Systemic juvenile idiopathic arthritis (sJIA) is systemic inflammatory disease classified as a subtype of juvenile idiopathic arthritis (JIA).Besides arthritis,it is characterised by systemic features such as spiking fever,skin rash,hepatosplenomegaly or serositis.It is becoming clear now that abnormalities in the innate immunity [cytokines such as interleukin (IL)-1,IL-6 and IL-18,and neutrophils and monocytes/macrophages rather than lymphocytes] play a major role in the pathogenesis of sJIA,distinguishing it from other JIA subtypes.Another distinctive feature of sJIA is its strong association with macrophage activation syndrome (MAS).Based on this,consensus is emerging that sJIA should be viewed as an autoinflammatory syndrome rather than a classic auto-immune disease.As a consequence of the progression in understanding the underlying mechanisms of sJIA,major changes in the management are evolving.Recently,remarkable improvement has been observed with IL-1 and IL-6 targeted therapies.These therapies might also change the long-term outcome of this disease.

Objective To investigate the clinical feature of Acinetobacter baumannii and to provide the basis for clinical rational use of anti-microbial agents.Methods ATB system was used to identify Acinetobacter baumanii, and antimicrobial resistance was determined by Kirby-Bauer method.The results were analyzed by Whonet 5.4 soft-ware.Results A total of the 358 strains Acinetobacter baumannii were isolated,91.62% were from sputum and throat swab.The main departments was ICU(52.23%);In 358 strains Acinetobacter baumannii,217 strains were multi-drug resistant strains(60.61%).The drug resistance to polymyxin B was the lowest 0% followed by minocy-cline 19.8% and cefoperazone/sulbactam 9.8%, the next was netilmicin 21.1% and meropenem 41.5%. Conclusion Acinetobacter baumannii shows multi-drug resistance, especially in ICU.Anti-microbial agents should be the rational use according to the results of drug susceptibility in order to reduce and control the incidence of noso-comial infections.

Objective To determine the main CT features and the key points of differential diagnosis of multilocular cystic renal cell carcinoma (MCRCC) classified according to 2004 WHO pathological diagnostic criteria. Methods According to the criteria, 40 patients were divided into two groups: MCRCC group and other subtypes of cystic renal cell carcinoma (CRCC). The CT findings were evaluated and compared between two groups for cystic content, wall, septum, nodularity, calcification and enhancement. ROC curve was used to determine the cut-off value of the possible CT feature which could distinguish MCRCC from other subtypes of CRCC. Results Seventeen cases of MCRCC group and 23 cases of CRCC group were included in this study according to the diagnostic criteria. MCRCC appeared as a well defined multilocular cystic mass with thin wall and sepia and no expansile solid nodules. Thickness of cystic wall and/or septum is was main CT findings to distinguish MCRCC from other subtypes of CRCC (P ＜ 0.01 ). The cut-off value of the thickness was 6 mm and its sensibility, specificity was 89% ,75% respectively. Conclusion Cystic wall and/or septum with a thickness of less than 6 mm are the main CT findings to dis tinguish MCRCC from other subtypes of CRCC.

Objective The purpose of this study was to explore the infection characteristic of Toxoplasma gondii (TOX),Rubella virus (RV),Cytomegalovirus (CMV) and Herpes simplex virus Ⅱ type (HSV-Ⅱ) (TORCH) infection in neonate in Tianjin area.Methods TOX-IgM/IgG,RV-IgM/IgG,CMV-IgM/IgG and HSV-Ⅱ-IgM/IgG were detected in serum of 2 273 neonate during 2015～2016 with enzymelinked immunosorbent assay (ELISA).Results The positive rates of TOX IgM,RV-IgM,CMV-IgM and HSV-Ⅱ-IgM were 0.00％(0/2 273),0.00％(0/2 273),0.88％(20/2 273) and 0.00％(0/2 273),respectively and those of TOX-IgG,RV-IgG,CMV-IgG and HSV-Ⅱ-IgG were 3.65％ (83/2 273),86.45％ (1 965/2 273),95.82％(2 178/2 273) and 8.27％(188/2 273),respectively.There was 0.66％ percent (15/2 273) of examinees who were infected by none of TORCH pathogens.There existed significant statistical difference for positive rate between TOX-IgG,RV-IgG,CMV-IgG and HSV-Ⅱ-IgG (x2 =6.747,P =0.000) with consequence of the highest positive rate being CMV-IgG.The positive rates of TOX-IgG and CMV-IgM in neonate of 2016 were significantly less than those in 2015 (x2 =5.789～7.505,P=0.006～0.016) but that of HSV-Ⅱ-IgG of 2016 was statistically higher than that in 2015 (x2 =6.073,P =0.014).The positive rate of CMV-IgM in male neonate in 2015 was significantly higher than that in 2016 (x2 =5.054,P =0.025).As a whole the positive rates of TOX-IgG,RV-IgG,CMV-IgG and HSV-Ⅱ IgG had no differences between different years,so did those between gender groups (x2 =2.23～6.963,P=0.073～0.526).The positive rates of TOX-IgG,RV IgG,CMV-IgG and HSV-Ⅱ-IgG in female neonate in 2015 were statistically different from those in 2016 (x2 =8.247,P =0.041).The female neonate in 2015 had higher infection proportion of TOX-IgG compared with that in 2016 (x2 =6.992,P=0.008).TORCH infection detected in 2 273 cases of neonate had one pathogen infection and multi-pathogen infection with overall six patterns of TORCH infection and all infection patterns had no relationship with year and gender,respectively (P＞0.05).Conclusion RV and CMV were primary pathogens in TORCH infection for neonate in Tianjin area and there were recent infections by CMV.TORCH infection varied in different years and gender groups,which provided experimental data and basis for epidemiology and prevention of TORCH in neonate.

Objective To explore the role of microRNA (miR)-22 and miR-1825 in the diagnosis and differential diagnosis of juvenile systemic lupus erythematous (JSLE).Methods The cases of JSLE hospitalized in Capital Institute of Pediatrics Teaching Hospital Affiliated to Peking University from June 2013 to May 2014 were selected as study group.The cases with systemic juvenile idiopathic arthritis (sJIA),nephrotic syndrome (NS),Kawasaki disease (KD),Henoch-Schonlein purpura(HSP) were selected as patients control group.The healthy children were selected as healthy control group.The expression levels of miR-22 and miR-1825 in the plasma of JSLE,sJIA,NS,KD,HSP and healthy children were detected by using real-time PCR respectively.Receiver operating characteristic curve (ROC) analysis was performed to evaluate the value of miR-22 and miR-1825 miRNA as a biomarker with the sensitivity and specificity.Three data bases,included Targetscan,PicTar and miRanda,were applied to predict the target gene.The target gene was analyzed by adopting Gene Ontology (GO) in terms of molecular function,biological process and cellular component,and by adopting Kyoto Encyclopedia of Genes and Genomes (KEGG) in terms of pathway.Results Compared with healthy children,the amount of miR-22 and miR-1825 in JSLE patients were lower,and there were significant differences(t =-3.076,-9.054,P ＜0.01,0.000 1).The levels of the miR-22 and miR-1825 miRNAs in controls of sJIA,NS,KD,HSP were significantly higher than those of JSLE (t =-4.410,-4.477,-4.494,-2.971,all P ＜ 0.000 1;t =-9.043,-6.045,-10.416,-8.712,all P ＜ 0.000 1),but there was no difference compared with healthy children(all P ＞ 0.05).The area under ROC curve(AUC) of miR-22 between JSLE and healthy children was 0.777.The AUC of miR-1825 between JSLE and healthy children was 1.000.The AUCs between JSLE and controls of sJIA,NS,KD,HSP of miR-22 were 0.731-1.000.The AUCs between JSLE and controls of sJIA,NS,KD,HSP of miR-1825 were 0.939-1.000.There was positive relation between the amount of miR-22 and complement C3 in plasma(r =0.493,P =0.027).Conclusions The amount of miR-22 and miR-1825 in the plasma of JSLE embrace the potential of distinguishing JSLE from healthy children,sJIA,NS,KD,HSP.MiR-22 has the ability to predict the activity of JSLE.

Objective To study the clinical manifestation,diagnosis,differential diagnosis and the essentials of management and treatment of fibrodysplasia ossificans progressiva (FOP).Methods Three cases of FOP were reported.The features of clinical manifestation and radiography were studied.The literature related to FOP was reviewed.Results FOP affected young children′s age of onset was between 10 days and 2 years (mean age 1 3 years).Mean disease duration was 5 3 years (range 2～11 years),and mean age 5 3 years (range 2～11 years) with sex ratio 1∶2 (boy∶girl).Soft tissue swelling in cervical and dorsal regions with or without local pain and warmth,and low fever were the early clinical manifestations.These nodules usually disappeared spontaneously,but some of nodules gradually developed ossification.The X ray features included ectopiac ossification most frequently in the soft tissue of the upper back and neck,next,the loin,chest and extremities.Two cases showed short hallux and hallux valgus.Exacerbation of the two cases was precipitated after muscle biopsy and careless venepuncture.All patients showed progressive extra articular bony ankylosis of most joints of axial and/or appendicular skeleton with severe movement restriction.Conclusion FOP is a rare and disabling genetic disorder of connective tissue.FOP should be diagnosed as early as possible and non invasively,based upon history,clinical and radiological findings.The finding of abnormalities of the great toe is helpful to diagnose FOP so that management can be early and adequate.Manogement principle includes avoiding conditions potentially provocative of abnormal ossification.The disease should be familiar to pediatricians.

Objective To explore the safety of mobilization and collection as well as the feasibility of selection of autologous peripheral blood stem cells (auto-PBSC) from patients with juvenile severe autoimmune diseases (AID) for autologous hematopoietic stem cell transplantation (auto-HSCT). The clinical significance of these procedure is evaluated. Methods Eight patients with AID, including four patients with systemic lupus erythematosus(SLE),two patients with dermatomysoitis, one patient with juvenile rheumatoid arthritis (JRA), one patient with multiple sclerosis(MS),underwent auto-HSCT. Auto-PBSCs were mobilized in 8 patients using cyclophosphamide(CTX) and granulocyte colony-stimulating factor (G-CSF), and their PBSCs were collected by CS-3000 Blood Cell Separator, then the CD34+cells were selected and purified by CliniMACS CD34+cell selection device. The CD34+ cells were frozenand preserved under -80 ℃ ALL patients received non-myeloablative or lymphoablative conditioning regimens which consisted of CTX/Mel/ATG or CTX/ATG or BEAM/ATG. All patient received CD34+ cells transplantation. The safety of mobilization and collection process of auto-PBSC as well asthe feasibility of selection and purification of CD34+cells were recorded and hematopoietic reconstruction were evaluated. Results All patients tolerated the collection process well, and there was no mobilization-related mortality. The number of collected MNCs and CD34+ cells were 8.35×108/kg and 7.92×106/kg respectively. The number of CD34+ and CD3+ cells after purification was 6.28×106/kg and0.71 ×105/kg respectively. The mean granulocytes and platelet engraftment occurred on days 11 and 15 after G-CSF regimen, and they can be collected using CS-3000 instrument. PBSC mobilization and collection from patients with juvenile severe AID is safe. The CD34+ cell can be highly purified. The auto-PBSC CD34+cell transplantation is an alternative therapy for severe AIDs that do not respond to conventional treatments.

Objective To summarize the imaging features of computed tomography (CT)and magnetic resonance imaging (MRI) combined with multi-technology imaging and compare its effects in the preoperative evaluation of malignant perihilar biliary obstruction.Methods The clinical data of 20 patients receiving CT and MRI who were diagnosed with malignant perihilar biliary obstruction by pathological examination at the Wuxi Second People's Hospital between January 2008 and April 2014 were retrospectively analyzed.Patients receiving CT combined with negative-contrast CT cholangiopancreatography (nCTCP) and computed tomography angiography (CTA) were allocated into the CT group,and patients receiving MRI combined with magnetic resonance cholangiopancreatography (MRCP) and magnetic resonance angiography (MRA) were allocated into the MRI group.The images of the 2 groups were analyzed by 2 independent reviewers.The classification of malignant perihilar biliary obstruction,hepatic artery and portal vein invasions and lymph node and organ metastases were evaluated respectively,and then the results of evaluation were compared with the results of surgery and pathological examinations.The comparison between the accuracy of imaging examination in the 2 groups and accuracy of perihilar biliary obstruction classification were analyzed by the chi-square test.The comparison of evaluating accuracy (sensitivity and specificity) among vascular invasion and lymph node and organ metastases were done by the receiver operating characteristic (ROC) curve analysis,and the comparison of its accuracy were done by the z-score test.Results The imagings of bile duct involvement of the 2 groups showed that there were irregular thickening bile duct wall combined with retrograde intrahepatic bile duct dilatation.The symptoms of vascular invasion included the stricture and occlusion of blood vessels or more than half of vascular contact surface with tumor.The symptoms of lymph node metastasis included the enlarging short-axis or round-like circular enhanced lesions.The symptoms of organ involvement included the unclear boundary of lesions or low-density necrotic foci within organ.All the 20 patients underwent the surgical treatment,including 13 patients with hilar cholangiocarcinoma and 7 patients with gallbladder carcinoma.Hepatic artery invasions were detected in 5 patients,portal vein invasions in 10 patients,lymph node metastases in 10 patients and organ metastases in 4 patients.The cases of classification of perihilar biliary obstruction,hepatic artery invasion,portal vein invasion,lymph node metastasis and organ metastasis which were evaluated respectively by reviewer 1 and 2 were 18/18,19/18,18/18,17/16 and 18/19 in the CT group and 17/16,14/13,17/16,15/14 and 19/18 in the MRI group.The imaging of the 2 groups were compared with the evaluating accuracies of classification of malignant hilar biliary obstruction,hepatic artery and portal vein invasions,lymph node and organ metastases in the 2 groups,showing no significant differences (x2=12.593,8.889,z=1.823,1.956,0.462,0.817,0.977,0.751,0.233,1.403,P＞0.05).Conclusion CT and MRI for malignant hilar biliary obstruction had the same imaging features,meanwhile,they can provide an equivalent performance in the classification of malignant hilar biliary obstruction,hepatic artery and portal vein invasions and lymph node and organ metastases.

Objective To semi-quantitatively assess the MRI manifestations of knee in patients with juvenile idiopathic arthritis (JIA) and to explore the relationship between the semi-quantitative scores with clinical inflammatory biomarkers. Methods Fifty children diagnosed as JIA and presented with knee pain, swelling or limitation were enrolled and their clinical and imaging findings were retrospectively analyzed. Contrast-enhanced MRI scan of the knee were performed in all cases (a total of 50 knees). MRI abnormalities, including synovial hypertrophy, joint effusion, bone marrow edema, joint cartilage injury and bone erosion, were assessed with a semi-quantitative score system. The erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) at the same period with MRI were collected. The relationships between the MRI scores and laboratory biomarkers (CRP and ESR) were analyzed with Spearman correlation analysis. Results In MR images of all the 50 knees with JIA, synovial hypertrophy(43, 86%)and joint effusion(40, 80%)were major abnormalities, bone marrow edema was seen in 6 knees, joint cartilage injury in 12 knees and no bone erosion was seen. The scores of synovial hypertrophy, joint effusion, marrow edema, joint cartilage injury and bone erosion were 7(0-12), 3(0-7), 0(0-6), 0(0-10), 0, respectively. There was significant correlation between synovial hypertrophy and joint effusion(r=0.719, P=0.001). There were positive relationship between synovial hypertrophy and ESR and CRP(r=0.306 and 0.285; P=0.031 and 0.043, respectively).Other indexes had no significant relationship with ESR or CRP. Conclusions MRI could comprehensively evaluate knee involvement in patients with JIA. Joint effusion could be a useful reference to evaluate the condition of synovitis for pediatric patients with non-enhanced MR images.

OBJECTIVETo investigate the effects of thoracic epidural blockade (TEB) on plasma fibrinogen (FIB) levels.

METHODSThirty cases of dilated cardiomyopathy (DCM) were selected randomly into a TEB group and a control group. TEB patients were subjected to a persistent TEB (T1 - 5), and injected with 0.5% lidocaine 3 - 5 ml every two or four hours for four weeks in addition to routine medicine, while patients in the control group were given routine medicine only. Plasma concentrations of FIB were measured using the micro-capillary assay. Doppler echocardiography was performed before and after the treatment.

RESULTSPlasma concentrations of FIB in two groups were greater than the normal value before the treatment. There was a significant decrease of plasma concentrations of FIB in the TEB group after the treatment (4.2 +/- 1.3 g/L vs 3.6 +/- 0.9 g/L, P < 0.05), but there was no significant change in the control group (4.2 +/- 1.2 g/L vs 4.3 +/- 1.9 g/L, P > 0.05). After four weeks of treatment, the left ventricular end diastolic diameters (LVEDD) of TEB patients were reduced (72 +/- 10 mm vs 69 +/- 10 mm, P < 0.05) and the left ventricular ejection fraction (LVEF) of TEB patients increased significantly (33% +/- 13% vs 44% +/- 14%, P < 0.05). In contrast, LVEDD (73 +/- 11 mm vs 73 +/- 12 mm, P > 0.05) and LVEF (32% +/- 14% vs 33% +/- 12%, P > 0.05) did not change significantly in the control group.

CONCLUSIONSThe results suggest that plasma FIB levels in patients with DCM were decreased by performing a TEB, in addition to a reduction of the enlarged cardiac cavity and an improvement in cardiac systolic dysfunction. TEB might contribute to lowering the occurrence of thrombus and thromboembolism in patients with DCM. TEB might be a promising therapeutic method to improve the prognosis of DCM patients.

Adult ; Aged ; Anesthesia, Epidural ; Autonomic Nerve Block ; Cardiomyopathy, Dilated ; blood ; therapy ; Female ; Fibrinogen ; analysis ; Humans ; Male ; Middle Aged