental results.To capture the damage localization,a micro-galvanic corrosion domain is defined,and the buffering effect on charge migration is explored.Three cases are investigated to reveal the effect of localization on corrosion acceleration and provide guidance for the design for resistance to SCCD at the crystal scale.

关键词： Phase field Mg alloys Stress-corrosion coupled damage Damage localization Finite element method

Multi-endpoint assays reveal more severe toxicity induced by chloraminated effluent organic matter than chloraminated natural organic matter

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Journal of Environmental Sciences 2024年第1期135卷 310-317页

作者： Hai-Yan Wang De-Xiu Wu Ye du xiao-Tong Lv Qian-Yuan Wu State Key Laboratory of Environmental Criteria and Risk Assessment Chinese Research Academy of Environmental SciencesBeijing 100012China Environmental Protection Key Laboratory of Microorganism Application and Risk Control Guangdong Provincial Engineering Research Center for Urban Water Recycling and Environmental SafetyTsinghua Shenzhen International Graduate SchoolTsinghua UniversityShenzhen 518055China College of Architecture and Environment Sichuan UniversityChengdu 610000China

Disinfection by chloramination produces toxic byproducts and the difference in toxicity of reclaimed and drinking water treated by chloramination remains unclear.This study investigated cytotoxic effects at the same concentrations of dissolved organic matter and showed that chloraminated effluent organic matter(EfOM)induced 1.7 times higher cytotoxicity than chloraminated natural organic matter(NOM)applied to simulate drinking water.Chloraminated EfOM induced more reactive nitrogen species than chloraminated NOM,and chloraminated EfOM and NOM induced similar and higher levels of reactive oxygen species than the negative control,respectively.Consequently,intracellular macromolecule damage indicated by DNA/RNA damage marker 8–hydroxy-(deoxy)guanosine and the intracellular protein carbonyl concentration induced by chloraminated EfOM was higher and slightly more than that induced by chloraminated NOM,respectively.These data were consistent with the effects on cell physiological processes.Cell cycle arrest mainly occurred in G2 phase by chloraminated EfOM and NOM.Early apoptotic cells,which could return to normal,increased upon exposure to high concentrations of chloraminated EfOM and NOM.Moreover,necrotic cells were significantly increased from 0.5%to 2.5%when the concentration increased from 20-to 60-fold chloraminated EfOM,but were not obviously changed by chloraminated NOM.These results indicated that the comprehensive intracellular changes induced by toxic substances in chloraminated EfOM were more irreversible and induced more cell death than chloraminated NOM.

关键词： Cytotoxicity Intracellular active nitrogen/oxygen Intracellular oxidative damage Cell necrosis Reclaimed water

Risk factors for cognitive impairment in patients with chronic kidney disease

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World Journal of Psychiatry 2024年第2期14卷 308-314页

作者： xiao-Hui Wang Yong He Huan Zhou Ting xiao Ran du Xin Zhang Department of Nephrology The Fifth Hospital of WuhanWuhan 430050Hubei ProvinceChina Department of Nephrology The Central Hospital of Enshi Tujia and Miao Autonomous PrefectureEnshi 445000Hubei ProvinceChina

BACKGROUND Chronic kidney disease(CKD)patients have been found to be at risk of concurrent cognitive dysfunction in previous studies,which has now become an important public health issue of widespread concern.AIM To investigate the risk factors for concurrent cognitive dysfunction in patients with CKD.METHODS This is a prospective cohort study conducted among patients with CKD between October 2021 and March 2023.A questionnaire was formulated by literature review and expert consultation and included questions about age,sex,education level,per capita monthly household income,marital status,living condition,payment method,and hypertension.RESULTS Logistic regression analysis showed that patients aged 60-79 years[odds ratio(OR)=1.561,P=0.015]and≥80 years(OR=1.760,P=0.013),participants with middle to high school education(OR=0.820,P=0.027),divorced or widowed individuals(OR=1.37,P=0.032),self-funded patients(OR=2.368,P=0.008),and patients with hypertension(OR=2.011,P=0.041)had a higher risk of cognitive impairment.The risk of cognitive impairment was lower for those with a college degree(OR=0.435,P=0.034)and married individuals.CONCLUSION The risk factors affecting cognitive dysfunction are age,60-79 years and≥80 years;education,primary school education or less;marital status,divorced or widowed;payment method,selffunded;hypertension;and CKD.

关键词： Cognitive impairment Cognitive dysfunction Chronic kidney disease

Afforestation with an age-sequence of Mongolian pine plantation promotes soil microbial residue accumulation in the Horqin Sandy Land, China

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Journal of Arid Land 2024年第4期16卷 567-579页

作者： GUO Jingwen SONG Xueshu WANG xiao du Zhangliu LU Sen State Key Laboratory of Efficient Production of Forest Resources Research Institute of ForestryChinese Academy of ForestryBeijing 100091China Co-Innovation Center for Sustainable Forestry in Southern China Nanjing Forestry UniversityNanjing 210037China College of Resources and Environmental Sciences China Agricultural UniversityBeijing 100193China

Land use change affects the balance of organic carbon(C)reserves and the global C cycle.Microbial residues are essential constituents of stable soil organic C(SOC).However,it remains unclear how microbial residue changes over time following afforestation.In this study,16-,23-,52-,and 62-year-old Mongolian pine stands and 16-year-old cropland were studied in the Horqin Sandy Land,China.We analyzed changes in SOC,amino sugar content,and microbial parameters to assess how microbial communities influence soil C transformation and preservation.The results showed that SOC storage increased with stand age in the early stage of afforestation but remained unchanged at about 1.27-1.29 kg/m2 after 52 a.Moreover,there were consistent increases in amino sugars and microbial residues with increasing stand age.As stand age increased from 16 to 62 a,soil pH decreased from 6.84 to 5.71,and the concentration of total amino sugars increased from 178.53 to 509.99 mg/kg.A significant negative correlation between soil pH and the concentration of specific and total amino sugars was observed,indicating that the effects of soil acidification promote amino sugar stabilization during afforestation.In contrast to the Mongolian pine plantation of the same age,the cropland accumulated more SOC and microbial residues because of fertilizer application.Across Mongolian pine plantation with different ages,there was no significant change in calculated contribution of bacterial or fungal residues to SOC,suggesting that fungi were consistently the dominant contributors to SOC with increasing time.Our results indicate that afforestation in the Horqin Sandy Land promotes efficient microbial growth and residue accumulation in SOC stocks and has a consistent positive impact on SOC persistence.

关键词： soil organic matter stand age biomarker amino sugars microbial residues

Rare esophageal carcinoma-primary adenoid cystic carcinoma of the esophagus: A case report

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World Journal of Clinical Cases 2024年第3期12卷 630-636页

作者： Li-Dan Geng Jie Li Li Yuan xiao-Bo du Department of Oncology Mianyang Central HospitalMianyang 621000Sichuan ProvinceChina

BACKGROUND Esophageal adenoid cystic carcinoma(EACC)is an exceedingly rare malignant tumor of the esophagus,posing significant challenges in the clinic.CASE SUMMARY This report detailed the case of a 72-year-old male whose diagnosis of EACC was confirmed through postoperative histopathological examination.The patient underwent thoracoscopy-assisted radical resection of the esophageal tumor,coupled with lymph node dissection.Pathological findings revealed an adenoid cystic carcinoma infiltrating the entire layer of the muscularis propria,locally extending into the outer membrane of the esophageal fiber,involving the cardia and exhibiting no lymph node metastasis.The patient’s condition was classified as primary EACC,T3N0M0,per the American Joint Committee on Cancer(2017;8th edition).One month after surgery,the patient received postoperative adjuvant radiation therapy.CONCLUSION In addressing the rarity and high potential for biopsy misdiagnosis of EACC,this study delved into its diagnostic methods and treatment.

关键词： Esophageal adenoid cystic carcinoma Malignant tumor Lymph node dissection Adjuvant radiation therapy Misdiagnosis Case report

A novel AMH variant at the prehelix loop impairs the binding to AMHR2 and causes persistent Mullerian duct syndrome

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Asian Journal of Andrology 2024年第2期26卷 222-224页

作者： Qiang du Chuang Qiu Yuan-Yuan Zhang Xue-Jun Shang xiao-Liang Liu Center of Reproductive Medicine Shengjing Hospital of China Medical UniversityShenyang 110004China Department of Orthopedics Shengjing Hospital of China Medical UniversityShenyang 110004China Department of Clinical Genetics Shengjing Hospital of China Medical UniversityShenyang 110004China Department of Urology Jinling HospitalSchool of MedicineNanjing UniversityNanjing 210012China

Dear Editor,Persistent Mullerian duct syndrome(PMDS;Online Mendelian Inheritance in Man[OMIM]:#261550)is a rare autosomal recessive inherent difference in sex development in males with Mullerian duct derivatives,including the uterus,fallopian tubes,and upper part of the vagina.'Approximately 88%of PMDS patients were found to have biallelic variants in the anti-Mullerian hormone(AMH;OMIM:*600957)or AMH type 2 receptor(AMHR2;OMIM:*600956)genes,and the rest were referred as idiopathic.I The serum AMH levels are usually very low or undetectable in PMDS patients with AMH gene mutations but normal or elevated in those with AMHR2 gene mutations.Here,an adult Chinese patient with PMDS carrying biallelic variants of the AMH gene was reported to have unexpectedly normal serum AMH level.Please refer to Supplementary Information of Patient and Methods for methodologies.

关键词： elevated AMH Muller

Clinical practice guideline for acupuncture and moxibustion:Allergic rhinitis

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Journal of Integrative Medicine 2024年第3期22卷 245-257页

作者： Shi-hao du Sheng Chen Shan-ze Wang Guan-qun Wang Shuo du Wei Guo xiao-long Xie Bi-hui Peng Chao Yang Ji-ping Zhao Institute of Acupuncture and Moxibustion China Academy of Chinese Medical SciencesBeijing 100700China Department of Acupuncture and Moxibustion Dongzhimen HospitalBeijing University of Chinese MedicineBeijing 100700China Department of Acupuncture and Moxibustion Guangdong Provincial Hospital of Chinese MedicineGuangzhou 510120Guangdong ProvinceChina Department of Traditional Chinese Medicine Shanghai Ninth People’s HospitalShanghai Jiao Tong University School of MedicineShanghai 200011China

Acupuncture is one of the most effective complementary therapies for allergic rhinitis(AR)and has been recommended by several clinical practice guidelines(CPGs)for AR.However,these CPGs mentioned acupuncture without making recommendations for clinical implementation and therapeutic protocols,therefore limiting the applicability of acupuncture therapies for AR.Hence,for the benefit of acupuncture practitioners around the world,the World Federation of Acupuncture-moxibustion Societies have initiated a project to develop the CPG for the use of acupuncture and moxibustion to treat AR.This CPG was developed according to the Grading of Recommendations Assessment,Development,and Evaluation(GRADE)methodology,referring to the principles of the World Health Organization Handbook for Guideline Development.during the development of the CPG,the guideline development group(GDG)played an important role.The clinical questions,recommendations and therapeutic protocols were all formulated by the GDG using the modified Delphi method.The CPG contains recommendations for 15 clinical questions about the use of acupuncture and moxibustion interventions.These include one strong recommendation for the intervention based on high-quality evidence,three conditional recommendations for either the intervention or standard care,and 11 conditional recommendations for the intervention based on very low quality of evidence.The CPG also provides one filiform needle acupuncture protocol and five moxibustion protocols extracted based on the protocols presented in randomized controlled trials reviewed by the GDG.

关键词： Clinical practice guideline Allergic rhinitis Acupuncture Moxibustion GRADE