1.Clinical analysis of 6 cases of Mycoplasma pneumoniae-induced rash and mucositis in children
Ting SONG ; Hui HU ; Ronghua YU ; Pei XIAO ; Xiaolu LI ; Ting ZHANG ; Yongmei XIAO
Chinese Journal of Pediatrics 2025;63(2):190-194
Objective:To summarize the clinical features, laboratory findings, treatment and prognosis of children confirmed as Mycoplasma pneumoniae-induced rash and mucositis (MIRM) in children. Methods:This retrospective study concluded 6 children diagnosed as MIRM in Department of Gastroenterology and Infectious Diseases, Shanghai Children′s Hospital, School of Medicine, Shanghai Jiao Tong University from August 2023 to April 2024. This paper described the characteristics of MIRM and analyzed the therapeutic strategy and prognosis.Results:A total of 6 children were diagnosed as MIRM including 2 boys and 4 girls with an age of onset was 6.4 (3.1, 7.5) years. Among the 6 patients, 4 patients had oral mucosal involvement among whom 2 showed crusting of the lips. Four patients had ocular involvement manifesting as conjunctival congestion and increased secretion. All patients presented with skin lesions, manifesting as target-shaped damage in 4 cases, herpes herpetiformis in 1 case and purpura-like rash in 1 case. Serological tests for Mycoplasma pneumoniae IgM and Mycoplasma pneumoniae nucleic acid test were positive in all 6 cases. Two cases received intravenous immunogloblin infusion combined with methylprednisolone, monotherapy of methylprednisolone in 4 cases. The course of glucocorticoids was 1-7 weeks, and the initial dose was 2-4 mg/(kg·d), which was gradually reduced according to the rash. The children were followed up for 3 to 9 months, no case suffered from long term ocular or cutaneous complications or recurrence of rash. All cases had good prognosis. Conclusions:Children diagnosed as MIRM present with mild symptoms and usually have good prognosis with early identification and appropriate intervention. Individualized therapy should be applied based on the severity of skin involvement.
3.USP20 as a super-enhancer-regulated gene drives T-ALL progression via HIF1A deubiquitination.
Ling XU ; Zimu ZHANG ; Juanjuan YU ; Tongting JI ; Jia CHENG ; Xiaodong FEI ; Xinran CHU ; Yanfang TAO ; Yan XU ; Pengju YANG ; Wenyuan LIU ; Gen LI ; Yongping ZHANG ; Yan LI ; Fenli ZHANG ; Ying YANG ; Bi ZHOU ; Yumeng WU ; Zhongling WEI ; Yanling CHEN ; Jianwei WANG ; Di WU ; Xiaolu LI ; Yang YANG ; Guanghui QIAN ; Hongli YIN ; Shuiyan WU ; Shuqi ZHANG ; Dan LIU ; Jun-Jie FAN ; Lei SHI ; Xiaodong WANG ; Shaoyan HU ; Jun LU ; Jian PAN
Acta Pharmaceutica Sinica B 2025;15(9):4751-4771
T-cell acute lymphoblastic leukemia (T-ALL) is a highly aggressive hematologic malignancy with a poor prognosis, despite advancements in treatment. Many patients struggle with relapse or refractory disease. Investigating the role of the super-enhancer (SE) regulated gene ubiquitin-specific protease 20 (USP20) in T-ALL could enhance targeted therapies and improve clinical outcomes. Analysis of histone H3 lysine 27 acetylation (H3K27ac) chromatin immunoprecipitation sequencing (ChIP-seq) data from six T-ALL cell lines and seven pediatric samples identified USP20 as an SE-regulated driver gene. Utilizing the Cancer Cell Line Encyclopedia (CCLE) and BloodSpot databases, it was found that USP20 is specifically highly expressed in T-ALL. Knocking down USP20 with short hairpin RNA (shRNA) increased apoptosis and inhibited proliferation in T-ALL cells. In vivo studies showed that USP20 knockdown reduced tumor growth and improved survival. The USP20 inhibitor GSK2643943A demonstrated similar anti-tumor effects. Mass spectrometry, RNA-Seq, and immunoprecipitation revealed that USP20 interacted with hypoxia-inducible factor 1 subunit alpha (HIF1A) and stabilized it by deubiquitination. Cleavage under targets and tagmentation (CUT&Tag) results indicated that USP20 co-localized with HIF1A, jointly modulating target genes in T-ALL. This study identifies USP20 as a therapeutic target in T-ALL and suggests GSK2643943A as a potential treatment strategy.
4.Relationship between serum sFlt-1,MCP-1 and sLOX-1 levels and the disease state and prognosis in patients with coronary heart disease
Guoling HU ; Lijie JIANG ; Tianjiao ZHANG ; Xiaolu HOU
International Journal of Laboratory Medicine 2025;46(18):2237-2242
Objective To investigate the relationship between the levels of soluble FMS-like tyrosine ki-nase-1(sFlt-1),monocyte chemoatgulant protein-1(MCP-1),and soluble lectin-like oxidized low-density lipo-protein receptor 1(sLOX-1)in the serum of patients with coronary heart disease and the disease state and prognosis.Methods A total of 126 patients with coronary heart disease treated in Zhongshan Hospital,Dalian University from May 2022 to May 2024 were selected as study objects.The patients were divided into good prognosis group(n=78)and poor prognosis group(n=48)according to prognostic results.The serum sFlt-1,MCP-1 and sLOX-1 levels were detected by enzyme-linked immunosorbent assay.The degree of coronary artery stenosis in patients was measured by coronary angiography,as indicated by the Gensini score.Spearman correlation analysis was used to analyze the correlation between Gensini score and serum sFlt-1,MCP-1 and sLOX-1 levels.Multivariate Logistic regression was used to analyze the factors affecting the prognosis of pa-tients with coronary heart disease.The predictive efficacy of serum sFlt-1,MCP-1 and sLOX-1 levels for the prognosis of patients with coronary heart disease was evaluated by receiver operating characteristic(ROC)curve.Results The age in the poor prognosis group was older than that in the good prognosis group(P<0.05),Gensini score was higher than that in the good prognosis group(P<0.05).Compared with the poor prognosis group,the serum sFlt-1,MCP-1 and sLOX-1 levels in the good prognosis group were lower(P<0.05).Gensini score was positively correlated with the serum sFlt-1,MCP-1 and sLOX-1 levels.Multivariate Logistic regression analysis showed that old age,high Gensini score,high sFlt-1 level,high MCP-1 level and high sLOX-1 level were all risk factors effecting the prognosis of patients with coronary heart disease(P<0.05).ROC curve analysis showed that the areas under the curve(AUC)of serum sFlt-1,MCP-1,and sLOX-1 for predicting the prognosis of coronary heart disease patients were 0.787(95%CI:0.703-0.870),0.815(95%CI:0.741-0.890)and 0.795(95%CI:0.715-0.876),respectively.The AUC for predicting the prognosis of coronary heart disease patients using a combination of three was 0.923(95%CI:0.876-0.970).Conclusion The serum levels of sFlt-1,MCP-1 and sLOX-1 in patients with coronary heart disease are signifi-cantly increased,and are positively correlated with the degree of coronary artery stenosis.The levels of sFlt-1,MCP-1 and sLOX-1 in serum have a certain predictive effect on the prognosis of patients with coronary heart disease.
5.Intrinsic specific TGF-β signaling in myofibers attenuates mouse acute skeletal myositis via LRP1
Qihui CAI ; Haiqiang LAN ; Bojun XIAN ; Nan WANG ; Xiaolei HUANG ; Xiaolu NIU ; Xinyu HU ; Chen LI ; Junyi XIE ; Zhaohong LIAO
Chinese Journal of Pathophysiology 2025;41(7):1365-1374
AIM:To investigate the effect of intrinsic specific transforming growth factor-β(TGF-β)signaling on regeneration and repair of myofibers in acute skeletal myositismice model induced by cardiotoxin(CTX).METHODS:One hundred and eighty-six wild C57BL/6 mice and one hundred and thirty-eight mice with conditional knockout of TGF-β receptor 2(TGF-βr2)in myofibers(SM TGF-βr2-/-mice)were selected.CTX injection to anterior tibial muscle(TA)in-duced acute myoinjury in mice.Some SM TGF-βr2-/-mice were given Smad signaling agonist SRI-011381(SRI)intramus-cular injection.All mice were mainly divided into the following groups:control group,SM TGF-βr2-/-group and SM TGF-βr2-/-+SRI group.Twenty-four mice were selected in each group.RT-qPCR and immunofluorescence staining were used to detect the relative mRNA level,protein expression of inflammatory cytokines and low-density lipoprotein receptor-related protein 1(LRP1),respectively,while the relative protein expression of myosin heavy chain 3(MHY3)and embryonic myosine heavy chain(eMHC)in damaged muscle was detected by Western blot and immunofluorescence staining.In vi-tro,after being extracted from neonatal mice,myogenic precursor cells(MPCs)were cultured in an pro-inflammatory mi-lieu and treated with SRI,recombinant mouse extracellular matrix protein 1(rmECM1)alone or in combination.Hereby,they were divided into the following seven groups:control-MPCs group,control-MPCs+LPS group,TGF-βr2-/--MPCs group,TGF-βr2-/--MPCs+LPS group,TGF-βr2-/--MPCs+LPS+SRI group,TGF-βr2-/--MPCs+LPS+rmECM1 group,and TGF-βr2-/--MPCs+LPS+SRI+rmECM1 group.Six mice were selected in each group.RT-qPCR and Western blot were used to detect the relative mRNA level,protein expression of major histocompatibility complex class I molecules(MHC-I/H-2Kb),major histocompatibility complex class II molecules(MHC-II/H2-Eα),Toll-like receptor 3(TLR3),and LRP1.And the relative protein expression of MoyD and myogenin in myotubes was detected by immunofluorescence staining.RE-SULTS:In vivo,compared with control group,SM TGF-βr2-/-group showed the significant upregulation of pro-inflamma-tory cytokines(P<0.05),and the opposite trend of anti-inflammatory cytokines,LRP1,MHY3,eMHC in the injured muscle(P<0.05),with delayed regeneration and repair of myofibers.In vitro,compared with control-MPCs+LPS group,LRP1,MoyD and myogenin significantly downregulated in TGF-βr2-/--MPCs+LPS group,but the downregulation trend was corrected after giving SRI treatment(P<0.05).In addition,compared with the TGF-βr2-/--MPCs+LPS group,the combi-nation of rmECM1 and SRI significantly upregulated the protein expression of MyoD and myogenin(P<0.05).CONCLU-SION:In a mouse model of acute skeletal myositis,intrinsic TGF-β signaling specifically in myofibers regulates local im-mune behavior.It promotes the expression of LRP1 in damaged muscle via Smad2/3 signaling,and LRP1 can then fully bind to ECM1,thereby facilitating muscle regeneration and repair,and improving the prognosis of acute skeletal myositis.
6.Application of transition period readiness assessment questionnaire in the transition from adolescence to adulthood of children with inflammatory bowel disease
Guoxin JIANG ; Xiaolu NIE ; Yuan MENG ; Fang HU ; Jie WU
Chinese Pediatric Emergency Medicine 2025;32(4):264-272
Objective:To study the current status of transitional readiness from adolescence to adulthood of patients with inflammatory bowel disease(IBD)in China,analyze the factors affecting transitional readiness,and provide a basis for developing scientific and targeted transitional preparedness programmes.Methods:The IBD patients aged 12-24 years who were admitted to the Department of Gastroenterology,Beijing Children's Hospital Affiliated to Capital Medical University from January 1,2016 to January 15,2025 were enrolled.From November 1,2024 to January 15,2025,the survey was conducted through online distribution of the transition readiness assessment questionnaire (TRAQ)6.0 Chinese version scale to IBD patients by using the convenient sampling method.The scores of each dimension of TRAQ scale were calculated,and multiple linear regression analysis was used to analyze the factors influencing the scores of TRAQ during the transitional period.Results:A total of 184 patients were included and 184 questionnaires were collected.Male patients were slightly more than female(109:75),with an average age of (14.94±2.35) years.70.11% of patients had a disease duration of 1 to 5 years,with Crohn's disease accounting for the highest proportion(54.89%),and the severity of the disease was mostly mild to moderate.Family support was generally higher(84.24%).Parents were highly involved in patient disease management(94.02%).There were 52.72% of the patients received social support.The mean score of the TRAQ was 4.14 ± 0.89,and the mean scores of the four dimensions from high to low were following health problems(4.28±0.97),insisting on making an appointment and seeing a doctor on time(4.20±1.10),talking with medical staff(4.17±1.05) and drug management(4.14±0.99).Multiple linear regression analysis showed that family status,the encourage,supports and help from the family members,and the supports from the society were the independent influencing factors of IBD patients' readiness score for transition from adolescence to adulthood( P<0.05).Among them,family status had a positive effect on transition readiness score,and the degree of readiness score increased with the increase of harmony( P<0.05).Social support also had a positive effect on transition readiness score( P<0.05).Compared with no encouragement,support and help from family members,the situation of little encouragement had a certain improvement on the readiness score( P<0.05). Conclusion:Children with IBD have better overall performance during the transition from adolescence to adulthood.Family status,the encourage,supports and help from the family members,and the supports from the society are the important factors influencing transitional preparedness.
7.Research on cultural adaptation in the Chinese version of the inflammatory bowel disease self-efficacy scale for adolescents and young adults
Yuan MENG ; Xiaolu NIE ; Xin WANG ; Fang HU ; Siyu CAI ; Zhaoxia WANG ; Xuemei ZHONG ; Jie WU
Chinese Pediatric Emergency Medicine 2025;32(5):341-346
Objective:By using cognitive interviews,the interviewees' cognition and understanding of the inflammatory bowel disease(IBD) self-efficacy scale for adolescents and young adults (IBDSES-A) were evaluated,and the semantic content of IBDSES-A,which was initially translated into Chinese,was tested and revised.Methods:Using purposive sampling,15 IBD patients aged 12-18 were selected from Beijing Children's Hospital,Capital Medical University,between January and February 2025,stratified by age group and disease type.Two rounds of cognitive interviews were conducted.Feedback and suggestions from interviewees were analyzed using a question appraisal system for coding and integration.Based on expert panel discussions,ambiguous items were revised to finalize the Chinese version of the IBDSES-A.Results:In the first round,10 interviewees were interviewed,followed by 5 interviewees in the second round.There were no statistically significant differences ( P>0.05) between the interviewees of two rounds in terms of age,gender,and education level.During the first round of interview,interviewees expressed comprehension difficulties with 76.9% (10/13) of the items.Coding analysis revealed that the primary issue was "clarification",as unclear wording made it difficult for interviewees to fully grasp the intended meaning of certain items.Based on these findings,the expert panel revised 10 of the 13 items in the IBDSES-A.The second round of cognitive interview showed that the interviewees generally understood the revised items,achieving linguistic and semantic consistency with the original scale. Conclusion:The application of cognitive interviews in the translation process of the IBDSES-A helps reduce comprehension biases caused by inappropriate wording,ensuring that the localized version of the scale is more accessible and understandable to the target population.
8.Intrinsic specific TGF-β signaling in myofibers attenuates mouse acute skeletal myositis via LRP1
Qihui CAI ; Haiqiang LAN ; Bojun XIAN ; Nan WANG ; Xiaolei HUANG ; Xiaolu NIU ; Xinyu HU ; Chen LI ; Junyi XIE ; Zhaohong LIAO
Chinese Journal of Pathophysiology 2025;41(7):1365-1374
AIM:To investigate the effect of intrinsic specific transforming growth factor-β(TGF-β)signaling on regeneration and repair of myofibers in acute skeletal myositismice model induced by cardiotoxin(CTX).METHODS:One hundred and eighty-six wild C57BL/6 mice and one hundred and thirty-eight mice with conditional knockout of TGF-β receptor 2(TGF-βr2)in myofibers(SM TGF-βr2-/-mice)were selected.CTX injection to anterior tibial muscle(TA)in-duced acute myoinjury in mice.Some SM TGF-βr2-/-mice were given Smad signaling agonist SRI-011381(SRI)intramus-cular injection.All mice were mainly divided into the following groups:control group,SM TGF-βr2-/-group and SM TGF-βr2-/-+SRI group.Twenty-four mice were selected in each group.RT-qPCR and immunofluorescence staining were used to detect the relative mRNA level,protein expression of inflammatory cytokines and low-density lipoprotein receptor-related protein 1(LRP1),respectively,while the relative protein expression of myosin heavy chain 3(MHY3)and embryonic myosine heavy chain(eMHC)in damaged muscle was detected by Western blot and immunofluorescence staining.In vi-tro,after being extracted from neonatal mice,myogenic precursor cells(MPCs)were cultured in an pro-inflammatory mi-lieu and treated with SRI,recombinant mouse extracellular matrix protein 1(rmECM1)alone or in combination.Hereby,they were divided into the following seven groups:control-MPCs group,control-MPCs+LPS group,TGF-βr2-/--MPCs group,TGF-βr2-/--MPCs+LPS group,TGF-βr2-/--MPCs+LPS+SRI group,TGF-βr2-/--MPCs+LPS+rmECM1 group,and TGF-βr2-/--MPCs+LPS+SRI+rmECM1 group.Six mice were selected in each group.RT-qPCR and Western blot were used to detect the relative mRNA level,protein expression of major histocompatibility complex class I molecules(MHC-I/H-2Kb),major histocompatibility complex class II molecules(MHC-II/H2-Eα),Toll-like receptor 3(TLR3),and LRP1.And the relative protein expression of MoyD and myogenin in myotubes was detected by immunofluorescence staining.RE-SULTS:In vivo,compared with control group,SM TGF-βr2-/-group showed the significant upregulation of pro-inflamma-tory cytokines(P<0.05),and the opposite trend of anti-inflammatory cytokines,LRP1,MHY3,eMHC in the injured muscle(P<0.05),with delayed regeneration and repair of myofibers.In vitro,compared with control-MPCs+LPS group,LRP1,MoyD and myogenin significantly downregulated in TGF-βr2-/--MPCs+LPS group,but the downregulation trend was corrected after giving SRI treatment(P<0.05).In addition,compared with the TGF-βr2-/--MPCs+LPS group,the combi-nation of rmECM1 and SRI significantly upregulated the protein expression of MyoD and myogenin(P<0.05).CONCLU-SION:In a mouse model of acute skeletal myositis,intrinsic TGF-β signaling specifically in myofibers regulates local im-mune behavior.It promotes the expression of LRP1 in damaged muscle via Smad2/3 signaling,and LRP1 can then fully bind to ECM1,thereby facilitating muscle regeneration and repair,and improving the prognosis of acute skeletal myositis.
9.Application of transition period readiness assessment questionnaire in the transition from adolescence to adulthood of children with inflammatory bowel disease
Guoxin JIANG ; Xiaolu NIE ; Yuan MENG ; Fang HU ; Jie WU
Chinese Pediatric Emergency Medicine 2025;32(4):264-272
Objective:To study the current status of transitional readiness from adolescence to adulthood of patients with inflammatory bowel disease(IBD)in China,analyze the factors affecting transitional readiness,and provide a basis for developing scientific and targeted transitional preparedness programmes.Methods:The IBD patients aged 12-24 years who were admitted to the Department of Gastroenterology,Beijing Children's Hospital Affiliated to Capital Medical University from January 1,2016 to January 15,2025 were enrolled.From November 1,2024 to January 15,2025,the survey was conducted through online distribution of the transition readiness assessment questionnaire (TRAQ)6.0 Chinese version scale to IBD patients by using the convenient sampling method.The scores of each dimension of TRAQ scale were calculated,and multiple linear regression analysis was used to analyze the factors influencing the scores of TRAQ during the transitional period.Results:A total of 184 patients were included and 184 questionnaires were collected.Male patients were slightly more than female(109:75),with an average age of (14.94±2.35) years.70.11% of patients had a disease duration of 1 to 5 years,with Crohn's disease accounting for the highest proportion(54.89%),and the severity of the disease was mostly mild to moderate.Family support was generally higher(84.24%).Parents were highly involved in patient disease management(94.02%).There were 52.72% of the patients received social support.The mean score of the TRAQ was 4.14 ± 0.89,and the mean scores of the four dimensions from high to low were following health problems(4.28±0.97),insisting on making an appointment and seeing a doctor on time(4.20±1.10),talking with medical staff(4.17±1.05) and drug management(4.14±0.99).Multiple linear regression analysis showed that family status,the encourage,supports and help from the family members,and the supports from the society were the independent influencing factors of IBD patients' readiness score for transition from adolescence to adulthood( P<0.05).Among them,family status had a positive effect on transition readiness score,and the degree of readiness score increased with the increase of harmony( P<0.05).Social support also had a positive effect on transition readiness score( P<0.05).Compared with no encouragement,support and help from family members,the situation of little encouragement had a certain improvement on the readiness score( P<0.05). Conclusion:Children with IBD have better overall performance during the transition from adolescence to adulthood.Family status,the encourage,supports and help from the family members,and the supports from the society are the important factors influencing transitional preparedness.
10.Research on cultural adaptation in the Chinese version of the inflammatory bowel disease self-efficacy scale for adolescents and young adults
Yuan MENG ; Xiaolu NIE ; Xin WANG ; Fang HU ; Siyu CAI ; Zhaoxia WANG ; Xuemei ZHONG ; Jie WU
Chinese Pediatric Emergency Medicine 2025;32(5):341-346
Objective:By using cognitive interviews,the interviewees' cognition and understanding of the inflammatory bowel disease(IBD) self-efficacy scale for adolescents and young adults (IBDSES-A) were evaluated,and the semantic content of IBDSES-A,which was initially translated into Chinese,was tested and revised.Methods:Using purposive sampling,15 IBD patients aged 12-18 were selected from Beijing Children's Hospital,Capital Medical University,between January and February 2025,stratified by age group and disease type.Two rounds of cognitive interviews were conducted.Feedback and suggestions from interviewees were analyzed using a question appraisal system for coding and integration.Based on expert panel discussions,ambiguous items were revised to finalize the Chinese version of the IBDSES-A.Results:In the first round,10 interviewees were interviewed,followed by 5 interviewees in the second round.There were no statistically significant differences ( P>0.05) between the interviewees of two rounds in terms of age,gender,and education level.During the first round of interview,interviewees expressed comprehension difficulties with 76.9% (10/13) of the items.Coding analysis revealed that the primary issue was "clarification",as unclear wording made it difficult for interviewees to fully grasp the intended meaning of certain items.Based on these findings,the expert panel revised 10 of the 13 items in the IBDSES-A.The second round of cognitive interview showed that the interviewees generally understood the revised items,achieving linguistic and semantic consistency with the original scale. Conclusion:The application of cognitive interviews in the translation process of the IBDSES-A helps reduce comprehension biases caused by inappropriate wording,ensuring that the localized version of the scale is more accessible and understandable to the target population.

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