Humans ; Receptors, Chimeric Antigen ; T-Lymphocytes ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy* ; Prognosis ; Immunotherapy, Adoptive ; Hematopoietic Stem Cell Transplantation ; Receptors, Antigen, T-Cell ; Recurrence

Background::The role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children with high-risk (HR) T-cell acute lymphoblastic leukemia (T-ALL) in first complete remission (CR1) is still under evaluation. Moreover, relapse is the main factor affecting survival. This study aimed to explore the effect of allo-HSCT (especially haploidentical HSCT [haplo-HSCT]) on improving survival and reducing relapse for HR childhood T-ALL in CR1 and the prognostic factors of childhood T-ALL in order to identify who could benefit from HSCT.Methods::A total of 74 newly diagnosed pediatric T-ALL patients between January 1, 2012 and June 30, 2018 were enrolled in this retrospective study. Patients were stratified into the low-risk chemotherapy cohort ( n = 16), HR chemotherapy cohort ( n = 31), and HR transplant cohort ( n = 27). Characteristics, survival outcomes, and prognostic factors of all patients were then analyzed. Results::Patient prognosis in the HR chemotherapy cohort was significantly worse than that in the low-risk chemotherapy cohort (5-year overall survival [OS]: 58.5% vs. 100%, P = 0.003; 5-year event-free survival [EFS]: 54.1% vs. 83.4%, P = 0.010; 5-year cumulative incidence of relapse [CIR]: 45.2% vs. 6.3%, P = 0.011). In HR patients, allo-HSCT improved the 5-year EFS and CIR compared to that of chemotherapy (5-year EFS: 80.1% vs. 54.1%, P = 0.041; 5-year CIR: 11.6% vs. 45.2%, P = 0.006). The 5-year OS was higher in the HR transplant cohort than that in the HR chemotherapy cohort (81.0% vs. 58.5%, P = 0.084). Minimal residual disease re-emergence was an independent risk factor for 5-year OS, EFS, and CIR; age ≥10 years was an independent risk factor for OS and EFS; and high white blood cell count was an independent risk factor for EFS and CIR. Conclusion::Allo-HSCT, especially haplo-HSCT, could effectively reduce relapse of children with HR T-ALL in CR1.

Objective:To assess the efficacy and the safety of the radiofrequency catheter ablation (RFCA) for the septal accessory pathway (AP) in children.Methods:From September 2013 to March 2019, 626 patients plan to underwent RFCA for paroxysmal supraventricular tachycardia (PSVT) in Shanghai Children′s Medical Center Affiliated to Shanghai Jiaotong University School of Medicine.Among them, 74 consecutive patients with right or left septal APs were included in the study and their clinical and RFCA data were analyzed.Results:The age of these 74 children (45 males, 29 female) was (7.8±3.5) years, ranging from 10 months to 13 years.The body weight (BW) was (27.7±14.4) kg, with 3 patients BW<15 kg.A discordant ventricular wall motion (DVWM) was found in 5 patients, and the combined congenital heart diseases were discovered in 2 patients.A three dimensional mapping system was applied in 69 ablations, and 3 ablations were performed only with the fluoroscopy monitor of 5 cases.According to the AP location, the number of cases located in the anteroseptal, the midseptal, the mouth of coronary sinus, the left posteroseptal and the right posteroseptal, were 28, 18, 10, 10 and 8, respectively.The ablation operations were applied in 72 patients.The initial acute success reached in 67 (93.1%) patients.The ablation energy was (18.0±1.8) W, the fluoroscopy time during the ablations was (4.7±2.7) minutes, and the procedure duration was (151.5±58.6) minutes.One inadvertent complete atrioventricular block (AVB) was noted as the ablation-related complication.All 5 children with the pre-DVWM were recovered after ablations.During a follow-up of (23.8±10.8) months, 4 patients experienced the recurrence of preexcitation syndrome atrioventricular reentrant tachycardia.Conclusions:With the 3D-mapping system, the RFCA of septal APs can be performed safely and effectively in pediatric patients of paroxysmal supraventri-cular tachycardia.However, as the ablation-related complication, AVB should not be ignored.

Objective:To establish a novel decision tree-based algorithm in complete Kawasaki disease(cKD)and evaluate its diagnostic value in incomplete Kawasaki disease(iKD)and pediatric infectious disease(IF)with common clinical characteristics, which facilitates early and accurate diagnosis of iKD.Methods:Based on inclusion criteria of KD and IF, clinical and laboratory data of patients with cKD, iKD and IF from Shanghai Children′s Medical Center between December 2018 and December 2019 were collected.The training data set included cKD and random half number of IF patients, and validation data was constituted with iKD and the rest of IF patients.The decision tree algorithm analysis was performed in training data set to generate a clinical diagnostic panel for cKD.Finally, the decision tree-based algorithm was verified and evaluated among the iKD patients.Results:A single statistical analysis was performed on 26 examination indexes of constructing decision tree-based algorithm.It was found that 16 examination indexes were obviously different between cKD and IF patients, and 17 examination indexes were significantly different between iKD and IF patients.According to date set of cKD and IF patients, the decision tree-based algorithm was established.The erythrocyte sedimentation rate>35mm/h, N-terminal atrial brain natriuretic peptide precursor≥315 pg/ml, CD3 -/CD19 + %≥21%, and the amount of neutrophil≥8.5×10 9/L were constructed as key elements.The algorithm had a sensitivity of 0.947 and a specificity of 0.963, and correctly classified subjects with iKD who were difficult to be distinguished from patients with IF. Conclusion:A decision tree-based algorithm based on the examination indexes of cKD is one of the effective methods to identify iKD and IF, which provides strong support for the early clinical diagnosis of iKD.

Objective:To review the imaging characteristics and evaluate the diagnostic value of Doppler echocardiography for congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa (PMAIVF).Methods:Between June 2008 and January 2020, 4 patients with PMAIVF were diagnosed by CTA, MRI and operative findings in Shanghai Children′s Medical Center and Children′s Hospital of Soochow University. The echocardiographic characteristics were analyzed retrospectively in these children.Results:PMAIVF was characterized by a pulsatile echo-free sac that expanded in systole and collapsed in diastole with to-and-fro blood flow on color and pulsed-wave Doppler echocardiography between the mitral leaflet and the aortic annulus. Three cases were diagnosed correctly, and 1 case was misdiagnosed as left atrial mass.Conclusions:PMAIVF can be diagnosed accurately by Doppler echocardiography, but it is prone to be misdiagnosed and must be distinguished from aortic root abscess, atrial mass and coronary artery fistula.

Objective: To investigate the correlations between single nucleotide polymorphisms (SNPs) in the D-loop of mitochondrial DNA (mtDNA) and the disease risk as well as the prognosis of diffuse large B cell lymphoma (DLBCL). Methods: Blood samples from 108 DLBCL patients treated at the Department of Hematology of the Fourth Hospital of Heibei Medical University during July, 1991 and July 2012 were collected for this study; in addition, blood samples from 159 healthy controls during the same period were also collected. DNA was extracted according to the standard protocols for PCR amplification and SNP locus genotype analyses. The risk of D-loop SNPs was investigated by case-control study. Results: The minor alleles of nucleotides 73A/G, 263A/G, 315C/C insert were associated with a decreased risk for DLBCL. The minor allele of the nucleotides 200G/Awas associated with an increased risk for DLBCL. To further evaluate the predictive function of D-loop SNPs in DLBCL patients, five SNP sites were identified by Log-Rank test that with statistically significant prediction value of DLBCL survival in a univariate analysis. In a multivariate analysis, allele 16304 was identified as an independent predictor of DLBCL prognosis. The survival time of DLBCL patients with 16304C was significantly shorter than that of patients with 16304T (RR=0.513, 95% CI=0.266-0.989, P<0.05). Conclusion: The analysis of D-loop SNPs in mtDNA can help identifying the occurrence risks and poor prognosis subtypes of DLBCL.

Objective To evaluate the effect of single-nucleotide polymorphisms at the miRNA binding site rs3660 in the 3'-untranslated region of the KRT81 gene (miR-SNPs) on the cancer risk and clinical prognosis of non-Hodgkin's lymphomas (NHL).Methods The single-nucleo-tide polymorphisms of rs3660 was genotyped with ligation detection reaction method.The association of rs3660 with NHL survival was calculated with log-rank test using Kaplan-Meier method.Multivariate survival analysis was performed using a Cox proportional hazards model.Results The rs3660 genotype distribution difference was not statistically significant between the case and control group (P =0.50).Patients carrying the rs3660 CG/CC genotype exhibited a significantly longer survival time than patients carrying the GG genotype (P =0.012).In addition,rs3660 was associated independently with the survival of NHL patients in multivariate analysis (RR=0.589,95％ CI:0.415-0.832,P =0.004).The association of this miR-SNP with NHL survival was further confirmed in the peripheral T cell lymphoma (PTCL) subtype.Conclusion Our results indicate that KRT81 rs3660 GG type is an independent prognostic marker in NHL.

Purpose To evaluate features of the left ventricular twist in patients with atrial septal defect (ASD) using speckle tracking imaging (STI) in order to guide clinical application.Materials and Methods Fifty-eight patients with ASD confirmed by ardiac ultrasound in Shanghai Children's Medical Center from October 2015 to January 2016 were enrolled in this study as case group,which were further divided into group ASD-A with 30 cases and group ASD-B with 28 cases according to the volume of right ventricular.The volume of right ventricular was significantly increased in the group ASD-A,but the volume of right ventricular was not significantly increased in the group ASD-B.At the same time,30 normal children with matched age and sex were chosen as control group.The parameters of left ventricular twist motion in each group were measured and compared by using STI.Results In group ASD-A,The basal and apical part of 6 children rotated counterclockwise.Compared with those in the control group,the basal rotation angle and apical rotation angle of left ventricular in group ASD-A were significantly higher (P＜0.01),and the peak twist and torison of left ventricular in group ASD-A were also higher (P＜0.05).Compared with those in the control group,only the apical rotation angle in group ASD-B was higher (P＜0.05),but the rest parameters of the left ventricular twist motion in group ASD-B were not statistically significantly higher (P＞0.05).Conclusion The significant increase in the volume of right heart load in ASD impacts on the basal and apical rotation of left ventricular.

The incidence of inflammatory bowel disease in China is rising on a yearly basis, followed by an increased number of patients who require surgery and those who suffer from maneuver postoperative complications. Surgical treatment is important in the management of patients with inflammatory bowel diseases. Management and prevention of postoperative complications is also a key factor to the success of surgical treatment if it is not more important than the surgical procedure itself. In this article, the most recent literatures in this field will be reviewed combined with our own clinical experiences. The types, risk factors, preoperative prevention strategies as well as postoperative management of surgical complications of patients with inflammatory bowel disease will be discussed.
China ; Digestive System Surgical Procedures ; adverse effects ; Humans ; Incidence ; Inflammatory Bowel Diseases ; surgery ; Postoperative Complications ; prevention & control ; therapy ; Risk Factors

Patients with inflammatory bowel disease (IBD) may require surgical intervention for refractory disease or complications. Prompt surgery and appropriate surgical procedures are critical when surgery is indicatedd. With continuous optimization and innovation of surgical procedures, there have been significant changes in the concepts and operations of IBD in the past century. Learning the evolution of surgical treatment for IBD could help us understand the rationale, indications, and pertinent techniques of surgical procedures. Innovations are emerging in IBD management including the advent of biological agents, laparoscopy, and multi-disciplinary team approach, it is imperative for IBD specialist to learn the state-of-the-art knowledge.
Digestive System Surgical Procedures ; Humans ; Inflammatory Bowel Diseases ; Laparoscopy