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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.
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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.
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Objective To investigate the expression and distribution of plasmacytoid dendritic cells(pDC) in peripheral blood and renal tissues in children with Henoch-SchSnlein purpura(HSP),and explore the role of pDCs in the pathogenesis of Henoch-Schtnlein purpura nephritis(HSPN).Methods Among the 40 children with HSP,28 cases were in the active phase(renal biopsy performed in 8 cases of them) and the other 12 in remission phase.Peripheral blood mononuclear cells were isolated,and the expression of pDC was detected by flow cytometry.The normal control group was established (n =15).Total RNA of peripheral blood was extracted and transcripted into cDNA.Sybr green dye based real-time quantitative PCR method was used to compare the expression(indicated as 2-△Ct value) of CXC motif chemokine 10 (CXCL10),CC chemokine ligand 5 (CCL5),chemokine CXC subfamily receptor 3 (CXCR3),CC chemokine receptor 5 (CCR5) in children with HSP and those in the controls.Immunohistochemistry labeling technique was used to detect the distribution of pDC in renal tissues from renal biopsy,and the normal controls were established (n =3).Results The expression percentage of pDC in peripheral blood in active phase was 0.051 ± 0.039,significantly lower than those in remission phase (0.181 ± 0.082) and the normal controls (0.166 ± 0.079) (P ＜ 0.000 1).Chemokines genes CXCL10 and CCL5 were overexpressed in peripheral blood ceils of acute phase HSP children,but chemokine receptors CXCR3,CCR5 were lowly expressed compared with normal controls.There was almost no expression of pDC in the normal control renal tissues,while pDC was infiltrated in glomeruli of HSPN children.Conclusions The number of pDC and chemokines' expression in peripheral blood is abnormal,and the pathogenesis of nephritis may be involved with the pDC in peripheral blood to migrate to the renal tissues.
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This paper summarized the features of pediatrician training in Cincinnati Children's Hospital Medical Center ( CCHMC ) , introduced their cultivation and appraisal system , compared American pediatrician training with Chinese training and absorbed advanced medical training experi-ences. CCHMC focus on training specialized skill, team spirit and communication ability of residents. Internal assessment and two-way selection help to retain the best fellows. Learning from advanced ex-periences, we hoped to improve the current pediatrician training system and contributed to medical and health services.
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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.
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Hemophagocytic lymphohistiocytosis (HLH) is characterized by hemophagocytosis and a reactive process resulting from prolonged and excessive activation of antigen presenting cells (macrophages,histiocytes) and CD8+ T cells.The majority of genetic causes identified to date affect the cytotoxic function of NK and T cells,crippling immunologic mechanisms that mediate natural immune contraction.The predominant clinical findings of HLH are fevers (often hectic and persistent),cytopenias,hepatitis and splenomegaly.Due to the life-threatening implications of the diagnosis of genetically determined HLH,antiinflammatory therapy,often consisting of steroids,etoposide or antithymocyte globulin,should be instituted promptly,followed by curative hematopoietic cell transplantation.Secondary HLH,associated with autoimmune disorders or viral infections in teens and adults,should control the primary inflammatory.
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Objective To study 6 type Ⅰ interferon (IFN)-inducible genes (IFIT4, IFI44, Ly6e,OAS1, OAS2 and OAS3) in patients with lupus nephritis (LN) and analyze its correlated expression levels with disease activity and/or clinical manifestations. Methods Total RNA was obtained simultaneously from kidney tissues and peripheral blood cells of 12 patients with diffuse proliferative lupus nephritis and 10 normal controls. Moreover, peripheral blood cells were obtained from 119 LN patients and 35 normal controls. Total RNA was extracted and reversely transcribed into complementary DNA. Gene expression levels were measured by real-time polymerase chain reaction by comparing to a housekeeping gene, and IFN score was calculated. Disease activity was determined by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Results The 6 genes were highly expressed in diffuse proliferative lupus nephritis patients compared with normal controls. IFN scores were positively correlated with SLEDAI score, the concurrent presences of anti-double-stranded DNA (anti-dsDNA) antibodies (P<0.05) and hypocomplementemia (P<0.01). Conclusion The 6 IFN-inducible genes are highly expressed iri LN patients. IFN scores are elevated in active lupus nephritis patients, in patients with positive anti-ds-DNA antibody and hypocomplementemia. IFN scores may be a useful biomarker for lupus nephritis therapy.
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Objective To explore the role of chemokines and ehemokine receptors in the etiopathog-enesis of diffuse proliferative lupus nephritis (LN). Methods ① Total RNA from the kidney tissues and peripheral blood cells of 12 patients with diffuse proliferative LN and 10 normal controls were prepared simultaneously and reverse transcribed into complementary DNA. Sybr green dye based real-time quantitative PCR method was used to compare the expression levels (indicated as-AACt value) of MCP-1, CCL19,CXCLg, CXCL10 and CCR2, CCR7, CXCR3. ② Immunofluoresceee labeling and immunohistochemical staining technique were used to observe the distribution of chemokines MCP-1, CCL19, CXCL9 and CXCL10 in normal and patients kidney tissues. Results The 4 chemokines genes (MCP-1, CCL19, CXCL9 and CXCL10) were consistently highly expressed in kidney tissues and peripheral blood ceils of diffuse proliferative LN patients compared with normal controls. The 2 chemokine receptors, CCR2 and CXCR3 were also overexpressed in peripheral blood cells of diffuse proliferative LN patients. There was nearly no expression of these 4 chemokine proteins in normal kidneys. But they were found in glomeruli of diffuse proliferative LN patients. Conclusion The expression of chemokines in the peripheral blood cells may be used as biomarkers for LN. Further study maybe lead to the development of specific drugs targeting at them for the treatment of systemic lupus erythematosus (SLE).