Objective:To analyze the clinical characteristics and treatment outcomes of children with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody-mediated necrotizing myopathy, and to explore early identification and management strategies to provide reference for clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the clinical data and treatment outcomes of 10 pediatric patients with anti-HMGCR antibody-mediated necrotizing myopathy admitted to the Department of Rheumatology, Children′s Hospital of Fudan University from December 2020 to December 2024. Statistical description was performed using SPSS 22.0.Results:Among the 10 patients, the male-to-female ratio was 1:4, the age of onset was (7.2±4.0) years, and the disease duration at diagnosis was (22.2±19.6) months. None had a history of statin exposure. Six patients presented with muscle weakness, and4 were diagnosed due to asymptomatic elevation of creatine kinase (CK); 4 had dermatomyositis-like rashes. All patients showed significantly elevated CK levels [median 3 291(1 969, 8 776)U/L] and underwent muscle biopsy. Histopathological findings revealed myofiber degeneration, necrosis, and regeneration in all cases, with inflammatory infiltration in 9 cases, MHC-Ⅰ positivity in all, and C5b-9 positivity in 9 cases. The median follow-up duration was (15.7±6.3) months. At the last follow-up, muscle strength was normal or nearly normal, and the CK median value had decreased to 977.5 (211.0, 3 536.0) U/L.Conclusion:For patients with suspected idiopathic inflammatory myopathy and significantly elevated CK, muscle-specific antibody testing-including anti-HMGCR-and muscle biopsy should be performed promptly regardless of the presence of skin rash, to ensure accurate diagnosis and guide treatment, thereby avoiding misdiagnosis or missed diagnosis.

Objective:To analyze the clinical characteristics and treatment outcomes of children with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody-mediated necrotizing myopathy, and to explore early identification and management strategies to provide reference for clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the clinical data and treatment outcomes of 10 pediatric patients with anti-HMGCR antibody-mediated necrotizing myopathy admitted to the Department of Rheumatology, Children′s Hospital of Fudan University from December 2020 to December 2024. Statistical description was performed using SPSS 22.0.Results:Among the 10 patients, the male-to-female ratio was 1:4, the age of onset was (7.2±4.0) years, and the disease duration at diagnosis was (22.2±19.6) months. None had a history of statin exposure. Six patients presented with muscle weakness, and4 were diagnosed due to asymptomatic elevation of creatine kinase (CK); 4 had dermatomyositis-like rashes. All patients showed significantly elevated CK levels [median 3 291(1 969, 8 776)U/L] and underwent muscle biopsy. Histopathological findings revealed myofiber degeneration, necrosis, and regeneration in all cases, with inflammatory infiltration in 9 cases, MHC-Ⅰ positivity in all, and C5b-9 positivity in 9 cases. The median follow-up duration was (15.7±6.3) months. At the last follow-up, muscle strength was normal or nearly normal, and the CK median value had decreased to 977.5 (211.0, 3 536.0) U/L.Conclusion:For patients with suspected idiopathic inflammatory myopathy and significantly elevated CK, muscle-specific antibody testing-including anti-HMGCR-and muscle biopsy should be performed promptly regardless of the presence of skin rash, to ensure accurate diagnosis and guide treatment, thereby avoiding misdiagnosis or missed diagnosis.

Objective: To explore the influence of pVR-IL15 gene adjuvant on the immune responses of different immunization strategies. Methods: The sequential immunization strategies were used in BALB/c mice with a DNA vaccine, recombinant modified vaccinia virus Ankara and recombinant adenovirus expressing HBsAg respectively and combined with a gene adjuvant pVR-IL15. Cellular and humoral immune responses were evaluated by IFN-γ enzyme-linked immunospot assay (ELISPOT) and enzyme-linked immunosorbent assay (ELISA), respectively. Results: The levels of humoral and cellular immune responses in the immune groups combined with pVR-IL15 were significantly higher than those of the non-combined with pVR-IL15. Conclusions The pVR-IL15 gene adjuvant can enhance the immune responses induced by the recombinant viruses expressing HBsAg.

Objective This study was aimed to characterize the swine leukocyte antigen( SLA) class I genes of GGTA1 -/ - Wuzhishan minipigs and compare their similarity to human leukocyte antigen( HLA) . It has important implica?tions for understanding the cellular rejection in xenotransplantation. Methods Specimens of ear tissue from six founding GGTA1 -/ - Wuzhishan minipigs were collected, and the SLA class I genes (SLA?1, SLA?3, SLA?2) were amplified by RT?PCR. Purified products were cloned into pEASY?T1 vectors and sequenced, followed by BLAST alignment and using bioin? formatc analysis to characterize the SLA class I genes and compare with the similarity to HLA. Results A total of six al?leles were detected, among them alleles were previously reported (SLA?1?0703,SLA?2?1102, SLA?3?0401, SLA?3?0403), and the other were novel (SLA?1?0401wz01, SLA?2?11wz01). The homology between alleles of SLA class I genes in Wuzhishan minipigs and HLA was from 70?5% to 72?1%. The homology analysis of critical amino acid residues on HLA binding with human CD8 + molecules showed that SLA?1?0401wz01, SLA?1?0703, SLA?2?11wz01, SLA?2?1102 and SLA?3?0401 occurred mutant at amino acid positions 225 and 228 ( T→S,T→M) , whereas the other loci were highly conserved. There was a high homology at amino acid level between SLA?2?11wz01, SLA?2?1102 and HLA class I genes which are NK cell KIRs binding sites. Conclusions The amino acid sequences of SLA class I genes of GGTA1 -/ -Wuzhishan minipigs have a high homology to HLA. From the point of view of cell?mediated xenograft rejection, the amino acid sequences of SLA class I genes of GGTA1 -/ - Wuzhishan minipigs have a high homology to HLA, therefore, Wzhishan minipigs may become a good potential donor for pig?human xenotransplantation.

Objective To construct the recombinant modified vaccinia Ankara vaccine expressing HBsAg and investigate its antigenicity and immune effect by different strategies.Methods Target gene was cloned into plasmid pSC1l,the recombinant plasmid pSC11-S was transfected into BHK-21 cells that infected with MVA.Homologous recombination occurred between MVA and pSC1 1-S.The recombinant virus MVA-S was selected by several rounds of blue/clear plaques.Target gene was identified by PCR.The expression of target gene was analyzed by Western Blot.The BALB/c mice were immunized with single immunization and consecutive immunization strategy.The level of specific cellular response was measured by ELISpot assays.Results The recombinant virus expressing target gene was confirmed by PCR and Western Blot showed that MVA-S could elicit specific cellular response.There were no significant differences among two kinds of DNA vaccine and recombinant MVA-S.The prime/boost regimen could induce higher cellular response than single DNA vaccine immunization.High doses of MVA-S boost with the same DNA vaccine prime could induce highest response.There was no significant difference between different DNA vaccines (pVR-S,pcDNA-S) followed by same MVA-S boost.Conclusions MVA-S expressing target was constructed successfully,and it could induce specific cellular immune response which was higher with the strategy of DNA prime/MVA-S boost than that of single immunization.

Objective The objective of this study was to analyze the immune effects of pVR-S and rAdV-S with different immune strategies.Methods HBsAg gene was used to construct recombinant DNA plasmid named pVR-S and recombinant adenovirus named rAdV-S,respectively.Firstly,BALB/c mice were injected with pVR-S by two methods,including electroporation and intramuscular injection.Secondly,two groups of mice were immunized with rAdV-S and pVR-S,respectively.Level of HBsAb was detected by ELISA and cellular responses were detected by Elispot to evaluate the immune effects.Finally,DNA primeAdenovirus boost strategy was used to evaluate the humoral and cellular immune responses.Results Compared with intramuscular immunization,electroporation-mediated immunization could induce stronger humoral immune response.Independent pVR-S increased HBsAb level slowly,while independent rAdV-S induced HBsAb at week 2 with higher HBsAb level than pVR-S.What is more,pVR-S prime-rAdV-S boost strategy could result in two to three times higher cellular responses than independent immunizations.Conclusion Recombinant adenovirus rAdV-S could induce strong humoral and cellular immune responses.pVR-S prime-rAdV-S boost immunization was better than independent rAdV-S immunization,contributing to excellent antiviral capability.

Objective To study the mechanisms of depression by exploring expressional differences of AQPs in the tissues of chronic stress depression rats.Methods Depression model was replicated by unpredicted chronic stress.20 rats were randomly separated into normal control group and model control group,AQP4 and AQP3,AQP8 expressions on hippocampus and gastrointestinal mucosa of rat model with depression were detected by immunochemical staining method.Results Means of optical density of AQP4 of normal group and model group hippocampus were 0.28 ± 0.02,0.22 ± 0.06 respectively,and the difference between two groups was significant statistically(t value was 2.756,P＜0.05).The expression of AQP3 on gastric mucosa and colonic mucosa between two groups had no significant statistically(t value were 1.814,1.812,P＞0.05).Two groups'means of optical density of AQP3 on small intestinal mucosa were 0.15 ± 0.02,0.17 ± 0.02,and the difference was significant statistically (t value was 2.769,P＜0.05).Two groups'means of AQP8 optical density in gastric mucosa were 0.15± 0.01,0.19 ± 0.04 ;0.16 ± 0.01 and 0.21 ± 0.04 in small intestinal mucosa;0.16 ± 0.01 and 0.22 ± 0.04 in colonic mucosa,and the differences were significant statistically(t values were 3.139,5.113,4.534,P＜0.05,P＜0.01,P＜0.01).Conclusion The expression of AQP4 on depression model rat hippocampus are lower than those of the normal group ; and the expression of AQP3 on gastric mucosa and colonic mucosa are no change obviously,but it(')s up-regulation on small intestinal mucosa.The expressions of AQP8 on gastric mucosa and small intestinal mucosa and colonic mucosa are up-regulating.

BACKGROUNDTo study the effect of DTB against HIV-1, for developing anti-HIV drugs.

METHODSDifferent concentration of DTB was added to cell culture system after viral inoculation, MTT staining method for viable cells (MTT assay) and p24 (ELISA) were used as markers to monitor the viral replication.

RESULTSThe inhibition rates of DTB at concentrations 160, 80, and 40mg/ml were 93.0%, 56.2% and 18.1%, respectively.

CONCLUSIONSDTB could effectively inhibit HIV-1 in vitro.

Anti-HIV Agents ; pharmacology ; Bilirubin ; analogs & derivatives ; pharmacology ; Dose-Response Relationship, Drug ; HIV-1 ; drug effects ; Humans ; In Vitro Techniques

Objective To assess therapeutic effects and safety of high intensity focused ultrasound ( HIFU ) and radiotherapy for the treatment of rectal carcinoma. Methods In combination with low dose of radiotherapy, we treated 121 cases of rectal carcinoma with FEP BY01 type HIFU therapy from Jul.1998 to Dec.2000 and 43 cases were followed up for more than one year. ResultsComplete response (CR) was achieved in 27 cases(22%), partial response (PR) in 80 cases (66%),and no change(NC)was found in 14 cases (12%) with a total response rate of 88 4%.There were no complications. One year survival rate was 81%(35/43) and 2 year survival rate was 77%(23/30). ConclusionHIFU in combination with radiotherapy is a new method to treat rectal carcinoma with significant therapeutic effect and high safety.