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Type I interferon pathway in pediatric systemic lupus erythematosus

World Journal of Pediatrics 2024年第7期20卷 653-668页

作者： Yu Zhou hong‑mei song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College HospitalBeijingChina

Background The role of type I interferon(IFN-I)signaling in systemic lupus erythematosus(SLE)has been well ***,unanswered questions remain regarding the applicability of these findings to pediatric-onset *** aim of this review is to provide an overview of the novel discoveries on IFN-I signaling in pediatric-onset *** sources A literature search was conducted in the PubMed database using the following keywords:“pediatric systemic lupus erythematosus”and“type I interferon”.Results IFN-I signaling is increased in pediatric SLE,largely due to the presence of plasmacytoid dendritic cells and pathways such as cyclic GMP-AMP synthase–stimulator of interferon genes–TANK-binding kinase 1 and Toll-like receptor(TLR)4/*** extracellular traps and oxidative DNA damage further stimulate IFN-I *** variants in IFN-I-related genes,such as IFN-regulatory factor 5 and tyrosine kinase 2,are linked to SLE susceptibility in pediatric *** addition,type I interferonopathies,characterized by sustained IFN-I activation,can mimic SLE symptoms and are thus important to *** on interferonopathies also contribute to exploring the pathogenesis of *** IFN-I activation is crucial for SLE diagnosis and *** IFNstimulated gene expression and serum IFN-α2 levels are common *** cytometry markers such as CD169 and galectin-9 are promising ***-IFN therapies,such as sifalimumab and anifrolumab,show promise in adult patients with SLE,but their efficacy in pediatric patients requires further *** kinase inhibitors are another treatment option for severe pediatric SLE *** This review presents an overview of the IFN-I pathway in pediatric *** the intricate relationship between IFN-I and pediatric SLE may help to identify potential diagnostic markers and targeted therapies,paving the way for improved patient care and outcomes.

关键词： Interferonopathies Pediatric systemic lupus erythematosus Type I interferon

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Keeping up with the progress in the diagnosis and management of pediatric rheumatic diseases

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World Journal of Pediatrics 2020年第1期16卷 1-4页

作者： hong-mei song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina

Rheumatic diseases are a group of challenging but fascinating disorders affecting multiple organ systems,and can be trickier in the pediatric *** have progressed in this field overtime,as such,the latest knowledge was summarized and presented in this issue,including the basic disease pathogeneses,diagnostic and evaluation tools,and management experiences.

关键词： diseases pediatric diagnosis

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Toward a better understanding of typeⅠinterferonopathies:a brief summary,update and beyond

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World Journal of Pediatrics 2020年第1期16卷 44-51页

作者： Zhong-Xun Yu hong-mei song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina

Backgrounds TypeⅠinterferonopathy is a group of autoinflammatory disorders associated with prominent enhanced typeⅠinterferon *** mechanisms are complex,and the clinical phenotypes are *** review briefly summarized the recent progresses of typeⅠinterferonopathy focusing on the clinical and molecular features,pathogeneses,diagnoses and potential *** sources Original research articles and literature reviews published in PubMed-indexed *** TypeⅠinterferonopathies include Aicardi-Goutières syndrome,spondyloenchondro-dysplasia with immune dysregulation,stimulator of interferon genes-associated vasculopathy with onset in infancy,X-linked reticulate pigmentary disorder,ubiquitin-specific peptidase 18 deficiency,chronic atypical neutrophilic dermatitis with lipodystrophy,and Singleton-Merten syndrome *** disorders including interferon-stimulated gene 15 deficiency and DNAseⅡdeficiency are believed to be interferonopathies as *** calcification,skin vasculopathy,interstitial lung disease,failure to thrive,skeletal development problems and autoimmune features are *** responses to nucleic acid stimuli and defective regulation of protein degradation are main mechanisms in disease *** generation Janus kinase inhibitors including baricitinib,tofacitinib and ruxolitinib are useful for disease *** transcriptase inhibitors seem to be another option for Aicardi-Goutières *** Tremendous progress has been made for the discovery of typeⅠinterferonopathies and responsible *** kinase inhibitors and other agents have potential therapeutic *** basic,translational and clinical studies towards disease monitoring and powerful therapies are warranted.

关键词： Hereditary autoinflammatory diseases Interferon typeⅠ Janus kinase inhibitors

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Preparation of Exfoliated Low-density Polyethylene/Montmorillonite Nanocomposites Through Melt Extrusion

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Chemical Research in Chinese Universities 2006年第3期22卷 383-387页

作者： YANG hong-mei song Yi-hu XU Bo ZHENG Qiang Department of Polymer Science ＆ Engineering Key Laboratory of Macromolecule Synthesis and Functionalization of MOE Zhejiang University Hangzhou 310027 P. R. China

The effects of ethylene vinyl acetate copelymer （EVA） as a compatibilizer on the dispersion of organically modified montmorillonite（org-MMT） into low-density polyethylene（LDPE） during melt extrusion compounding were studied. The X-ray diffraction patterns reveal that as compared with LDPE, EVA can intercalate more easily into the interlay gallery of org-MMT when the composites contain a low org-MMT content. Exfoliated LDPE/org-MMT nanocompesites in the presence of an EVA compatibilizer could be prepared by using a two-step melt compounding technique with a twin-screw extruder.

关键词： Polyethylene Montmorillonite Nanocompesite Intercalation Exfoliation

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Acute severe hepatitis of unknown origin in children:considerations from the perspective of immunology

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World Journal of Pediatrics 2022年第8期18卷 529-532页

作者： Si‑Hao Gao Meng‑Chun Gong hong‑mei song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina Institute of Health Management Southern Medical UniversityGuangzhouChina

Acute severe hepatitis of unknown origin in children,since it was frstly reported in Scotland on March 31,2022,has become an international concern with a high risk of acute liver failure requiring liver *** typical causes for hepatitis have been ruled out and investigations revealed possible relationship between the disease and two potential pathogens,a special form of human adenovirus(HAdV)and severe acute respiratory syndrome coronavirus 2(SARSCoV-2).Acute hepatitis has been reported in children with multisystem infammatory syndrome(MIS-C)and incorporating insights from the immunology perspective is necessary to fully understand this disease.

关键词： Acute acute hepatitis

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The clinical phenotype and genotype of NLRP12-autoinflammatory disease: a Chinese case series with literature review

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World Journal of Pediatrics 2020年第5期16卷 514-519页

作者： Wei Wang Yu Zhou Lin-Qing Zhong Zhuo Li Shan Jian Xiao-Yan Tang hong-mei song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina School of Medicine Tsinghua UniversityBeijingChina

Background The nucleotide-binding oligomerization domain-like receptor protein 12(NLRP12)-autoinflammatory disorder(NLRP12-AD)is a rare autoinflammatory disease characterized by recurrent fever,rash as well as musculoskeletal symptoms,which is rarely reported in Asian *** Three cases of NLRP12-AD presented to our hospital were studied after parental consents were *** presentations were recorded on a standardized case report *** of NLRP12 were detected by primary immunodeficiency disease panels and further examined by Sanger *** literature search for relevant studies of systemic autoinflammatory disorders,especially NLRP12-AD between January,2000 and January,2019 was carried and the clinical data were *** were made between groups in terms of onset age and of *** All our patients presented with fever,rash and arthritis/arthralgia,and sensorineural as well as sensorineural deafness(1/3),uveitis(1/3),abdominal pain(1/3),and myalgia(1/3).Two novel mutation variations,p.W581X and p.L558R,are reported *** addition,we also found that two patients inherited the mutated alleles from their healthy parents,and this may be evidence of *** Although the genotypes are similar,the clinical manifestations between Chinese patients and Western patients vary thus highlighting the possible influence of ethnic and environmental *** the other hand,some genetic mutations may lead to specific phenotype,as we have found a high prevalence of sensorineural hearing loss among p.R284X patients.

关键词： Autoinflammatory disease NLRP12-AD Recurrent fever

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Matrix metalloproteinase-9 was involved in the immuno-modulatory defect of mesenchymal stem cell from chronic myeloid leukemia patients

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Chinese Medical Journal 2011年第16期124卷 2423-2430页

作者： ZHU Xi-shan SHI Wei AN Guang-yu ZHANG hong-mei song Yu-guang LI You-bin Institute of Internal Oncology Center of Tumor Research andTherapy Beijing Shijitan Hospital Capital Medical University (theNinth Medical College of Peking University) Beijing 100038China

Background Overwhelming evidences on chronic myeloid leukemia （CML） indicate that patients harbor quiescent CML stem cells that are responsible for blast crisis. While the hematopoietic stem cell （HSC） origin of CML was first suggested over 30 years ago, recently CML-initiating cells beyond HSCs are also being investigated. Methods We have previously isolated fetal liver kinase-l-positive （Flkl＋） cells carrying the BCR/ABL fusion gene from the bone marrow of Ph＋ patients with hemangioblast property. In this study, we isolated CML patient-derived FIk1＋CD31-CD34- mesenchymal stem cells （MSCs） and detected their biological characteristics and immunological regulation using fluorescence in situ hybridization （FISH） analysis, fluorescence activated cell sorting （FACS）, enzyme-linked immunoadsorbent assay, mixed lymphocyte reaction assays; then we compared these characters with those of the healthy donors. Results CML patient-derived FIk1＋CD31-CD34- MSCs had normal morphology, phenotype and karyotype while appeared impaired in immuno-modulatory function. The capacity of patient FIk1＋CD31-CD34- MSCs to inhibit T lymphocyte activation and proliferation was impaired in vitro. Conclusions CML patient-derived MSCs have impaired immuno-modulatory functions, suggesting that the dysregulation of hematopoiesis and immune response may originate from MSCs rather than hematopoietic stem cells （HSCs）. MSCs might be a potential target for developing efficacious treatment for CML.

关键词： chronic myeloid leukemia mesenchymal stem cell immune regulation matrix metalloproteinase-9

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Analysis of clinical characteristics of children with Aicardi-Goutieres syndrome in China

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World Journal of Pediatrics 2022年第7期18卷 490-497页

作者： Wei Wang Ting-Yan He Li-Ping Zou Wen-Dao Li Zhong-Xun Yu Ming-Sheng Ma Jun Yang hong-mei song Peking Union Medical College Hospital.Chinese Academy of Medical Sciences Peking Union Medical CollegeNo.1 Shuaifuyuan WangfujingDongcheng DistrictBeijing 100730China Shenzhen Children's Hospital No.7019 Yitian RoadFutian DistrictShenzhen 518048China The General Hospital of the People's Liberation Army No.28 Fuxing RoadHaidian DistrictBeijing 10000China

Background Aicardi-Goutieres syndrome(AGS)is an inflammatory disorder belonging to the type I interferonopathy *** clinical diagnosis of AGS is difficult,which can lead to a high mortality ***,there is a lack of large-sample research data on AGS in *** aim to summarize the clinical characteristics of Chinese patients with AGS and provide clues for clinical *** The genetic and clinical features of Chinese patients with AGS were ***-time polymerase chain reac-tion was used to detect expression of interferon-stimulated genes(ISGs).Results A total of 23 cases were included,consisting of 7 cases of AGS1 with three prime repair exonuclease 1 mutations,3 of AGS2 with ribonuclease H2 subunit B(RNASEH2B)mutations,3 of ASG3 with RNASEH2C,1 of AGS4 with RNASEH2A mutations,2 of AGS6 with adenosine deaminase acting on RNA 1 mutations,and 7 of AGS7 with interferon induced with helicase C domain 1 *** before the age of 3 years occurred in 82.6%.Neurologic involvement was most common(100%),including signs of intracranial calcification which mainly distributed in the bilateral basal ganglia,leukodystrophy,dystonia,epilepsy,brain atrophy and *** disability,language disability and motor skill impairment were also *** manifestations(60.87%)were dominated by a chilblain-like *** such as microcephaly(47.62%),short stature(52.38%),liver dysfunction(42.11%),thyroid dysfunction(46.15%),positive autoimmune antibod-ies(66.67%),and elevated erythrocyte sedimentation rate(53.85%)were also *** phenotypes of 2 cases fulfilled the diagnostic criteria for systemic lupus erythaematosus(SLE).One death was *** expression were *** AGS is a systemic disease that causes sequelae and mortality.A diagnosis of AGS should be considered for patients who have an early onset of chilblain-like rash,intracranial calcification,leukodystrophy,dystonia,developmental delay,positive autoimm

关键词： Aicardi-Goutieres syndrome Chinese Diagnosis Manifestations

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Characterization of microbiota in systemic-onset juvenile idiopathic arthritis with different disease severities

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World Journal of Clinical Cases 2019年第18期7卷 2734-2745页

作者： Yan-Qing Dong Wei Wang Ji Li Ming-Sheng Ma Lin-Qing Zhong Qi-Jiao Wei hong-mei song Department of Pediatrics Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College

BACKGROUND Systemic-onset juvenile idiopathic arthritis (SoJIA) is one of most serious subtypes of juvenile idiopathic arthritis. Although the pathogenesis of SoJIA remains unclear, several studies have suggested a correlation between gut dysbiosis and JIA. Further understanding of the intestinal microbiome may help to establish alternative ways to treat, or even prevent, the disease. AIM To explore alterations in fecal microbiota profiles in SoJIA patients and to evaluate the correlations between microbiota and clinical parameters. METHODS We conducted an observational single-center study at the Pediatric Department of Peking Union Medical College Hospital. Children who were diagnosed with SoJIA at our institution and followed for a minimum period of six months after diagnosis were recruited for the study. Healthy children were recruited as a control group (HS group) during the same period. Clinical data and stool samples were collected from SoJIA patients when they visited the hospital. RESULTS The SoJIA group included 17 active and 15 inactive consecutively recruited children;the control group consisted of 32 children. Firmicutes and Bacteroidetes were the two most abundant phyla among the total sample of SoJIA children and controls. There was a significant difference among the three groups in observed species, which was the highest in the Active-SoJIA group, followed by the Inactive-SoJIA group and then HS group (Active-SoJIA vs HS: P = 0.000;and Inactive-SoJIA vs HS: P = 0.005). We observed a lower Firmicutes/Bacteroidetes ratio in SoJIA patients (3.28 ± 4.47 in Active-SoJIA, 5.36 ± 8.39 in Inactive-SoJIA,and 5.67 ± 3.92 in HS). We also observed decreased abundances of Ruminococcaceae (14.9% in Active-SoJIA, 17.3% in Inactive-SoJIA, and 22.8% in HS;Active-SoJIA vs HS: P = 0.005) and Faecalibacterium (5.1% in Active-SoJIA, 9.9% in Inactive-SoJIA, and 13.0% in HS;Active-SoJIA vs HS: P = 0.000) in SoJIA compared with HS. By contrast, the abundance of Bacteroidacea

关键词： Microbiota Systemic-onset juvenile idiopathic arthritis Disease activity Dysbiosis

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Beyond surgery:Overcoming postoperative depression in cancer patients

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World Journal of Psychiatry 2024年第7期14卷 1140-1142页

作者： Shao-Ming song Xiao Wang hong-mei Yue Rong Liu The First School of Clinical Medicine Lanzhou UniversityLanzhou 730000Gansu ProvinceChina Faculty of Hepato-pancreato-biliary Surgery The First Medical Center of PLA General HospitalBeijing 100853China

Depression is a common occurrence among cancer patients,and it significantly impacts their clinical outcomes and quality of life,with a high incidence during anti-tumor treatment or after *** association between surgery and depression is the result of the interaction of various factors,including physio-logical,psychological,and social factors,all of which are intertwined and make patients susceptible to depression after surgical *** depression has a significant negative impact on many aspects of cancer patients,and it requires timely identification and intervention to improve the overall outcome.

关键词： Depression Surgery Tumor Anti-tumor therapy

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