Objective To investigate the protective effects and potential mechanisms of tetrahydrocurcumin(THC)on thoracic aortic aneurysm and dissection(TAAD)in mice.Methods TAAD was induced in 3-week-old C57BL/6J mice by oral administration of β-aminopropionitrile(BAPN)(diluted in drinking water,1 g/(kg·d)).Eighty mice were divided randomly into Con,BAPN,BAPN+THC,and BAPN+THC+3-TYP(SIRT3 inhibitor)groups(n=20 mice per group).The survival rate of mice in each group was recorded after 4 weeks.The maximum diameter of the aorta was measured and the histomorphology and aortic wall elastin integrity were evaluated by hematoxylin and eosin and elastin van Gieson staining.Macrophage infiltration was detected by immunohistochemical staining and α-smooth muscle actin(α-SMA)and osteopontin(OPN)expression were detected by immunofluorescence staining.The production of reactive oxygen species(ROS)was measured by dihydroethidium staining and superoxide dismutase(SOD)activity and malondialdehyde(MDA)levels were determined using kits.Protein expression levels of matrix metalloproteinase(MMP)2,MMP9,interleukin(IL)-6,tumor necrosis factor(TNF)-α,nuclear factor erythroid 2-related factor 2(NRF2),NADPH oxidase 2(NOX2),α-SMA,OPN,sirtuin 3(SIRT3),Ac-SOD2,and SOD2 were measured by Western Blot.Results Mice in the BAPN+THC group showed significantly higher survival and a lower incidence of TAAD compared with the BAPN group and the degree of aortic dilatation and morphology and structure were improved(P＜0.05).Infiltration of CD68-positive macrophages and MMP2,MMP9,IL-6,and TNF-α expression levels were lower(P＜0.05),ROS generation,MDA content,and NOX2 expression in aortic tissue were also significantly decreased,while SOD activity and NRF2 expression were increased(P＜0.05).α-SMA expression was also increased,while OPN expression was reduced(P＜0.05).SIRT3 expression was increased while the Ac-SOD2/SOD2 ratio was decreased(P＜0.01).Treatment with the SIRT3-specific inhibitor and silencing of SIRT3 counteracted the ability of THC to resist TAAD via the SIRT3 signaling pathway(all P＜0.05).Conclusions THC alleviated inflammation and oxidative stress in aortic tissues by activating the SIRT3 signaling pathway,thus inhibiting the phenotypic transformation of vascular smooth muscle cells and resisting the formation of TAAD in mice.

Objective To investigate the protective effects and potential mechanisms of tetrahydrocurcumin(THC)on thoracic aortic aneurysm and dissection(TAAD)in mice.Methods TAAD was induced in 3-week-old C57BL/6J mice by oral administration of β-aminopropionitrile(BAPN)(diluted in drinking water,1 g/(kg·d)).Eighty mice were divided randomly into Con,BAPN,BAPN+THC,and BAPN+THC+3-TYP(SIRT3 inhibitor)groups(n=20 mice per group).The survival rate of mice in each group was recorded after 4 weeks.The maximum diameter of the aorta was measured and the histomorphology and aortic wall elastin integrity were evaluated by hematoxylin and eosin and elastin van Gieson staining.Macrophage infiltration was detected by immunohistochemical staining and α-smooth muscle actin(α-SMA)and osteopontin(OPN)expression were detected by immunofluorescence staining.The production of reactive oxygen species(ROS)was measured by dihydroethidium staining and superoxide dismutase(SOD)activity and malondialdehyde(MDA)levels were determined using kits.Protein expression levels of matrix metalloproteinase(MMP)2,MMP9,interleukin(IL)-6,tumor necrosis factor(TNF)-α,nuclear factor erythroid 2-related factor 2(NRF2),NADPH oxidase 2(NOX2),α-SMA,OPN,sirtuin 3(SIRT3),Ac-SOD2,and SOD2 were measured by Western Blot.Results Mice in the BAPN+THC group showed significantly higher survival and a lower incidence of TAAD compared with the BAPN group and the degree of aortic dilatation and morphology and structure were improved(P＜0.05).Infiltration of CD68-positive macrophages and MMP2,MMP9,IL-6,and TNF-α expression levels were lower(P＜0.05),ROS generation,MDA content,and NOX2 expression in aortic tissue were also significantly decreased,while SOD activity and NRF2 expression were increased(P＜0.05).α-SMA expression was also increased,while OPN expression was reduced(P＜0.05).SIRT3 expression was increased while the Ac-SOD2/SOD2 ratio was decreased(P＜0.01).Treatment with the SIRT3-specific inhibitor and silencing of SIRT3 counteracted the ability of THC to resist TAAD via the SIRT3 signaling pathway(all P＜0.05).Conclusions THC alleviated inflammation and oxidative stress in aortic tissues by activating the SIRT3 signaling pathway,thus inhibiting the phenotypic transformation of vascular smooth muscle cells and resisting the formation of TAAD in mice.

Beh?et′s syndrome is a kind of chronic systemic vasculitis with involvement of multiple organs. Intestinal involvement of Beh?et′s syndrome is presently named as intestinal Beh?et′s syndrome. Recently, there is considering another kind of disease type with only typical intestinal ulcers. Since it is difficult to differentiate intestinal Beh?et′s syndrome from Crohn′s disease, intestinal tuberculosis, intestinal lymphoma, and intestinal manifestations of many other autoimmune diseases, and there is limited evidence for the therapy of intestinal Beh?et′s syndrome, proposing diagnosis and treatment recommendations for intestinal Beh?et′s syndrome through evidence-based judgment will be of great significance for clinical practice.

Behçet’s syndrome is a kind of chronic systemic vasculitis with involvement of multiple organs. Intestinal involvement of Behçet’s syndrome is presently named as intestinal Behçet’s syndrome (disease). Recently, there is considering another kind of disease type with only typical intestinal ulcers. Since it is difficult to differentiate intestinal Behçet’s syndrome from Crohn’s disease, intestinal tuberculosis, intestinal lymphoma, as well as intestinal manifestations of many other autoimmune diseases, and there is limited evidence for the therapy of intestinal Behçet’s syndrome, proposing diagnosis and treatment recommendations for intestinal Behçet’s syndrome through evidence-based judgment will be of great significance for clinical practice.

Pathology plays an important role in diagnosis and differential diagnosis of inflammatory bowel disease (IBD) . However, histopathological features of IBD vary among different patients and at different stages in the same patient. There is a lack of definitive morphological diagnostic criteria for IBD. Meanwhile, diagnostic accuracy is affected by training level and diagnostic experience of pathologists. In order to improve the pathological diagnosis of IBD, and to provide advice on practical diagnostic issues of IBD in China, experts guideline of pathological assessment was discussed by an expert group of pathologists and gastroenterologists.

Pathology plays an important role in diagnosis and differential diagnosis of inflammatory bowel disease (IBD) . However, histopathological features of IBD vary among different patients and at different stages in the same patient. There is a lack of definitive morphological diagnostic criteria for IBD. Meanwhile, diagnostic accuracy is affected by training level and diagnostic experience of pathologists. In order to improve the pathological diagnosis of IBD, and to provide advice on practical diagnostic issues of IBD in China, experts guideline of pathological assessment was discussed by an expert group of pathologists and gastroenterologists.

Objective:To investigate the clinical significance of serum resistin in juvenile idiopathic arthritis(JIA) patients.Methods:A prospective observational study was performed and 32 cases of patients with systemic onset JIA(SOJIA)(SOJIA group) in children admitted to the nephrorheumatology and outpatient were enrolled at Children′s Hospital of Shanghai between October 2013 and September 2015, 52 cases of other types(N-SOJIA group), and 33 cases of other rheumatic diseases(other rheumatic diseases group), 30 cases of children undergoing health checkups in the child health outpatient clinic(healthy control group)were involved as well.Serum resistin levels were measured by enzyme-linked immunosorbent assay(ELISA), and comprehensive analysis was carried out with clinical data and related laboratory findings.The basic data of gender, age and body mass index(BMI) of each group were collected, and the duration of disease in children in JIA group, rheumatoid factor, antinuclear antibody, white blood cell, hemoglobin, platelet, C reacting protein(CRP), erythrocyte sedimentation rate(ESR), clinical manifestations and current drug use were collected.Using the receiver operating characteristic(ROC)curve analysis of sensitivity and specificity resistin levels in diagnostic systemic juvenile idiopathic arthritis.Results:There was no statistically significant difference in the age, gender and BMI of children in SOJIA group, N-SOJIA group, other rheumatism group and healthy control group.Children in the SOJIA group and the N-SOJIA group had arthritis in clinical manifestations.Fever and rash were more common in the SOJIA group, and the difference was statistically significant ( P<0.01). Laboratory results showed that the sedimentation rates of white blood cells, CRP, and red blood cells were in the SOJIA group was significantly elevated.The antinuclear antibody was mainly found in the N-SOJIA group with a higher positive rate ( P<0.05). The mean serum resistin in the SOJIA group [(17.98±13.78) mg/L] was higher compared to the healthy control group [(1.84±1.66) mg/L], other rheumatic diseases group [(8.00±6.28) mg/L]and the N-SOJIA group [(9.86±6.11) mg/L], the differences were statistically significant ( F=21.625, P<0.01). Resistin was positively correlated with white blood cells and CRP( r=0.532, 0.351, all P<0.05), and had no correlation with BMI, hemoglobin, platelets, and ESR( r=0.059, -0.176, 0.152, 0.203, all P>0.05). Based on serum resistin≥5.55 mg/L as the positive threshold value, the area under ROC curve was 0.802, and the sensitivity and specificity in diagnosis of SOJIA was 96.9% and 49.6%, respectively. Conclusions:Serum resistin is increased in patients with JIA, especially in SOJIA increased significantly; Serum resistin can be used for the diagnosis of SOJIA, and ≥5.55 mg/L can be a suitable cut-off level.

Objective To explore the relationship between the expression of transient receptor potential cation channel subfamily C member 6 (TRPC6) and podocyte injury in children with primary nephrotic syndrome (PNS) and its clinical significance. Methods The renal tissue of 18 children with PNS was obtained. The pathological changes of kidney were observed by routine section staining and light microscopy. The structural changes of podocyte were observed by electron microscope. The mRNA and protein expressions of TRPC6 in tissues were determined by qPCR and immunohistochemistry, respectively. Further the correlation of TRPC6 mRNA with serum levels of albumin (Alb), creatinine (Cr), triacylglycerol (TG), cholesterol (Tch), complement C3 and 24 h urinary protein quantitation and estimated glomerular filtration rate (eGFR) were analyzed respectively. Results The expression of TRPC6 protein in renal tissue of children with PNS was higher than that in the control group, and the difference was statistically different (P<0.05). The relative expression of TRPC6 mRNA in renal tissue of children with PNS was positively related to the expression of TRPC6 protein (r=0.508, P<0.05), but there was no correlation of expression of TRPC6 mRNA with serum levels of Alb, Cr, TG, Tch, C3, eGFR and 24h urinary protein quantitation (P>0.05). Conclusion The pathological types of PNS were mainly podocyte lesions, and the expression of TRPC6 protein was increased in podocytes. TRPC6 detection may be helpful in the diagnosis of podocyte lesions.

Systemic lupus erythematosus(SLE)is a common and complicated autoimmune disease. The Sys-temic Lupus International Collaborating Clinics Group undertook a revision of the American College of Rheumatology (ACR)classification criteria for SLE in 2009. The new revision included clinical criteria and immunogic criteria,and had greater sensitivity but lower specificity than ACR - 1997. Kidney Disease:Improving Global Outcomes(KDIGO) clinical practice guideline and ACR guideline elaborated the treatment for lupus nephritis. Children with lupus nephritis should receive the same therapies as adults with dosing based on patient size and glomerular filtration rate. Biological agents could be used to treat refractory SLE or those who were intolerant to traditional immunosuppressant.

Inlfammatory bowel disease (IBD) is characterized by chronic, relapsing inlfammation of the gastrointestinal (GI) tract. In recent years, the incidence is increasing in the pediatric population. Insufifcient recognition of extraintestinal manifestations (EIMs) of IBD clinically may delay diagnosis. Therefore, improving the recognition of EIMs of IBD has an important signiifcance in its early diagnosis and treatment. The pediatricians should be alerted that renal lesions of EIMs in IBD are mainly immunoglobulin A (IgA) nephropathy and interstitial nephritis.