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Objective To study the demographic and clinical characteristics, correlation of genotype and phenotype and treatment of Blau syndrome to facilitate early diagnosis and timely treatment of Blau syndrome. Methods Seventy-two patients with Blau syndrome from 11 centers from May 2006 to April 2022 were retrospectively analyzed, and their general information, clinical data, laboratory examination and treatment medication were collected. Results The distribution of patients with Blau syndrome was uniform in geographical north and south of China, and there was no obvious gender bias. The mean age of onset was (14.30±12.81) months, and the age of diagnosis was (55.18±36.22) months. 35% of patients with Blau syndrome happened before 1 year old, and all patients developed before 5 years old. 87.50% (63/72) had granulomatous arthritis, 65.28% (47/72) had rash, 36.11% (26/72) had ocular involvement, 27.78% (20/72) had fever, and 15.28% (11/72) had pulmonary involvement. Arthritic manifestations of Blau syndrome were most at risk, followed by rash, ocular involvement, and fever.The first 25 months of the disease, the risk of developing a rash was the greatest. The risk of developing arthritis was the greatest between 25 months and 84 months. The main mutations were p.R334Q and p.R334W, and patients with p.R334Q mutation had relatively high incidence of fever (35.71%[5/14] vs. 14.29%[1/7], P=0.43) and ocular involvement (42.86%[6/14]vs. 28.57%[2/7], P=0.51). There was a relatively high incidence of rash (85.71%[6/7] vs. 64.29%[9/14], P=0.59) in patients with the p.R334W mutation. Forty-five patients(62.50%)were treated with a combina-tion of glucocorticoid and methotrexate. Twenty-two patients were treated with tumor necrosis factor antagonist in addition to glucocorticoid and methotrexate. Conclusions The risk of different clinical manifestations of Blau syndrome from high to low was arthritis, followed by rash, ocular involvement and fever. The main treatment was glucocorticoid combined with methotrexate, to which biological agents could be added.
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Objective To evaluate the clinical significance of serum thrombospondin-1 (TSP-1) in systemic lupus erythematosus (SLE), and explore its possible involvement in SLE pathogenesis. Methods One hundred and thirty-eight patients diagnosed with SLE, including 124 cases of females and 14 males, as well as 60 healthy controls were recruited into this study. Enzyme-linked immunosorbent assay (ELISA) was used to determine serum TSP-1 expression level between the two groups. Spearman correlation analysis method was used to analyze the correlation between TSP-1 level of complement 3, complement 4, anti-double-stranded DNA, Rheumatoid factor and systemic lupus erythematosus disease activity index (SLEDAI). Results TSP-1 level in healthy control group was much higher than that in SLE patients. TSP-1 serum levels in SLE patients was positively correlated with complement 3 (r=0.386, P<0.01), complement 4 (r=0.301, P<0.01), and negatively correlated with anti-double-stranded DNA (r=-0.221, P=0.009), RF (r=-0.186, P=0.029) and SLEDAI (r=-0.273, P=0.001). Conclusion TSP-1 may play an immune-regulatory role in the development of SLE by inhibiting inflammation and autoantibody production. It could become a potential therapeutic target for the treatment of SLE.
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Objective To evaluate the clinical significance of serum thrombospondin-1 (TSP-1) in systemic lupus erythematosus (SLE), and explore its possible involvement in SLE pathogenesis. Methods One hundred and thirty-eight patients diagnosed with SLE, including 124 cases of females and 14 males, as well as 60 healthy controls were recruited into this study. Enzyme-linked immunosorbent assay (ELISA) was used to determine serum TSP-1 expression level between the two groups. Spearman correlation analysis method was used to analyze the correlation between TSP-1 level of complement 3, complement 4, anti-double-stranded DNA, Rheumatoid factor and systemic lupus erythematosus disease activity index (SLEDAI). Results TSP-1 level in healthy control group was much higher than that in SLE patients. TSP-1 serum levels in SLE patients was positively correlated with complement 3 (r=0.386, P<0.01), complement 4 (r=0.301, P<0.01), and negatively correlated with anti-double-stranded DNA (r=-0.221, P=0.009), RF (r=-0.186, P=0.029) and SLEDAI (r=-0.273, P=0.001). Conclusion TSP-1 may play an immune-regulatory role in the development of SLE by inhibiting inflammation and autoantibody production. It could become a potential therapeutic target for the treatment of SLE.
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Objective To evaluate the clinical value of Chinese Multi-Dimensional Health Assessment Questionnaire (MDHAQ-C) in patients with rheumatoid arthritis (RA). Methods From December 2015 to October 2016,four hundred and twelve RA patients were recruited in this study and completed the MDHAQ-C independently and routine assessment of patient index data 3 (RAPID3) was calculated. Then correlations were assessed by Spearman correlation coefficient of RAPID3 with indices of clinical relevance and disease activity to evaluate its clinical values. Results MDHAQ-C is moderately correlated with ESR (r=0.399), CRP (r=0.381), SJC (r=0.499), TJC (r=0.558) (P<0.01), but highly correlation with VAS of pain (r=0.836), VAS of global status (r=0.915) and overall assessment of physician (r=0.784)(P<0.01), and highly correlated with a Spearman's coefficient of 0.784 for DAS 28-ESR, 0.734 for CDAI and 0.682 for SDAI (P<0.001). Conclusion MDHAQ is a reliable, valid instrument for disease activity assessment and a feasible index for clinical settings in Chinese RA patients.
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To observe the effects of Feiyanning (FYN) formula, a compound traditional Chinese herbal medicine, on the expressions of CXCL12 and CXC chemokine receptor 4 (CXCR4) mRNAs and proteins in Lewis tumors in C57 mice.
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Background and purpose:CA-125 is already recognized as one of the biomarkers for ovarian cancer.The serum CA-125 also had some significance in terms of the diagnosis and determination of the prognostic values for lung cancer.The purpose of this study was to investigate the effect of traditional Chinese medicine(TCM)therapy on serum CA-125 level in lung cancer patients.At the same time,we investigated the relationship between the serum CA-125 level and the clinical stages as well as the pathological types.Methods:Serum CA-125 levels from 30 normal donors were detected and compared to 60 patients with lung cancer by microparticle enzyme immunosorbant assay(MEIA).Results:Serum CA-125 levels in lung cancer group were(91?45)and significantly higher than those in healthy control group(18?5)(P
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Objective To study the expressions of epithelial symbol factors including E-cadherin,?-catenin,and ?-catenin related to epithelial-mesenchymal transition(EMT) in C57 mice Lewis lung cancer and its mRNA expression affected by Feiyanning Decoction(Decoction for lung cancer).Methods The Real-Time PCR method was adopted to observe the E-cadherin,?-catenin,?-catenin mRNA expression of C57 mice with transplanted tumor in the right armpit and distal metastases and the affection of Feiyanning Decoction on the expression.Results The lung transplanted rate in the Feiyanning group was clearly lower than that in the model group(P