A case with the diagnosis of the onset of inflammatory bowel disease (IBD) with chronic granulo-matous disease (CGD) in Children′s Hospital Affiliated to Zhengzhou University in June 2016 was chosen, and the patient′s clinical data and whole treatment process were analyzed.According to the relevant literature from Chinese and foreign databases, the clinical characteristics were analyzed and summarized, principles of diagnosis and treatment for children who had the onset of IBD with CGD.This patient was a child of 1 year and 9 months old, and the initial symptoms included repeated diarrhea and bloody stools.He was diagnosed as " ulcerative colitis" in the local hospital.After admission, the neutrophil respiratory burst test was positive.The genetic analysis result suggested that the CYBB gene was mutated, thus obtaining the diagnosis of CGD.Then, he was given prophylactic antibiotic therapy and symptomatic treatment.After the 3 months of follow-up after discharge, the patient still had intermittent diarrhea and bloody stools.CGD is a rare primary immunodeficiency disease, and current treatment methods of CGD include hematopoietic stem cell transplantation and anti-infection treatment.When IBD patients have complications other than gastrointestinal symptoms, the IBD treatment is not effective, or there are suspected parents who are married to close relatives, or with the family history of IBD, CGD should be considered.

（正）目前对于可切除食管癌的治疗方式是以手术为主的综合治疗[1]。微创食管癌手术经过多年的发展和普及，其远近期疗效已被广泛认可。目前对于中、下段食管癌，胸腹腔镜联合 Ivor-Lewis 已是主流术式[2]。相对微创 McKeown 手术，全腔镜下Ivor-Lewis 食管癌根治术在近期疗效（特别是在吻合口瘘、吻合口狭窄和喉返神经损伤）方面具有明显优势[3]。本视频旨在展示全腔镜下 Ivor-Lewis 食管癌根治术的手术入路及操作要点。

Objectives: To evaluate the diagnostic value of CT for lymph node metastasis of thoracic esophageal carcinoma with a diameter of more than 1 cm, and to find the optimal diagnostic index by comparing relevant CT indexes. Methods: Totally 80 patients with pathologically proved thoracic esophageal cancer with preoperative CT examination revealed lymph node diameter greater than 1 cm admitted at Department of Thoracic Surgery, the First Affiliated Hospital of University of Science and Technology of China from January 2016 to January 2018 were enrolled in this study. There were 70 males and 10 females, aging of (60±14) years (range: 40-85 years). According to the pathological result of lymph nodes, all the patients and lymph nodes were divided into two groups (N+group: 47 patients, 62 lymph nodes; N-group: 33 patients, 39 lymph nodes). The average number of dissected lymph nodes were 21±4 and 101 lymph nodes′ diameter were greater than 1 cm. The clinicopathologic factors, postoperative complications, lymph node dissection and relevant CT indexes like the minimum diameter of lymph nodes (Min D), the maximum diameter of lymph node (Max D), lymph node axial ratio(LAR), the enhancement of lymph node (ELN) and the boundary of lymph node (BLN) were compared. The clinicopathological data, lymph node dessection and CT parameters of the two groups were compared by t test, χ² test or Wilcoxon rank sum test. Receiver operating characteristic (ROC) curve analysis was used to compare the ability to predict lymph node metastasis between Min D, Max D, LAR, ELN and BLN. Multiple Logistic regression analysis were performed to determine the independent variables for prediction of lymph node metastasis. Results: The difference of tumor segmentation, pN stage, pTNM stage, total number of metastatic lymph nodes, total number of abdominal lymph node metastases, Min D, Max D, ELN and BLN between the two groups were statistically significant. The results of univariate and multivariate analyses showed that gender (OR=0.128, 95%CI: 0.019 to 0.858, P=0.034), pTNM stage (OR=1.514, 95%CI: 1.020 to 2.247, P=0.039), Min D (OR=0.102, 95%CI: 0.010 to 0.995, P=0.050) and LAR (OR=0.195, 95%CI: 0.052 to 0.731, P=0.015) were the independent relative factors. The area under the curve of ROC curve analysis of Min D, Max D, LAR, ELN and BLN were 0.679, 0.666, 0.561, 0.650 and 0.820, respectively. BLN was the best CT index to diagnosis lymph node metastasis, while the accuracy of dignosis of lymph node metastasis of BLN was 97.0%. The Youden index of Min D, Max D and LAR were 1.25 cm, 1.64 cm and 0.77, respectively. Combining the BLN and ELN had a higher diagnostic rate (97.0%) of lymph node metastasis. Conclusions CT has high diagnostic value for lymph node metastasis in thoracic esophagel cancer when the lymph node diameter is greater than 1 cm. BLN is the best diagnostic index for lymph node metastasis.

To investigate the feasibility, safety and short-term efficacy of modified inflatable video-mediastinoscopy in patients with early esophageal cancer. Methods The study retrospectively evaluated 54 patients with cT1N0M0 esophageal carcinoma who received minimally invasive esophagectomy in the First Affiliated Hospital of University of Science and Technology of China between July 2017 and June 2018. Of those patients, 23 patients underwent modified inflatable video-assisted mediastinoscopic transhiatal esophagectomy(MIVMTS) and 31 underwent minimally invasive McKeown esophagectomy (MIME). The clinicopathologic factors, operational factors, postoperative complications and lymph node dissection of patients were compared. Results There was no significant difference in clinicopathological data between the MIVMTS group and MIME group. The incidence of total minor postoperative complications, pulmonary infection of minior postoperative complications, total postoperative complications and total pulmonary complications in MIME group were higher than MIVMTS group. The incidence of recurrent laryngeal nerve injury, arrhythmia and air leaks in minior and pulmonary infection, chylothorax, anastomotic fistula in major postoprative complications were no different in the two groups with P >0. 05. The intraoperative blood loss, duration of surgery and postoperative thoracic drainage fluid volume of MIVMTS group were less than MIME group, the difference was statistically significant. The postoperative hospitalization of the two groups have no statistics significance(P >0. 05). There were no significant difference in the lymph node dessection of the left laryngeal recurrent nerve lymph nodes, paraesophageal lymph nodes, subcarinal lymph nodes and superior phrenic lymph node of the two groups. However, when compared with MIVMTS group, the MIME group have advantage in the right laryngeal recurrent para-nerve lymph node dissection. Conclusion MIVMTS can be safely and effectively performed for early esophageal cancer with favorable short-term efficacy.

Objective: To investigate the feasibility, safety, short-term efficacy and long-term efficacy of elective lymph node dissection in patients with early esophageal cancer. Methods: The study retrospectively evaluated 405 patients with cT1N0M0 esophageal carcinoma who received minimally invasive esophagectomy in the First Affiliated Hospital of University of Science and Technology of China between March 2007 and March 2013. Of those patients, 208 patients underwent systematic lymph node dissection (SLND) and 197 patients underwent elective lymph node dissection (ELND). The clinicopathologic factors, operational factors, postoperative complications, lymph node dissection and prognosis of patients were compared by independent sample t test, χ² test, or Mann-Whitney rank test. The 5-year overall survival was calculated by the Kaplan-Meier estimation method using the Log-rank test. Results: There was no significant difference in clinicopathological data between the SLND group and the ELND group. The incidence of pulmonary infection (8.2% vs. 2.9%, P=0.04) and arrhythmia (6.2% vs. 2.0%, P=0.03) of the minor postoperative complications in the SLND group were higher than the ELND group. The incidence of pulmonary infection (6.2% vs. 2.0%, P=0.03), Chylothorax (5.8% vs.1.5%, P=0.02), anastomotic or pleural hemorrhage requiring reoperation (2.9% vs.0.5%, P=0.04) of major postoperative complications in the SLND group were higher than the ELND group, the difference was statistically significant. In the perioperative data of two groups, the incidence of total postoperative complications, total pulmonary complications, operation time, intraoperative blood loss, postoperative hospitalization, postoperative thoracic drainage duration and postoperative thoracic drainage fluid volume of the SLND group were higher than the ELND group, the difference was statistically significant. The mean numbers and stations of dissected lymph node in the SLND were 30.2±4.2 and 12.1±2.7, the mean numbers and stations of dissected lymph node in the ELND were 25.7±3.8 and 8.4±3.6. The survival rates of 1, 3, 5 years of all patients were 100%, 95.9% and 82.5%, respectively. The median survival time was 87.4 months. Further analysis showed that the 1, 3 and 5 years survival rate of patients with stage Ⅰ esophageal cancer was 100%, 97.1% and 88.9%, respectively. The median survival time was 89.3 months. The 1, 3 and 5 years survival rate of patients with stage Ⅱa esophageal cancer was 100%, 93.2% and 76.8%, respectively. The median survival time was 77.2 months. There was no significant difference in survival rate between the SLND group and the ELND group in 1, 3 and 5 years. When taking a further analysis of stage Ⅰ esophageal cancer, the survival rates between 188 patients in the SLND group and 180 patients in the ELND group were no significant difference. When focus on the stage Ⅱa esophageal cancer, the 1, 3 and 5 years survival rate were higher in the SLND group than that in the ELND group (100%, 94.5%, 83.2% vs. 100%, 91.3%, 72.1%, P=0.047), the difference was statistically significant. Conclusion ELND can be safely and effectively performed for early esophageal cancer with favorable short-term efficacy and long-term efficacy.

Objective To observe the impact of minimally invasive Sweet esophagectomy on short term quality of life for patients with Siewert typeⅡesophagogastric junction adenocarcinoma.Methods From January 2015 to September 2017, 60 patients underwent minimally invasive Sweet esophagectomy(MISE group) and 60 patients underwent open sweet esophagecto-my(OSE group).The questionnaires(EORTC QLQ-C30 and QLQ-OES18) were applied to assess the quality of life of the pa-tients before and 1,2,4,8 weeks after operation.The clinicopathologic factors, operational factors and postoperative complica-tions of the two groups were compared by t test and χ2test.The Mann-Whitney test was used to test for statistical significance because the responses were not normally distributed.Results The two groups were similar in terms of clinical characteristics and preoperative QOL scores(P ＞0.05).The MISE group was associated with a significant decrease in surgical blood loss [(88.1 ±32.2)ml vs.(119.5 ±34.1)ml, t＝5.052, P＝0.001], chest tube duration[(8.1 ±4.4)d vs.(10.5 ±4.0)d, t＝3.110, P＝0.002] and postoperative stay[(9.1 ±4.6)d vs.(11.6 ±3.8)d, t＝3.167, P＝0.002] relative to the OSE group.The postoperative in-hospital mortality and total morbidity did not differ between the two groups (P＞0.05).The MISE group was associated with significantly fewer respiratory complications than the OSE group (8.5%vs.22.7%, t＝4.063, P＝ 0.044).The MISE group was associated with a significant increase in hospitalization costs [(54 106 ±4 352) yuan vs. (51 143 ±5 315)yuan, P＝0.001] relative to the OSE group.MISE group gained higher scores in physical function, role func-tion, emotional function, Global QOL and lower scores in pain, fatigue, acid reflux than OSE group after surgery.Conclusion Our minimally invasive technique for Siewert typeⅡesophagogastric junction adenocarcinoma can be safely and effectively performed with favorable early outcomes.Compared to conventional open Sweet approach, MISE impoved the short term quality of life after surgery.

OBJECTIVETo discuss the safety and feasibility of gastric tube in construction technique in total thoracoscopic and laparoscopic Ivor-Lewis esophagectomy.

METHODSClinical data of 358 patients with esophageal cancer who underwent the Ivor-Lewis procedure by the same surgical team in our department from June 2015 to June 2016 were retrospectively analyzed. Patients were divided into two groups: group MI (mini-incision): 92 patients undergoing extracorporeally gastric tube through a 4-cm abdominal mini-incision after gastric mobilization; group TL (total laparoscopy): 266 patients undergoing gastric tube made by total laparoscopy and jejunostomy. Intra-operative and postoperative data were compared between two groups.

RESULTSOf 358 patients, 290 were male and 68 were female. The average age was (62.3±15.6) years. The tumor location ratio of group TL and group MI was 27.1%(72/266) and 25.0%(23/92) in middle thoracic esophageal cancer, 35.3%(94/266) and 34.8%(32/92) in mid-lower thoracic esophageal cancer, and 37.6%(100/266) and 40.2%(37/92) in lower thoracic esophageal cancer, respectively. In group TL and group MI, 42.1% (112/266) and 46.7%(43/92) patients were stage I( to II(a; 57.9%(154/266) and 53.3%(49/92) patients were stage II(b to III(a, respectively. All the patients from two groups received successful Ivor-Lewis esophagectomy. There were no significant differences between group TL and MI in operative time [abdominal operative time: (65.6±25.8) min vs. (62.3±25.6) min; thoracic operative time: (180.3±37.4) min vs. (178.1±39.2) min; time of making gastric tube:(16.1±3.2) min vs.(15.7±3.5) min], blood loss [abdominal: (60.5±19.8) ml vs. (62.3±20.9) ml; thoracic: (228.7±47.3) ml vs. (231.6±46.8) ml], and the number of lymph nodes dissection (abdominal: 8.9±1.8 vs. 8.7±1.6; mediastinal: 21.2±3.6 vs. 20.8±3.8)(all P>0.05). And there were no significant differences in occurrence of postoperative anastomotic leakage [4.5%(12/266) vs. 4.3%(4/92), χ=0.845, P=0.948], postoperative hospital stay [(12.2±4.8) d vs. (13.1±5.1) d, t=1.525, P=0.128] and average hospitalization cost(5.5±2.1 vs. 5.3±1.2, t=0.865, P=0.172) (ten thousand yuan, RMB) between group TL and MI.

CONCLUSIONDuring minimally invasive Ivor-Lewis esophagectomy, gastric tube by total laparoscopy is safe and reliable.

Objective To investigate the feasibility and clinical effect of laparoscopic and thoracoscopic Ivor Lewis esophagectomy without an abdominal small incision.Methods Compared 80 cases underwent laparoscopic and thoracoscopic Ivor Lewis esophagectomy without an abdominal small incision with 68 patients receivesd laparoscopic and thoracoscopic Ivor Lewis esophagectomy with an abdominal small incision.The peri operative conditions and complications of the two groups were analyzed.Results There were no significant difference in the operation time [(263.3 ± 71.5) min vs (273.3 ± 73.7) min,t =-0.750,P =0.454],intraoperative blood loss [(246.9 ± 150.4) ml vs (252.9 ± 159.7) ml,t =-0.238,P =0.812],the number of lymph node dissection [(19.2 ±4.3) vs (19 ±4.5),t =0.272,P =0.786],gastrointestinal decompression time [(11 ± 3.4) d vs (11.9±3.3) d,t=-1.647,P=0.102],chest tube indwelling time [(6.6±2.7) d vs (6.3±2.6) d,t=0.544,P=0.587],postoperative hospitalization time [(13.2 ±3.4) d vs (14 ±3.4) d,t=-1.493,P=0.138] and rate of early gastric emptying dysfunction [6.25％ (5/80) vs 4.41％ (3/68),x2 =0.016,P =0.898].Comparing to patients in the small incision group,the visual analogue scale evaluation score of postoperative pain was lower in the groups without small incision (P ＜ 0.05).There were no anastomotic fistula,thoracic gastric fistula,upper gastrointestinal bleeding and death during perioperative periods.Conclusion It is safe and feasible to treat middle and lower esophageal carcinoma with laparoscopic and thoracoscopic Ivor Lewis esophagectomy without an abdominal small incision,which can further reduce abdominal trauma,relieve postoperative pain and make the abdominal incision more beautiful.

Objective To investigate the changes in Th17 cells and CD4+CD25+regulatory T lym-phocytes ( Treg) as well as transcription factors and cytokines relating to them in children with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) and to analyze their role and clinical significance. Methods Thirty-two children with newly diagnosed EBV-associated HLH in the Hematology/Oncology Department of Zhengzhou Children′s Hospital from January 2012 to December 2016 were enrolled in this study. Thirty healthy children taking physical examination in the same hospital in the corresponding period were recruited as controls. Percentages of Th17 and Treg cells in peripheral blood T lymphocytes were detected by flow cytometry. Expression of RORγt and Foxp3 at mRNA level in peripheral blood mononuclear cells was detected by real-time PCR. Levels of IL-6, IL-17, IL-10 and TGF-β1 in serum samples were measured by ELISA. Results Compared with the control group, the EBV-associated HLH group showed in-creased percentage of Th17 cells [(1. 09±0. 43)％ vs (0. 39±0. 19)％, P<0. 05] and enhanced expres-sion of RORγt at mRNA level [(1. 41±0. 37) vs (0. 67±0. 13), P<0. 05], but decreased percentage of Treg cells [(3. 66±1. 13)％ vs (6. 80±1. 15)％, P<0. 05] and inhibited expression of Foxp3 at mRNA level [(15. 97±5. 11) vs (30. 23±4. 95), P<0. 05]. All of the above mentioned changes were reversed af-ter treatment (P<0. 05). Serum levels of IL-6 and IL-17 of EBV-associated HLH group were higher than those of control group, while serum levels of IL-10 and TGF-β1 were lower (P<0. 05). Conclusion Im-balanced Th17/Treg cells might play an important role in the pathogenesis of EBV-associated HLH. Cyto-kines relating to the maintenance of Th17/Treg cell balance could be used as indicators of disease develop-ment.

Objective To describe the technique for minimally invasive Sweet esophagectomy and to evaluate the feasibility,safety and the short-term clinical outcomes of this approach in the treatment of Siewert type Ⅱ esophagogastric junction adenocarcinoma.Methods The clinical data of 122 patients with Siewert type Ⅱ csophagogastric junction adenocarcinoma who received Sweet esophagectomy between October 2013 and June 2015 in Department of Thoracic Surgery,Auhui Provincial Hospital Affiliated with Anhui Medical University was analyzed retrospectively.The study group consisted of 87 men and 35 women,and the ages ranged from 48 to 78 years (median:67 years).Of those 122 patients,47 underwent minimally invasive approach and 75 underwent open left transthoracic sweet esophagectomy.This study included16 stage Ⅰa patients,35 stage Ⅰb patients,32 stage Ⅱ a patients,28 stage Ⅲ b patients,and 11 stage]Ⅲa patients.The clinicopathologic factors,operational factors and postoperative complications of the two groups were compared by t test and X2 test.Results The two groups were similar in terms of gender,age,American Society of Anesthesiologists grade,preoperative staging and incidence of comorbidities (P ＞0.05).The minimally invasive approach was associated with significant increase in the number of total lymph nodes dissected or the stations of the total lymph nodes dissected (1 8.1 ± 2.7 vs.15.0 ± 2.5,t =6.612,P =0.001;8.9 ± 1.1 vs.6.7 ± 1.2,t =9.960,P =0.003),significant decrease in surgical blood loss ((88 ±32) ml vs.(120 ±34) ml,t =5.052,P =0.001),chest tube duration ((8 ±4) d vs.(10 ±4) d,t=3.110,P=0.002) and postoperative stay ((9 ±5) d vs.(12 ±4) d,t=3.167,P=0.002)relative to the open approach.The postoperative in-hospital mortality and total morbidity did not differ between the two groups (P ＞ 0.05).The minimally invasive approach was associated with significantly fewer respiratory complications than the open approach (8.5％ vs.22.7％,X2 =4.063,P =0.044).Conclusion Minimally invasive technique for Siewert type Ⅱ esophagogastric junction adenocarcinoma can be safely and effectively performed for intrathoracic anastomosis with favorable early outcomes.