Objective: To analyze the clinical characteristics and infection of children with Takayasu′s arteritis(TA) for improving the awareness of the disease. Methods: A retrospective study was performed on the 24 children with TA in our hospital. Results: The average onset age was 9.3±3.2 years old, the ratio of male to female was 1∶3. The most common TA type was thoracic abdominal aortic type (54.2%) in clinical classification. The initial symptoms included high blood pressure, dizziness/headache, fever and fatigue, etc.Six cases (25%) had tuberculosis infection, including 1 case of tuberculosis. There are 3 patients (8.3%) with elevated O levels, 2 patients (8.3%) with EB virus infection and 1 patient (4.2%) with small viral B19 infection. Conclusions The onset of TA in children is complicated. It is important to take examinations carefully for early diagnosis, avoiding delay treatment and bad prognosis.

OBJECTIVETo observe the in vivo antitumor activity of murine liver tumor vaccine expressing MIP-1alpha mediated by recombinant adenoviral vector.

METHODSThe infection efficacy was measured by GFP expression 48 hours after infection of Hepa1-6, and the number of cells was counted daily for 14 days. 5 x 10(6) modified Hepa1-6 cells were inoculated subcutaneously to C57BL/6 mice and the tumor-free animals were rechallenged by 2 x 10(6) wild-type Hepa1-6 cells or syngenic EL4 cells four weeks later. The tumor volume was measured twice a week.

RESULTSAdenoviral vectors could efficiently infect Hepa1-6 cells in vitro, and the in vitro growth rate of AdmMIP-1alpha modified Hepa1-6 cells was not affected; however the in vivo tumorigenicity was significantly decreased, compared with that of control vector modified Hepa1-6. Rechallenge of the tumor-free mice four weeks after administration of AdmMIP-1alpha with the parental Hepa1-6 cells resulted in significant inhibition of tumor growth, but there was no significant difference when rechallenged with EL4.

CONCLUSIONSThe liver cancer cells expressing mMIP-1alpha mediated by recombinant adenoviral vector decrease tumorigenicity and elicit specific immunological protection, and could be used as an effective liver tumor vaccine.

Adenoviridae ; genetics ; Animals ; Cancer Vaccines ; immunology ; Chemokine CCL3 ; Chemokine CCL4 ; Genetic Therapy ; Liver Neoplasms, Experimental ; therapy ; Macrophage Inflammatory Proteins ; genetics ; Mice ; Mice, Inbred C57BL ; Vaccines, Synthetic ; immunology

Objective: To evaluate the efficacy and side effects of tocilizumab for the treatment of systemic juvenile idiopathic arthritis. Method: In this prospective self case-control study, the children diagnosed with refractory systemic juvenile idiopathic arthritis admitted to Department of Rheumatism and Immunology of Children's Hospital Affiliated to Capital Institute of Pediatrics from December 2013 to June 2016 were enrolled and information before and after treatment of tocilizumab was analyzed. The tocilizumab was introvenously guttae in a dose of 8-12 mg/kg every 2 weeks. Complete blood count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) were tested before and after the application of tocilizumab. Detailed clinical manifestations were recorded. All results were analyzed by χ² test and t test. Result: Forty patients with a median age of (6.6±3.7) years were enrolled, including 15 males and 25 females. All of the patients presented with fever and 38 patients got normal temperature 24-48 hours after treatment with tocilizumab. Symptoms disappeared in 13 and improved in 4 patients after treatment among the 17 patients who presented with arthritis. Within the 10 patients who manifested with rashes, 9 patients' rashes disappeared without relapse accompanied by the normalization of temperature after the treatment of tocilizumab. One patient got normal temperature but intermittently emerged rashes after symptoms of arthritis improved. In the 40 patients, 38 well tolerated tocilizumab while 2 showed rashes and chill which disappeared shortly after antianaphylaxis treatment. No severe treatment-related infection was found in any patients. According to the study, the white blood cell counts(×10⁹/L), CRP(mg/L) and ESR(mm/1h) tested 2 weeks after the treatment with tocilizumab were significantly lower than that before treatment(12.1±1.2 vs. 16.5±1.8, 47±8 vs. 67±9, 21±5 vs. 57±6, t=2.75, 3.98, 5.22, P=0.009, 0, 0, respectively). No significant changes were found in concentration of IL-6 and TNF-α (65(207) vs. 45(137) ng/L, and 14(6) vs. 17(19)ng/L, Z=-1.247 and-1.285, P=0.212 and 0.199 respectively). Conclusion Tocilizumab is a treatment with good efficacy and safety for refractory systemic juvenile idiopathic arthritis. Adverse effects would be found in some patients.

Objective: To summarize the clinical data of 15 patients with fibrodysplasia ossificans progressiva (FOP), follow up and analyze the characteristics of the joint involvement in FOP. Method: From May 2005 to December 2016, fifteen FOP cases had been diagnosed in the Children′s Hospital Capital Institute of Pediatrics. All medical records and follow-up data were collected and a retrospective analysis was made on the joint involvement in FOP. Pearson correlation analysis was used for data, P<0.05 for the difference was statistically significant. Result: There were 8 males and 7 females in 15 cases. The age of onset was 2(1-6)years. The age at diagnosis was 6 (4-9) years. All cases had hallux valgus deformity and bone mass formation. Twelve cases had joints involvement on enrollment into this study: 8 cervical vertebra, 7 shoulder joint, 5 hip joint, 4 elbow joint, 3 wrist joint, 2 temporomandibular joint, 2 lumbar vertebra. The age of diagnosis and duration of disease were positively correlated with the number of the involved joints (r=0.523, 0.628; P=0.045, 0.012); mild changes were found in joint imaging. Thirteen cases received telephone follow-up, the average duration of follow-up was 6(3-7)years, no change in 11 cases, disease progress in 2 cases. Conclusion Joint involvement is a common complication of FOP, especially the cervical vertebra.Multiple joints involvement, dominant functional impairment, and mild imaging changes are the characteristics of joint lesions caused by FOP.The number of involved joints gradually increases with increase of age of the patients and the prolonged course of the disease.

Objective:To investigate the prognostic factors of renal function after nephron sparing surgery (NSS) in renal tumor patients.Methods:The data of 115 patients who underwent NSS in our hospital from December 2016 to December 2018 were retrospectively analyzed. There were 75 males and 40 females, aged (49.50±12.94) years. The body mass index was (24.59±3.59) kg/m 2. The maximum diameter of the tumor was (3.66±1.32) cm. The R. E.N.A.L. score was (6.43±1.60). Laparoscopic partial nephrectomy was performed in 61 cases and robot-assisted laparoscopic partial nephrectomy was performed in 54 cases, and all of which were successfully completed. Operative time, WIT and postoperative pathological results were recorded. Blood creatinine value, GFR of affected kidney, GFR of healthy kidney, total GFR, GFR preserving rate (the ratio of postoperative GFR to preoperative GFR), functioning parenchymal volume (FPV) of the affected kidneys, and FPV preserving rate of the affected kidneys (the ratio of postoperative FPV and preoperative FPV) were recorded 6 months after surgery. FPV was measured by the ellipsoid approximation on CT images before and after surgery. Paired sample t test was used to compare GFR and FPV before and after surgery. Spearman rank correlation analysis was used to evaluate the correlation between the study factors and GFR preserving rate of the affected kidneys. Multivariate linear regression models were used to analyze independent predictors of renal function of the affected kidneys. Independent sample t test was used for comparison between group of WIT≤25 min and group of WIT>25 min. Results:All of the 115 patients in this study underwent successfully operations, with the median operation time of 135(75-245) min, and WIT(24.57±5.51) min. Postoperative GFR of the affected kidneys(35.50±7.81)ml/(min·1.73 m 2) was significantly different from preoperative GFR( P<0.001). The FPV preserving rate of the affected kidneys was (84.28±4.37)%, which was significantly lower than that preoperative FPV of the affected kidneys ( P<0.001). Spearman rank correlation analysis showed that there was a strong positive correlation between the FPV preserving rate of the affected kidneys and the GFR preserving rate of the affected kidneys ( r=0.802), WIT was negatively correlated with the GFR preserving rate of the affected kidneys ( r=-0.503). Multiple linear regression analysis showed that preoperative GFR of the affected kidneys ( b=-0.150, P=0.008), WIT ( b=-0.443, P<0.001) and the FPV preserving rate of the affected kidneys ( b=1.638, P<0.001) were independent predictors of the GFR preserving rate of the affected kidneys. WIT>25 min group had a significantly lower GFR preserving rate of the affected kidneys than WIT≤25 min group [(68.77±10.88)% vs.(79.34±8.88)%, P<0.001]. Conclusions:In the case of short WIT (<30 min), the reservation of normal renal tissue is the most important variable prognostic factor of renal function after NSS, and short WIT plays a secondary role. Under the premise of complete tumor resection, normal renal tissue should be reserved as much as possible and WIT should be controlled within 25 min.