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.

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.

As patients with type 1 diabetes,both the function and number of their beta cells decreased.Despite treating with insulin in early stage,it can't avoid the occurrence of serious complications or death.However,Immunotherapy,different from the previous replacement therapy,emphasizes re-balance between affected T cells and immune regulative effect,suppresses autoimmune reaction,and strengthens the tolerance of beta cells of islet to it.At present,a variety of immune medicines have already been approved for the treatment of type 1 diabetes,such as anti-CD3 monoclonal antibody,GAD-alum,etc.

Objective To observe the incidence,clinical types,and prognosis of pulmonary involvement in children with systemic lupus erythematosus(SLE),and to probe the relationship between the relative factors.Methods The clinical data of 61 children with SLE,who treated in Children's Hospital of Shanghai from Jan.2006 to Dec.2011 were analyzed retrospectively.The lung manifestations of the patients were classified according to their clinical symptoms and signs,and lung imaging examination (chest X-ray and CT).Other data of the SLE patients with lung injury or without lung injury were collected,including the course of the disease,activity index [SLE disease activity index (SLEDAI) scoring 0-4 was divided into no activity,5-9 was divided into low activity,10-14 was divided into moderate activity,and more than 15 as severe activity],blood sedimentation,the values of serum complement and the positive rate of dsDNA in autoantibody,and a comparison was made and statistical analysis was performed.Results Pulmonary injury occurred in 17 cases with an incidence rate of 27.9％ (17/61 cases),and the average course of the disease was (8.76 ± 6.96) months.The clinical manifestations of 17 cases included fever,cough,dyspnea,hypoxemia,chest pain,lower breath sound,and lung rale,which were not non-specificity.According to imageological examination,in 17 cases there were 2 cases with pulmonary hypertension (the incidence rate was 11.8％),4 cases with acute lupus pneumonitis (the incidence rate was 23.5％),6 cases with interstitial pneumonia (the incidence rate was 35.3％),7 cases with lesion of pleura (the incidence rate was 41.2％),10 cases with pulmonary infection (the incidence rate was 58.8％).There were no statistical differences between patients with and without lung injury(infectious and non-infectious)in the course,the values of serumn complement,the positive rate of dsDNA and activity index.Lung lesions of the 17 patients recovered quickly after early diagnosis and active treatment.Conclusions Pulmonary manifestation in children with SLE is not uncommon,and it can happen at any stage of the disease,not just on the stage of lupus activity,but it usually indicates disease was not controlled.Pulmonary manifestation of SLE in children involve many parts of lung,including pulmonary parenchyma,interstitial,blood vessels,pleura,etc.Early diagnosis and active treatment can improve the short-term prognosis of lung injury with lupus,and long-term outcome remains to be observed.

Objective Tumor size discrepancy in measurement between real-time ultrasonic elastography and conventional uItrasound(US) was found in some breast lesions.The study was designed to investigate the value of the feature in the diagnosis of breast cancer,and its relationship with the margin features on gray-scale US.Methods Both US and real-time elastogram using a Hitachi EUB-8500 US system were performed in 308 consecutive Datients.The margin features on US and the size measured on both methods were documented.A lesion was defined as a significant size discrepancy when a larger size measured at elastogram.Sensitivity,specificity,and overall accuracy of feature to diagnosis malignancy were determined by surgical pathology as gold standard.Results There were total 166 benign,163 malignant and 3 borderline lesions.of 99 lesions with larger measurement on elastography,there were 91 malignant lesions and 8 benign lesions.By the feature of size discrepancY,the sensitivity,specificity and accuracy was achieved 55.8%,96.9%,75.7%,respectively.The size measured on elastogram was statistically larger than that on gray scale US(t=-11.0,P＜0.05).The ratio of indistinct,angular and spiculated margin characteristics were significantly higher in breast cancers with larger size measurement than those with unchanged or decreased size measurement(P＜0.05).Conclusions The increased size-measurement at elastography is helpful to diagnosis breast cancers.And it is more likely to be present in breast cancers with indistinct,angular or spiculated margin.

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.

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.

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.

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.