Objective:To explore the clinical features and risk factors of systemic lupus erythematosus(SLE) concomitant with interstitial lung disease(ILD) in children.Methods:A retrospective analysis was performed.A total of 111 hospitalized children diagnosed with SLE in the Department of Rheumatology and Immunology, Children′s Hospital of Soochow University from February 2016 to November 2018 were selected as the research subjects and divided into the SLE-ILD group(18 cases) and the SLE-non-ILD group(93 cases)according to the lung high-resolution CT manifestations. T-test and Wilcoxon rank sum test were used to compare and analyze the general situation, clinical manifestations and laboratory results.Multivariate Logistic regression was used to analyze the risk factors of SLE-ILD. Results:The prevalence of SLE-ILD was 16.2%(18/111 cases). There were significant differences between the SLE-ILD group and the SLE-non-ILD group in the course of disease [14.00 (12.00-24.25) months vs.1.00(1.00-2.00) months], the incidence of serositis [55.6%(10/18 cases) vs.8.6%(8/93 cases)], post-activity shortness of breath [83.3%(15/18 cases) vs.25.8%(24/93 cases)], nervous system damage [27.8%(5/18 cases) vs.6.5%(6/93 cases)], cardiovascular system damage [38.9%(7/18 cases) vs.9.7%(9/93 cases)], the occu-rrence of increased erythrocyte sedimentation rate [66.7%(12/18 cases) vs.31.2%(29/93 cases)], the decreased C 3[88.9%(16/18 cases) vs.62.4%(58/93 cases)], positive anti neutrophil cytoplasmic antibody (ANCA) [88.9%(16/18 cases) vs.18.3%(17/93 cases)], positive anti-Sm antibody [61.1%(11/18 cases) vs.15.1%(14/93 cases)] and anti ribonucleoprotein antibody (anti RNP antibody)[66.7%(12/18 cases) vs.16.1%(15/93 cases)](all P<0.05). Logistic regression analysis demonstrated that serositis( OR=30.535, 95% CI: 2.167-430.336, P=0.011), shortness of breath after exercise( OR=55.115, 95% CI: 1.117-2 579.852, P=0.041), positive ANCA( OR=65.090, 95% CI: 4.488-944.071, P=0.002) and positive anti-RNP antibody( OR=10.007, 95% CI: 1.362-73.500, P=0.024) were risk factors for SLE-ILD. Conclusions:The longer the course of SLE, the higher the incidence of ILD; serositis, shortness of breath after exercise, positive ANCA and positive anti RNP antibody may be risk factors for SLE-ILD.

Juvenile idiopathic arthritis (JIA) is defined as arthritis of unknown etiology during childhood.The pathogenesis of this disease is still unclear and the main principle of treatment is to eliminate symptoms, control infla-mmation, and protect joint function.Clinical prognosis can be improved in most JIA patients, but still some patients will eventually become disabled.With the advent of new biologic treatment agents, the treatment management model of JIA is also facing great challenges.Currently, there is still no unified standard to measure and monitor the clinical situation in all patients.This review aims to describe the disease assessment indices important to routine clinical care and integral to the design of outcome studies and clinical trials in JIA.

Objective To explore the dynamic changes and significance of costimulatory molecules CD40 and lymphocyte subsets in peripheral blood of children with eosinophilic gastroenteritis (EG). Methods The CD40 expression and lymphocyte subsets in peripheral blood were detected by flow cytometry (FCM) in 15 children with EG (acute stage and remission stage) and 15 healthy controls. The level of serum interleukin (IL) -4 was detected by enzyme-linked immunosorbent assay (ELISA). The eosinophil (EOS) was count by blood cell analyzer. Results In acute stage, the children with EG had significantly higher expression of CD40, CD3+, CD4+, CD4+/CD8+, and CD19+CD23+ in peripheral blood, higher serum IL-4 level, higher EOS count and lower CD8+ than in remission stage and control group (P all<0.05). There were no differences between remission stage and control group (P>0.05). In acute stage, the expression of CD40 in peripheral blood in children with EG was positively correlated with the expression of CD4+ and IL-4 (P all<0.05). Conclusions CD40 may be involved in the pathogenesis of EG. That the increase of IL-4 secreted by CD4+ T cells that were induced by CD40 results in abnormal increase of EOS may be one of mechanisms of the pathogenesis of EG.

Objective To analyze the expressions of lymphocytes in the peripheral blood and serum cytokines in the children with secretory otitis media (SOM) and the effects of glucocorticoids interventions.Methods Totally 90 SOM children were selected as case group,and 30 healthy children were selected as control group.The case group was randomly divided into group A (simple oral antibiotic treatment),group B (oral antibiotics combined with local glucocorticoid treatment) and group C (oral antibiotics combined with systemic glucocorticoid treatment),30 cases in each group.The CD4 + T lymphocytes percentage,CD8 + T lymphocytes percentage,the ratio of CD4 +/CD8 + T lymphocytes in peripheral blood and the serum interleukin-2 (IL-2),interferon gamma (IFN-γ),tumor necrosis factor alpha (TNF-α),interleukin-6 (IL-6),interleukin-10 (IL-10) levels were detected and compared between case group and control group.The air conduction auditory thresholds under different frequency of the patients in group A,group B and group C were examined and compared.Results The CD4 + T lymphocytes percentage,the ratio of CD4 +/CD8 + T lymphocytes in peripheral blood and the serum IL-2,IFN-γ,TNF-α,IL-6,IL-10 levels of the patients in the case group were significantly higher than those in the control group (P ＜ 0.0 5).After the treatment,the above indicators of the children in group A,group B and group C decreased,and there were significant differences before and after the treatment between two groups (P ＜ 0.05).The air conduction auditory thresholds under different frequency of the patients in group B or group C were significantly lower than those in group A (P ＜ 0.05),while there were no significant differences in the air conduction auditory thresholds under different frequency between the children in group B and group C (P ＞ 0.05).Conclusion The patients with SOM show imbalanced cell immune function and cytokines expressions in the peripheral blood.The combination of glucocorticoids therapy and routine antibiotic therapy can effectively improve the immune function and the therapeutic effects.

Objective To analyze the expressions of lymphocytes in the peripheral blood and serum cytokines in the children with secretory otitis media (SOM) and the effects of glucocorticoids interventions.Methods Totally 90 SOM children were selected as case group,and 30 healthy children were selected as control group.The case group was randomly divided into group A (simple oral antibiotic treatment),group B (oral antibiotics combined with local glucocorticoid treatment) and group C (oral antibiotics combined with systemic glucocorticoid treatment),30 cases in each group.The CD4 + T lymphocytes percentage,CD8 + T lymphocytes percentage,the ratio of CD4 +/CD8 + T lymphocytes in peripheral blood and the serum interleukin-2 (IL-2),interferon gamma (IFN-γ),tumor necrosis factor alpha (TNF-α),interleukin-6 (IL-6),interleukin-10 (IL-10) levels were detected and compared between case group and control group.The air conduction auditory thresholds under different frequency of the patients in group A,group B and group C were examined and compared.Results The CD4 + T lymphocytes percentage,the ratio of CD4 +/CD8 + T lymphocytes in peripheral blood and the serum IL-2,IFN-γ,TNF-α,IL-6,IL-10 levels of the patients in the case group were significantly higher than those in the control group (P ＜ 0.0 5).After the treatment,the above indicators of the children in group A,group B and group C decreased,and there were significant differences before and after the treatment between two groups (P ＜ 0.05).The air conduction auditory thresholds under different frequency of the patients in group B or group C were significantly lower than those in group A (P ＜ 0.05),while there were no significant differences in the air conduction auditory thresholds under different frequency between the children in group B and group C (P ＞ 0.05).Conclusion The patients with SOM show imbalanced cell immune function and cytokines expressions in the peripheral blood.The combination of glucocorticoids therapy and routine antibiotic therapy can effectively improve the immune function and the therapeutic effects.

Objective To observe the effect of bone morphogenetic protein 4(BMP4) on the infil-tration of macrophages and the expression of nuclear factor-κB in mice model of unilateral ureteral obstruc-tion(UUO).Methods C57BL/6 mice were randomly divided into four groups:injected intra-abdominally with saline-sham-operated group(saline-sham group,n =8),injection intra-abdominally with saline-UUO-operated group(saline-UUO group,n=8),injection intra-abdominally with anti-BMP4-sham-operated group (anti-BMP4-sham group,n=8),and injection intra-abdominally with anti-BMP4-UUO-operated group(anti-BMP4-UUO group,n=8).Either saline or anti-BMP4(group 200 μl/gram of body weight per day) were injected intra-abdominally for 7 days after surgery.Mice were sacrificed at 7th day to evaluate the expression of CD68 and p-P65 by immunohistochemical staining in each group.Besides,the p-P65 protein level was also analyzed by Western blotting in four groups.Results Immunohistochemical staining showed that the expres-sions of CD68 and p-P65 were significantly reduced in the kidney cortices in anti-BMP4-UUO group than in saline-UUO group(P<0.05,respectively).Similar to that,the p-P65 protein level was significantly reduced in the kidney cortices in anti-BMP4-UUO group than in saline-UUO group(P < 0.05,respectively). Conclusion BMP4 participates in the process of renal interstitial inflammation in obstructive nephropathy, and may play a role through the activation of nuclear factor-κB signaling pathway.

Objective To evaluate the efficacy and safety of tacrolimus (TAC) for treatment of Henoch-SchSnlein purpura nephritis in children.Methods Forty children with Henoch-SchSnlein purpura nephritis (pathological grade Ⅲ-Ⅵ) in Children's Hospital Affiliated to Soochow University from January 2013 to June 2016 were selected and divided into TAC group (n =19) and cyclophosphamide (CTX) group (n =21).The children in TAC group were given TAC orally;the children in CTX group were given CTX pulse therapy intravenously.The 24 h urine protein,urine red blood cell count,serum albumin,serum creatinine and blood urea nitrogen of children before treatment and after 6 months treatment were observed and compared between the two groups.The treatment effects and adverse reactions of patients were observed and compared between the two groups.Results There was no statistic difference in the 24 h urine protein,urine red blood cells count,serum albumin,blood creatinine and blood urea nitrogen levels of children between the two groups before treatment (P ＞ 0.05).The 24 h urinary protein and urine red blood cells count of children after 6 months of treatment in the two groups were significantly lower than those before treatment (P ＜ 0.05);there was no statistic difference in the serum albumin,serum creatinine and blood urea nitrogen levels of children in the two groups before treatment and after 6 months of treatment (P ＞ 0.05).Mter 6 months of treatment,the 24 h urine protein and urine red blood cells count of children in TAC group were significantly lower than those in the CTX group (P ＜ 0.05);there was no statistic difference in the serum albumin,serum creatinine and blood urea nitrogen levels of children between the two groups (P ＞ 0.05).After 6 months treatment,the effective rate of children in the TAC group was significantly higher than that in the CTX group (x2 =4.607,P ＜ 0.05).The incidence of adverse reactions of children in the TAC group was significantly lower than that in the CTX group (x2 =4.043,P ＜ 0.05).Conclusion TAC is effective in treatment of Henoch-Sch(o)nlein purpura nephritis in children.It is easy to take,and has less adverse reactions.

Objective To compare the clinical efficacy of different surgical treatment on cholesteatoma otitis media and analy‐sis its differences .Methods Totally 100 cases of patients with cholesteatoma otitis media in hospital were divided into two groups , 50 cacse in each group ,the control group used open mastoidectomy for treatment ,the experimental group used complete bi‐style mastoidectomy ,and the postoperative clinical efficacy were observed .Results The experimental group patients after radical mas‐toidectomy complete bi‐style treatment ,air conduction hearing threshold (26 .89 ± 7 .23)dBHL and gas bone conduction (12 .54 ± 3 .78)dBHL were significantly lower than after open radical mastoidectomy treatment of patients in the control group (41 .38 ± 10 .56)dBHL and gas bone conduction (22 .57 ± 5 .96)dBHL(P<0 .05);after different surgical treatment ,the cholesteatoma otitis media total efficiency (92 .00% ) of experimental group was significantly higher than control group (64 .00% ) ( P< 0 .05 ) . Conclusion The complete bi‐style mastoidectomy is the treatment of cholesteatoma otitis media ,which can effectively repair dam‐aged eardrum ,restore patient hearing to the maximum extent ,help to restore patient′s disease and improve the quality of life of pa‐tients .

The resistance to steroid remains a major trouble in the treatment of idiopathic nephrotic syn-drome,and the mechanisms are complicated. Recent studys have shown that multidrug resistance gene l and P-glycoprotein170,glucocorticoid receptor,renal pathology,gene mutation and complications are all closely related to the resistance to steroid. This review is focused on the mechanisms of steroid resistance of children′s idiopath-ic nephrotic syndrome.

Objective To compare the clinical manifestations,renal histological lesions,and the levels of urinary protein markers between the children with IgA nephropathy (IgAN) and those with IgM nephropathy (IgMN), and to determine whether urinary protein markers could predict the severity of renal histological lesions in children with IgAN and IgMN.Methods Seventy-four children with renal biopsy-proven IgAN and IgMN from January 2002 to October 2014 were enrolled in the study.The levels of IgG, albumin (Alb), transferrin (TRF), α1-microglobulin (α1-MG) ,β2-microglobulin (β2-MG) and N-acetyl-β-glucosaminidase (NAG) in morning urine samples before biopsy were measured.The semi-quantitative scores of mesangial hypercellularity (MC), glomerulosclerosis (GS), and tubule-interstitial damage (TID) were used to assess renal histological lesions.Multivariate Logistic regression analysis was used to determine whether urinary protein levels were independently associated with renal histological lesions.The area under the receiver-operating-characteristic curve (AUC) was calculated to assess the predictive ability of urinary protein markers.Results Seventy-four children (44 cases with IgAN,30 cases with IgMN) were included.The urinary levels of α1-MG and Alb were significantly higher in children with IgAN as compared to those with IgMN.The differences, however, did not remain significant after adjustment for age.The urine protein, as an independent factor associated with severe MC(＞ 5 mesangial cells per mesangial area) was TRF(B =0.010), and severe GS (≥ 10％ glomeruli showing segmental adhesion or sclerosis) was significantly correlated with Alb(B =0.001) ,and severe TID (focal or diffuse tubular and interstitial lesions) was significantly correlated with NAG(B =0.038).Urinary β2-MG was not significantly associated with severe MC, GS and TID.Urinary TRF, Alb and NAG achieved the best AUC of 0.85 (P ＜ 0.001) ,0.78 (P =0.002), and 0.78 (P =0.003), respectively, for predicting severe MC, GS, and TID.Conclusions Urinary proteins are useful to predict the severity of renal histological lesions in children with IgAN and IgMN.Urinary TRF, Alb and NAG have better predictive value.