Objective To summarize our experience and the early and midterm outcomes of stented elephant trunk procedure for right-sided aortic arch (RAA) with Kommerell's diverticulum (KD). Methods From April 2013 to July 2020, patients with RAA and KD who underwent stented elephant trunk procedure at our center were collected. Surgery was performed under moderate hypothermic circulatory arrest combined with selective antegrade cerebral perfusion via median sternotomy. Results A total of 8 patients were included, including 7 males and 1 female with a mean age of 51.88±9.61 years. All patients had an aneurysmal KD and aberrant left subclavian artery. Preoperative comorbidities included acute Stanford type B aortic dissection in 1 patient, aortic arch pseudoaneurysm in 1 patient, acute type B intramural hematoma in 2 patients, and coronary artery disease in 1 patient. Concomitant procedures included reconstruction of the left subclavian artery in all patients and coronary artery bypass grafting in 1 patient. The mean time of operation, cardiopulmonary bypass, aortic cross-clamping, and selective cerebral perfusion was 6.25±1.16 h, 157.75±40.07 min, 77.75±33.10 min, and 28.50±5.55 min, respectively. No intraoperative death occurred. There was 1 in-hospital death. Follow-up was completed in all patients with a mean period of 3.58±2.08 years. No late death occurred. A persistent anastomotic leak of the proximal arch was detected in 1 patient, but reintervention was not performed because neither aortic dilatation nor symptoms of tracheal and esophageal compression were observed during the follow-up. The remaining 6 patients showed positive aortic remodeling with complete thrombosis of the aneurysmal KD, and neither aortic event nor tracheal and esophageal compression occurred. Conclusion Stented elephant trunk procedure is a safe and feasible technique for selected patients with RAA and KD, which can achieve favorable early and midterm outcomes.

Objective To investigate the changes in interleukin-34 (IL-34)levels in serum and bronchoalveolar lavage fluid (BALF) of patients with severe pneumonia and their prognostic value. Methods A total of 66 patients with severe pneumonia (severe pneumonia group), 35 patients with non-severe pneumonia (non-severe pneumonia group), and 27 healthy adults (control group) were enrolled. The severe pneumonia group was further divided into survival group of 38 patients and non-survival group of 28 patients based on 28-day survival. Clinical data of all subjects were analyzed. Receiver operating characteristic (ROC) curves were plotted to assess the predictive power of serum IL-34 and relative IL-34 gene expression in BALF for 28-day mortality in patients with severe pneumonia, as well as the predictive power of serum IL-34 for severe pneumonia. Kaplan-Meier survival curves were plotted, and the Log-rank test was used to compare cumulative survival rates. Cox regression analysis was conducted to explore risk factors for 28-day mortality in patients with severe pneumonia. Pearson correlation analysis was used to assess the correlation between serum IL-34 levels and relative IL-34 gene expression in BALF of patients with severe pneumonia. Results Serum IL-34 levels were higher in the severe pneumonia group than those in the non-severe pneumonia group, and were higher in the non-severe pneumonia group than in the control group (P < 0.05). Serum IL-34 levels and relative IL-34 gene expression in BALF were higher in the non-survival group than in the survival group (P < 0.05). ROC curve analysis showed that the areas under the curve for predicting 28-day mortality in patients with severe pneumonia were 0.908 for serum IL-34 levels and 0.878 for relative IL-34 gene expression in BALF. The optimal cutoff value for serum IL-34 levels in predicting severe pneumonia was 129.9 pg/mL. Multivariate Cox regression analysis showed that increased serum IL-34 levels and increased relative IL-34 gene expression in BALF were independent risk factors for 28-day mortality in patients with severe pneumonia (P < 0.05). The Kaplan-Meier survival analysis results indicate that, with a cutoff value of 129.9 pg/mL, patients with severe pneumonia who had high serum levels of IL-34 exhibited a lower cumulative survival rate compared to those with low IL-34 level(Log-rank P < 0.001). Conclusion Serum IL-34 levels are significantly increased in patients with severe pneumonia, and relative IL-34 gene expression in BALF is positively correlated with serum IL-34 levels. Both can be used as indicators for predicting the prognosis of 28-day mortality in patients with severe pneumonia.

Objective:To summarize our experience and outcomes of surgical repair of type Ⅱ right-sided aortic arch(RAA) with Kommerell's diverticulum(KD).Methods:From May 2010 to August 2020, a total of 13 patients with type Ⅱ RAA and KD underwent surgery at our center. Mean age was(50.46±10.31) years, 10 were male, and 3 were female. All patients had an aneurysmal KD and aberrant left subclavian artery(ALSA). Preoperative comorbidities included type B aortic dissection in 1 case, aortic arch pseudoaneurysm in 2 cases, and type B intramural hematoma in 2 cases, respectively. Eight(61.5%) patients underwent stented elephant trunk procedures under moderate hypothermic circulatory arrest combined with selective antegrade cerebral perfusion via median sternotomy, and all of them had ALSA reconstruction. Five(38.5%) patients underwent distal arch and descending thoracic aortic replacement through a right posterolateral thoracotomy, the ALSA was reconstructed or ligated in 1 each, and ALSA embolization was performed before surgery in the other 3 cases.Results:No operation deaths occurred. Recurrent laryngeal nerve injury occurred in 2 cases. There was 1(7.69%) in-hospital death. Follow-up was complete in 100 % at mean(5.28±3.84) years. No late death occurred. A persistent anastomotic leak of the proximal arch was detected in a patient who underwent stented elephant trunk procedure, but no aortic dilatation or tracheal and esophageal compression was observed during follow-up. Meanwhile, aortic events, limb ischemia, or symptoms of tracheal and esophageal compression were not observed in the remaining 11 patients.Conclusion:Surgical repair of type Ⅱ RAA with KD can achieve favorable early and midterm outcomes. Surgical strategies should be chosen based on the anatomy of the aorta and whether it is combined with compression symptoms.

Objective:To analyse the long-term efficacy in childhood T-cell acute lymphoblastic leukemia (T-ALL) cases enrolled in the national protocol of childhood leukemia in China-acute lymphoblastic leukemia (NPCLC-ALL) 2008.Methods:Clinical data of 96 patients diagnosed as T-ALL and treated with NPCLC-ALL2008 protocol between January 2009 and December 2017 in the Department of Hematology-Oncology, the Children′s Hospital, Zhejiang University School of Medicine were analyzed retrospectively. Predictive value of minimal residual disease (MRD) monitored by flow cytometry was analyzed. Kaplan-Meier method was used for long-term survival analysis.Results:A total of 96 evaluable patients with newly diagnosed T-ALL were analysed, including 72 males and 24 females. The age was 9.5 (ranged from 1.0 to 16.0) years. The follow-up time was 5.7 (ranged from 1.0 to 9.7) years. Among 96 patients, 92 (96%) achieved complete remission. The 5-year event free survival (EFS) and overall survival (OS) rates were (61±6) % and (70±5) %, respectively. Relapse occurred in 18 cases and the 5-year cumulative incidence of relapse was (27±6) %. Twenty-four patients died. The 5-year OS rates of patients with MRD>5% on day 15 of induction therapy was significantly worse than those with MRD≤5% ((60±12) % vs. (72±6) %, χ 2=3.904, P=0.048) . The 5-year EFS and OS rates were obviously lower in patients with MRD>10% before the consolidation therapy ((50±35) %). The 5-year OS rates of patients with relapsed disease was significantly worse than those without ((26±13) % vs. (81±5) %, χ 2=18.411, P<0.01). The earlier the relapse, the worse the prognosis. The 5-year OS rates for patients relapsed within 6 months, within 3 years and more than 3 years, were (25±22) %, (30±14) % and (50±35) % respectively (χ 2=13.207, P<0.01). Conclusions:NPCLC-ALL2008 protocol is effective for childhood T-ALL. The MRD guided accurate risk stratification and individualized treatment can reduce the relapse and improve the survival rate of pediatric T-ALL.

Objective To explore the value of cytokines and procalcitonin (PCT) in predicting the potential pathogen and the severity of infection in children with hematological malignancies.Methods This was a prospective cohort study.A total of 1 543 children (3 912 episodes) with hematological malignancies admitted to the Department of Hematology/Oncology in Children's Hospital of Zhejiang University School of Medicine from November 2010 to June 2018 were prospectively enrolled in this study.According to the pathogen,the patients were divided into sepsis group with positive blood culture (380 patients/608 episodes),sepsis group with negative blood culture (539/1 484),non-septic infection group (549/1 709),viral infection group (18/21) and fungal infection group (57/90).The patients were also divided into two groups in either shock group (171/187) or non-shock group (1372/3725).Meanwhile,89 children (112 episodes) with matched hematological malignancies without fever were enrolled as the control group.The levels of PCT and cytokines were measured for detecting the potential relationship between these markers and infection severity.Mann-Whitney U test or Kruskal-Wallis H test was used to compare the differences between groups.The Receiver Operator Characteristic curve analysis was used to explore the predictive value.Results (1) There were significantly higher levels of interleukin (IL) 6 (H=210.002,P＜0.01),IL-10 (H=223.045,P＜0.01),and PCT (H=76.799,P＜0.01) in the infection group,with area under curre (AUC) of 0.67 (95％CI 0.64-0.69),0.69 (95％CI 0.66-0.71) and 0.59 (95％CI 0.56-0.61),respectively.(2) Among the patients in the shock group,IL-6 (Z=11.460,P＜0.01),IL-10 (Z=14.242,P＜0.01) and PCT (Z=10.813,P＜0.01) were significantly higher than those in the non-shock group,with the AUC of 0.75 (95％CI 0.70-0.79),0.81 (95％CI 0.77-0.84) and 0.73 (95％CI 0.69-0.77),respectively.(3) In patients with septic shock,the incidence of Gram negative bacteria (GNB) infection (60 episodes) was significantly higher than Gram positive bacteria (GPB) infection (22 episodes) (x2=29.665,P＜0.01).The AUC of IL-6,IL-10 and PCT for predicting GNB infection were 0.75 (95％CI 0.72-0.78),0.82 (95％CI 0.79-0.85) and 0.67 (95％CI 0.63-0.70),respectively.(4) The predictive power of IL-6,IL-10 and PCT combination (AUC=0.994) was superior to IL-10 alone (AUC=0.810) in predicting septic shock (Z=10.211,P＜0.01).Conclusions IL-6and IL-10 are better predictors of GNB infection than PCT in hematology-oncology children.Furthermore,the combination of these two cytokines and PCT is more valuable in predicting the severity of infection.

Objective To observe the influencing of Yiqi Huayu Jiedu Prescription on the growth of HepG2 nude mice transplantation tumor and the expression of related factors of vascular mimicry. Methods Models of transplanted tumors, which were made by HepG2 cells in nude mice, were randomly divided into 7 groups, Yiqi Huayu Jiedu Prescription group, Astragali Radix group, Curcumae Rhizoma group, Paridis Rhizoma group, Gecko group, cis-platinum group, and model group. Except for the model group, the rest groups were given relevant medicine for intervention. 21 days later. HIF-1α, MMP-2, MMP-9, and E-cad were detected by immunohistochemistry, and Twist1 and Bcl-2 were detected by fluorescence quantitative PCR. Results Compared with the model group, tumor volume in the rest groups decreased (P<0.05), and the effect in the Yiqi Huayu Jiedu Prescription group was more obvious than the Astragali Radix group, Paridis Rhizoma group and Gecko group (P<0.05);The expression of vasculogenic mimicry structure was rare in each group, and the model group and cis-platinum group were the most obvious;Except for the Astragali Radix group, the expressions of HIF-1α, MMP-2, and MMP-9 showed statistical significance compared with model group (P<0.05);The expression of E-cad in the Yiqi Huayu Jiedu Prescription group and Astragali Radix group showed statistical significance (P<0.05);The expression of Bcl-2 in the Yiqi Huayu Jiedu prescription group, Paridis Rhizoma group, and Gecko group decreased significantly compared with the model group (P<0.05);The expression of Bcl-2 in the Yiqi Huayu Jiedu prescription group was much better than the other groups (P<0.05);The expression of Twist1 showed statistical significance in the Yiqi Huayu Jiedu Prescription group, Curcumae Rhizoma group, Paridis Rhizoma group, and cis-platinum group (P<0.05). Conclusion Yiqi Huayu Jiedu Prescription can reduce expression of HIF-1α, Twist1, Bcl-2, MMP-2, and MMP-9, and increase expression of E-cad, thereby inhibiting the formation of vascular mimicry.

DNA Mutational Analysis ; Diagnosis, Differential ; Humans ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; genetics ; therapy ; Mutation ; genetics ; Perforin ; Pore Forming Cytotoxic Proteins ; genetics ; Qa-SNARE Proteins ; genetics

Adolescent ; Antibodies, Monoclonal ; therapeutic use ; Antigens, Neoplasm ; immunology ; Cancer Vaccines ; immunology ; Child ; Child, Preschool ; Humans ; Immunotherapy ; methods ; Infant ; Killer Cells, Natural ; immunology ; Lymphocyte Transfusion ; Neoplasms ; immunology ; therapy ; Pediatrics ; Stem Cell Transplantation