Objective To summarize experiences of surgical correction of aortopulmanary septal defect (APSD) in children. Methods Fifteen children with APSD,aged 5 months to 11 years,weighed 4.5 to 21.0kg,underwent surgical correction. Based on Richardson's classification,type I in 7 cases,type II in 3,and type III in 5. Eight cases were associated with other cardiac defects (53.5%),including 4 cases with complicated cardiac defects (26.7%). Operative technique included patch repair of defect in 8 cases with type I and II,an intraaotic synthetic baffle directed pulmonary blood from the APSD to the right pulmonary artery (RPA) in 3 cases with type III,an artificial conduit was used to connect the RPA with main pulmonary artery (MPA) and a flap of aortic wall was excised along with the anomalous RPA to extend the anastomosis in each case with type III,direct suture was used in 2 cases. Other associated cardiac defects were repaired simultaneously. Results The post-operative mortality rate was 6.7% (1/15). Eleven cases were followed-up from 3 months to 13 years in good condition. Conclusion APSD associated with complicated cardiac defects is apt to be misdiagnosed. Correct diagnosis can be made by 2-D echocardiography, cardiac catheterization angiography,and MRI. The operation should be done as early as possible once definite diagnosis is made. Operation should be done infancy to prevent development of pulmonary vascular disease. In type III APSD and APSD associated with complicated cardiac defects,operative mortalith is high. Preoperative accurate diagnosis and full understanding of the pathophysiology are the keys to an optimal surgical correction.

ObjectiveCardiopulmonary bypass (CPB) and its related ischemia reperfusion injury may cause endothelial cell injury.To study the protective effects of sodium ferulate in vascular endothelial function during CPB by testing the changes of vascular endothelial cell( CEC),nitric oxide( NO) and endothelin-1 ( ET-1 ) in children with congenital heart disease.MethodsSixty patients with congenital heart disease,including 28 males and 32 females were studied.The mean age was (19.7 ±10.4) months and body weight (10.5 ±6.1) kg.There were 37 VSD,8 ASD,7 TOF,5 TAPVC and 3 CAVC,among them 26 patients had pulmonary hypertension.They were randomly divided in to two groups:sodium ferulate group ( group S,n = 30),and control group ( group C,n =30) .Sodium ferulate (8 mg/kg) was given intravenously before CPB.Blood samples were taken from the arterial line at following time points:before CPB (TO),bypass 30 min(Tl ),the termination of CPB (T2 ),2h after operation ( T3 ) and 6h after operation ( T4 ),respectively for determination the concentration of vascular endothelial cell (CEC) in the blood,the concentration of nitric oxide (NO) and endothelin-1 ( ET-1) in the plasma.ResultsThere were no significant difference for the two groups regarding above parameters at TO ( P > 0.05).The level of CEC was significantly elevated after CPB in both groups ( P < 0.05 ) .CEC were lower at T2 in group S than in group C ( P < 0.05 ) .NO was decreased in both groups,but was higher in group S at T2,T3 and T4 ( P < 0.05 ) .The concentration of plasma ET-1 was not significantly different before CPB,but there was a slight decrease at T1,and then it was significantly increased in both groups (P<0.05).But it was lower in group S than in group C at T1,T2,T3 and T4(P<0.05 orP<0.01).ConclusionThere was severe endothelial cell damage during CPB.Sodium Ferulate can effectively antagonize the secretion of ET-1 to promote the formation of NO.Therefore,it reduces CPB-induced endothelial cell damage and protects vascular endothelial function during CPB.

This study was undertaken to investigate the anti-lymphocytic malignacy immunologic effects induced by TCR idiotypic DNA vaccine on BALB/c mice. CEM lymphoma cell line and BALB/c mice were used as models. The rearrangement gene fragment coding TCR Vbeta region of CEM cell line was obtained by RT-PCR technique. The PCR product was cloned into the eukaryocytic expression vector pcDNA3 to be used as DNA vaccine. The experimental animals were immunized by intramuscular injection with DNA vaccine. The specific anti-idiotypic antibody was detected by indirect immunofluorescence assay. The specific anti-idiotypic cellular immunity was detected by CTL activity assay using MTT method. The results showed that specific anti-idiotypic antibody in the immunized mice sera could be found since four weeks after immunization and came to the peak of titer on the sixth week. Using IL-6 as immunological adjuvant could significantly increase the antibody titers. It was concluded that the TCR idiotypic DNA vaccine could induce effectively the specific anti-lymphoma idiotypic antibody in BALB/c mice. Using IL-6 as immunological adjuvant could significantly increase the antibody titers induced by idiotipic DNA vaccine.

OBJECTIVETo investigate the anti-human CEM lymphoma cell activities induced by TCR idiotypic DNA vaccine containing different antigen determinants in BALB/c mice.

METHODSThe specific rearranged gene fragment encoding TCRVbeta region of CEM cell line was obtained by RT-PCR technique. The PCR product was cloned into eukaryocytic expression vector pcDNA3, which was used as DNA vaccine and template for PCR amplifying different antigen determinant. Gene fragments encoding different antigen determinant were amplified and cloned into pcDNA3, separately. The experimental mice were immunized by intramuscular injection of the DNA vaccines. The specific anti-idiotype antibodies were detected by indirect immunofluorescence assay.

RESULTSTCRbetaV of CEM cell line contains five antigen determinants. Specific anti-idiotype antibody was detected in all of the six mice immunized with DNA vaccine containing all the five determinants (the highest titer was 1:480). Although the antibody could also be detected in four of the six mice immunized with DNA vaccine containing four of the five antigen determinants, the antibody titer was lower (the highest titer was 1:80). DNA vaccine containing two of the five determinants could not induce the specific antibody.

CONCLUSIONThe idiotypic DNA vaccine containing the whole TCRbetaV five antigen determinants could induce the specific anti-lymphoma idiotypic antibody in BALB/c mice.

Amino Acid Sequence ; Animals ; Antibodies, Anti-Idiotypic ; blood ; immunology ; Base Sequence ; Complementarity Determining Regions ; genetics ; immunology ; Epitopes ; genetics ; immunology ; HL-60 Cells ; Humans ; Lymphoma ; immunology ; Mice ; Mice, Inbred BALB C ; Molecular Sequence Data ; Receptors, Antigen, T-Cell, alpha-beta ; genetics ; immunology ; Sequence Analysis, DNA ; Tumor Cells, Cultured ; Vaccines, DNA ; genetics ; immunology

Objective:Analyzed the clinicopathological features of thymoma with alopecia areata, and discussed the pathogenesis and treatment methods.Methods:The clinicopathologic data of patients with thymoma who underwent surgery from August 1, 2015 to July 31, 2020 in Beijing Tongren Hospital, Capital Medical University were reviewed. Transversally analyzed the patients of thymoma with alopecia areata and longitudinally compared with the patients of thymoma without alopecia areata after 1﹕10 matched by propensity score matching.Results:A total of 252 patients of thymoma were enrolled, including 6 patients with alopecia areata, accounting for 2.38%. The anti-AchR antibody, CD4 + /CD8 + T inversion in serum and myasthenia gravis were present in the all 6 thymoma patients with alopecia areata, which were significantly higher than those in the group of thymoma without alopecia areata. Besides myasthenia gravis, the proportion of complicated with other autoimmune diseases in thymoma patients with alopecia areata was significantly higher than that of thymoma patients without alopecia areata[83.33%(5/6) vs. 20.00%(12/60), P=0.003]. After operation, 5 patients’ alopecia areata were improved in 6 thymoma patients with alopecia areata(83.33%, 5/6). Conclusion:The thymoma patients with alopecia areata always complicated with myasthenia gravis and other autoimmune diseases. The pathogenesis may be associated with autoimmune CD8 + T lymphocytes produced by thymoma. At present, surgery is still the most effective way to improve thymoma-associated alopecia areata.

BACKGROUND: Thymomas are the most common primary malignant tumors of anterior mediastinal. However, there are no specific laboratory indicator for the diagnosis the diagnosis of thymoma. The aim of this study was to screen out a tumor marker for diagnosis of thymoma by mRNA microarray analysis and confirmed it. METHODS: By mRNA microarray analysis of 31 thymomas and peritumoral thymic tissues, we found that the transcription level of neuronal pentraxin 1 (NPTX1) gene was up-regulated more than 4 times in thymomas. To further verify the above results, we detected the transcription and expression level of NPTX1 in 60 thymoma and 30 thymic cyst patients by quantitative Real-Time polymerase chain reaction (PCR), immunohistochemistry and enzyme-linked immunosorbent assay (ELISA). Furthermore, the diagnostic value of NPTX1 in thymoma by receiver operating characteristic curve (ROC) was analyzed. RESULTS: The transcription level of NPTX1 mRNA in thymoma tissues was significantly higher than that in the thymic tissues of control group [(2.88±1.02) vs (1.35±0.47), P<0.001); The expression level of NPTX1 in thymoma tissues was significantly higher than that in the thymic tissues of control group (2 vs 1, P<0.001); The preoperative serum level of NPTX1 protein in thymoma patients were significantly higher than that in the thymic cyst patients of control group [(1,018.29±209.38) pg/mL vs (759.95±66.02) pg/mL, P<0.001]; At the threshold of 842.22 pg/mL, sensitivity and specificity of NPTX1 as a serologic marker were 85.00% and 93.33%, respectively for thymoma. ROC showed that the area the under curve (AUC) of NPTX1 was 0.902. CONCLUSIONS NPTX1 was highly expressed in thymoma patients, and had diagnostic value for thymoma.