Objective:To investigate the related risk factors of surgical appendectomy after endoscopic retrograde appendicitis therapy (ERAT) in children.Methods:From September 2019 to November 2023 at the Children's Hospital Affiliated to Shandong University,the data from all related children with appendectomy after ERAT were analyzed. The general situation and main clinical manifestations of the children were collected. According to the effect of ERAT,the patients were divided into transfer to surgical group and appendicitis recurrence group by the reasons and time of transfer to surgical treatment. The indexes such as ERAT and reoperation process and pathological types of appendix after operation were collected,and the clinical experience was summarized.Results:Among the 242 children who underwent ERAT,32 cases underwent appendectomy again,including 19 males and 13 females,with an average age of（9.16±2.77）years,and the reoperation rate was 13.2%. The clinical manifestation was abdominal pain (32 cases,100.0%),mainly right lower abdominal pain (28 cases,87.5%),and the main duration was less than 3 months (30 cases,93.8%). Abdominal ultrasound or CT before ERAT mainly indicated appendicitis (11 cases,34.4%) and appendicitis complicated with appendiceal calculus (11 cases,34.4%). There were 9 children in transferred to surgical group,of which 5 cases were transferred to surgery because of ERAT intubation failure,and 4 cases were transferred to surgery because of appendix perforation or abscess. Postoperative pathology showed acute suppurative appendicitis in 5 cases and acute gangrenous appendicitis in 4 cases. There were 23 children in appendicitis recurrence group,whose abdominal pain was relieved after ERAT,but their symptoms were repeated after discharge. After clinical evaluation,they were diagnosed as recurrent appendicitis and underwent surgical appendectomy. Most of them were reoperated within 6 months after ERAT (21 cases,91.3%).Postoperative pathology was mainly chronic appendicitis (10 cases,43.5%).Conclusion:Acute suppurative appendicitis and acute gangrenous appendicitis are prone to catheterization or endoscopic failure during ERAT,and there is a high risk of surgical transfer. ERAT may relieve the symptoms of chronic appendicitis in children. If the symptoms are repeated,surgery is still needed to remove the appendix.

Objective:To investigate the specimen quality of intersphincteric resection with transabdominal transanal mixed approach for rectal cancer in the Chinese Transanal Total Mesorectal Excision Registry Collaborative (CTRC) database.Methods:The retrospective case-control study was conducted. Based on the concept of real-world research, the clinicopathological data of 281 pati-ents with rectal cancer in the CTRC database who underwent intersphincteric resection with trans-abdominal transanal mixed approach in 19 medical centers, including the Beijing Friendship Hospital of Capital Medical University et al, from November 15,2017 to December 31,2023 were collected. There were 196 males and 85 females, aged 61(range, 27-87)years. Observation indicators: (1) preoperative examinations; (2) neoadjuvant therapy; (3) postoperative examinations; (4) analysis of influencing factors for positive circumferential margin in surgical specimen of intersphincteric resec-tion for rectal cancer. Measurement data with normal distribution were represented as Mean±SD. Measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers or percentages. The chi-square test was used for univariate analysis. Logistic regression model was used for multivariate analysis. Results:(1) Preoperative examinations. Of the 281 patients, 234 cases underwent preoperative pelvic magnetic resonance imaging (MRI) examina-tion. There were 2 cases in clinical stage T0, 3 cases in clinical stage T1, 58 cases in clinical stage T2, 137 cases in clinical stage T3, 24 cases in clinical stage T4, 3 cases in clinical stage Tx, 7 cases missing clinical T staging data. There were 87 cases in clinical stage N0, 68 cases in clinical stage N1, 60 cases in clinical stage N2, 9 cases in clinical stage Nx, 10 cases missing clinical N staging data. There were 30 cases with mesorectal fascia invasion, 53 cases with extramural venous invasion. The distance from lower margin of tumor to anal margin was 41.9(range, 1.0-80.0)mm. (2) Neoadjuvant therapy. Of the 281 patients, 125 cases underwent neoadjuvant therapy, including 39 cases receiving chemo-therapy alone, 6 cases receiving short-course simultaneous chemoradiotherapy, 5 cases receiving short-course simultaneous chemoradiotherapy and delayed surgery, 48 cases receiving long-course simultaneous chemoradiotherapy, 2 cases receiving other treatments, and 25 cases missing neoadju-vant therapy data. (3) Postoperative examinations. Of the 281 patients, 249 cases achieved R 0 resection, 9 cases achieved R 1 resection, and there were 23 cases missing surgical margin data. The maximum tumor diameter, the number of lymph nodes harvested and positive rate of vessel carcinoma embolus were 30.0(range, 0.5-200.0)mm, 13(range, 0-70) and 27.55%(73/265) in 281 patients. There were 252 patients with circumferential margin records, showing positive in 15 cases, with a positive rate as 5.95%(15/252). The minimum distance from deep part of tumor to circumferential margin was 7.0(range, 0-150.0)mm in 252 patients. There were 85 cases with distal margin records, showing positive in 1 case, and the distance from lower margin of tumor to distal margin was 10.0(range, 0-202.0)mm. There were 273 patients with specimen integrity records, which showed intact specimen in 208 cases, fair specimen in 58 cases, poor specimen in 4 cases, unevaluated specimen in 3 cases. There were 7 cases with rectal perforation. Of the 281 patients, cases in pathological stage T0, Tis, T1, T2, T3, T4 were 14, 5, 22, 107, 113, 12, respectively, and there were 8 cases missing pathological T staging data. Of the 281 patients, cases in pathological stage N0, N1a, N1b, N1c, N2a, N2b were 176, 27, 27, 11,20, 12, respectively, and there were 8 cases missing pathological N staging data. Of the 281 patients, there were 4 cases with distant metastasis, 262 cases without distant metastasis, 5 cases not evaluated, and 10 cases missing tumor metastasis data. Of the 125 patients undergoing neoadjuvant therapy, there were 85 cases with tumor regression grade records, including 16 cases as grade 1, 27 cases as grade 2, 19 cases as grade 3, 15 cases as grade 4, 8 cases as grade 5. (4) Analysis of influencing factors for positive circumferential margin in surgical specimen of intersphincteric resection for rectal cancer. Results of univariate analysis showed that preoperative T staging on preoperative pelvic MRI, mesorectal fascia invasion, extramural venous invasion, pathological T staging, and pathological N staging were related factors for positive circumferential margin in surgical specimen of intersphincteric resection for rectal cancer ( P<0.05). Conclusions:Intersph-incteric resection with transabdominal transanal mixed approach has good specimen quality and low positive rate of surgical margin. T staging on preoperative pelvic MRI may be related to positive circumferential margin after intersphincteric resection for rectal cancer.

Objective:To analyze the quality of surgical specimens of rectal cancer in the Chinese transanal total mesorectal excision (taTME) registry collaborative (CTRC) database.Methods:The retrospective and descriptive study was conducted. Based on the concept of real-world research, the clinicopathological data of 1 761 patients with rectal cancer in the CTRC database who underwent taTME in 40 medical centers, including the Beijing Friendship Hospital of Capital Medical University et al, from November 15, 2017 to December 31, 2022 were collected. There were 1 212 males and 549 females, aged 62(range, 53-68)years. Observation indicators: (1) preoperative examinations; (2) neoadjuvant therapy; (3) postoperative examinations. Measurement data with skewed distri-bution were represented as M(range). Count data were described as absolute numbers. Results:(1) Preoperative examinations. Of the 1 761 patients, 1 324 patients underwent preoperative pelvic magnetic resonance imaging examination, and the results showed that 4 cases as clinical T0 stage, 30 cases as clinical T1 stage, 250 cases as clinical T2 stage, 828 cases as clinical T3 stage, 141 cases as clinical T4 stage, 11 cases as clinical Tx stage, 60 cases missing clinical T staging data, 490 cases as clinical N0 stage, 373 cases as clinical N1 stage, 311 cases as clinical N2 stage, 86 cases as clinical Nx stage, 64 cases missing clinical N staging data, 156 cases with mesorectal fascia invasion, 223 cases with extraintestinal blood vessels invasion. The distance from lower margin of tumor to anal margin of 1 324 patients was 50(range, 40-60)mm. (2) Neoadjuvant therapy. Of the 1 761 patients, 873 patients underwent neoadjuvant therapy, including 17 cases receiving radiotherapy alone, 155 cases receiving chemotherapy alone, 43 cases receiving short-course simultaneous chemoradiotherapy, 26 cases receiving short-course simultaneous chemoradiotherapy and delayed surgery, 1 case receiving contact radiotherapy, 277 cases receiving long-course simultaneous chemoradiotherapy, 9 cases receiving other treatments, and 345 cases missing neoadjuvant therapy data. (3) Postoperative examinations. Of the 1 761 patients, 1 584 cases achieved R 0 resection, 23 cases achieved R 1 resection, 1 case achieved R 2 resection, and there were 153 cases missing surgical margin data. The tumor diameter, number of lymph nodes harvest and positive rate of intravascular tumor thrombus were 30(range, 20-45)cm, 13(range, 10-17) and 20.794%(330/1 587) in 1 761 patients. There were 1 647 patients with circumferential margin records, which showed positive in 51 cases, and the minimum distance from deep part of tumor to circumferential margin was 5(rang, 3-13)mm in 1 647 patients. There were 547 cases with distal margin records, which showed positive in 4 cases, and the distance from lower margin of tumor to distal margin was 20(10-25)mm in 547 cases. There were 1 698 patients with specimen integrity records, which showed intact specimen in 1 436 cases, fair specimen in 233 cases, poor specimen in 8 cases, unevaluated specimen in 21 cases, and there were 20 cases with rectal tube perforation. Of the 1 761 patients, cases as pathological T0 stage, Tis stage, T1 stage, T2 stage, T3 stage, T4 stage was 103, 23, 145, 515, 712, 179, respectively, and there were 4 cases of pathology that could not be evaluated and 80 cases missing pathological T staging data. Of the 1 761 patients, cases as pathological N0 stage, N1a stage, N1b stage, N1c stage, N2a stage, N2b stage was 1 117, 189, 133, 66, 109, 68, respectively, and there were 79 cases missing pathological N staging data. Of the 1 761 patients, there were 79 cases with distant metastasis, 1 591 cases without distant metastasis, and 91 cases without data of tumor metastasis. Of the 873 patients undergoing neoadjuvant therapy, there were 405 patients with tumor regression grade records including 105 cases as grade 1, 142 cases as grade 2, 91 cases as grade 3, 43 cases as grade 4, 24 cases as grade 5. Conclusions:In China, the quality of surgical specimens of taTME for rectal cancer is good with low positive rate of resection margin. It is recommended that using a formatted postoperative pathological report for good quality control of pathological report of surgical specimen.

Abdomen/diagnostic imaging* ; Artificial Intelligence ; Body Composition ; Magnetic Resonance Imaging ; Practice Guidelines as Topic

Objective:To investigate the learning curve of laparoscopic transanal total mesorectal excision (taTME) for rectal cancer operated by one or two surgery teams.Methods:The retrospective cross-sectional study was conducted. Based on the concept of real-world research, the clinical data of 1 458 patients undergoing laparoscopic rectal cancer taTME from 44 medical centers who were registered in the Chinese taTME registry collaborative (CTRC) database from May 2010 to May 2020 were collected. The 1 458 patients were divided into cohorts with one surgery team or two surgery teams according to the operation method. Patients with one surgery team underwent taTME by transabdominal operation and then by transanal operation. Patients with two surgery teams underwent taTME by transabdominal and transanal operation simultaneously with duration of the simutaneous operation time ≥30 minutes. The entire surgical process of patients with two surgery teams is not required to be performed by two surgery teams simutaneously. The clinical data were collected from the medical centers with similar operation amount according to the operation time sequence to analyze the difference between different operation stages and explore the learning curve. The operation time was taken as the parameter to carry out cumulative sum analysis and draw the learning curve of laparoscopic rectal cancer taTME in each medical center. The clinicopathological characteristics of patients from two medical centers with the largest difference in learning curves were analyzed. Observation indicators: (1) screening results of clinical data; (2) clinical data collection of patients with one surgery team; (3) surgical situations of laparoscopic rectal cancer taTME from the one surgery team in different operation stages; (4) learning curve of the one surgery team; (5) clinical data collection of patients with two surgery teams; (6) surgical situations of laparoscopic rectal cancer taTME from the two surgery teams; (7) learning curve of the two surgery teams. The cumulative sum was calculated by the CUSUM=∑i=1nXi-U, where Xi represented the operation time of each taTME, U represented the average operation time of all cases, and n represented the operation number. Fitting process was conducted on scatter plot of learning curves. Taking the apex of learning curve as the boundary, the learning curve was divided into two stages. The abscissa corresponding to the apex of learning curve was the number of operations that needed to be performed to cross the learning curve. Measurement data with normal distribution were represented as Mean±SD. Comparison between two groups was conducted using the t test and comparison between multiple groups was conducted using the ANOVA. Measurement data with skewed distribution were represented as M( P25,P75), and comparison between groups was conducted using the Mann-Whitney U test. Comparison of ordinal data was analyzed using the rank sum test. Count data were analyzed using the chi-square test or Fisher exact probability. Results:(1) Screening results of clinical data:the clinical data of 661 patients from 7 medical centers with one surgery team and two surgery teams were collected. (2) Clinical data collection of patients with one surgery team: the clinical data of 312 patients undergoing laparoscopic rectal cancer taTME from 5 medical centers were collected including 42 cases in the number 2 medical center, 97 cases in the number 20 medical center, 82 cases in the number 33 medical center, 35 cases in the number 37 medical center and 56 cases in the number 39 medical center, respectively. (3) Surgical situations of laparoscopic rectal cancer taTME from the one surgery team in different operation stages: three medical centers including the number 2, number 37 and number 39 medical center with close operation volume provided the clinical data of cases distributed in five operation stages. Among the five operation stages, the proportion of high-quality operation of total mesorectal excision (TME) was ≥17/18, the incidence of postoperative complications was ≤13.3%(4/30) and the incidence of anastomotic leakage was ≤10.0%(3/30). There was no significant difference in the TME quality, postoperative complications or anastomotic leakage among the five operation stages ( P>0.05). There was no significant difference in the operation time among the five operation stages ( χ2=6.950, P>0.05). (4) Learning curve of the one surgery team: the number of operations corresponding to the turning point of learning curve in number 2 and number 20 medical center was 22 and 39, respectively. The number of operations corresponding to the turning points of learning curve in number 33 and number 37 medical center was 15, 66 and 10, 28, respectively. The number of operations corresponding to the turning point of learning curve in number 39 medical center was 20. The overall curve of number 20 medical center was in line with the trend of learning curve and 39 cases of operations was the minimum number needed to cross the learning curve. The biggest difference in learning curve was shown between the number 20 and number 33 medical center. Cases with the gender of male or female, age, body mass index, cases classified as stage 1, stage 2, stage 3 or stage 4 of the American Society of Anesthesiologists (ASA) Classification, cases with neoadjuvant therapy, duration of postoperative hospital stay of the number 20 medical center were 77, 20, (60±10)years, 24 kg/m 2(22 kg/m 2, 26 kg/m 2), 1, 88, 8, 0, 8, 8, 11 days (9 days, 13 days), respectively, versus 51, 31, (64±11)years, 23 kg/m 2(21 kg/m 2, 26 kg/m 2), 0, 35, 43, 1, 31, 16 days (13 day, 21 day) of number 33 medical center, showing significant differences in the above indicators between the two medical centers ( χ2 =6.442, t=-2.265, Z=-2.032, -6.870, χ2 =22.120, Z=-8.408, P<0.05). (5) Clinical data collection of the two surgery teams: the clinical data of 259 patients undergoing laparoscopic rectal cancer taTME from 5 medical centers were collected, including 46 cases in the number 2 medical center, 47 cases in the number 8 medical center, 78 cases in the number 18 medical center, 43 cases in the number 33 medical center and 45 cases in the number 44 medical center, respectively. (6) Surgical situations of laparoscopic rectal cancer taTME from the two surgery teams: four medical centers including the number 2, number 8, number 33 and number 44 medical center with close operation volume provided the clinical data of cases distributed in four operation stages. Among the four operation stages, the proportion of high-quality operation of TME was ≥50.0%(13/26), the incidence of postoperative complications was ≤35.0%(14/40) and the incidence of anastomotic leakage was ≤22.5%(9/40). There was no significant difference in the TME quality, postoperative complications or operation time among the four operation stages ( χ2 =3.252, 4.733, 8.848, P>0.05). There was a significant difference in the incidence of anastomotic leakage among the four operation stages ( P<0.05). (7) Learning curve of the two surgery teams: the number of operations corresponding to the turning point of learning curve in number 2 and number 8 medical center was 28 and 16, respectively. The number of operations corresponding to the turning points of learning curve in number 18, number 33 and number 44 medical center was 12 and 58, 10 and 36, 14 and 36, respectively. The overall curve of number 2 medical center was in line with the trend of learning curve and 28 cases of operations was the minimum number needed to cross the learning curve. The biggest difference in learning curve was shown between the number 2 and number 33 medical center. The age and cases with tumor in stage T0 and (or) Tis, stage T1, stage T2, stage T3 or stage T4 of the T staging of the number 2 and number 33 medical center were (60±12)years, 3, 1, 9, 11, 20 and (65±10)years, 2, 3, 22, 15, 0, respectively, showing significant differences in the above indicators between the two medical centers ( t=-2.280, Z=-4.033, P<0.05). Conclusion:Thirty-nine cases of operations was the minimum number for the one surgery team to cross the learning curve of laparoscopic rectal cancer taTME and 28 cases of operations was the minimum number for the two surgery teams to cross the learning curve of laparoscopic rectal cancer taTME.

Objective:To investigate the safety and the efficacy of percutaneous and surgical approach in femoro-femoral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) cannulation.Methods:All consecutive patients implanted with femoro-femoral VA-ECMO between January 2018 and December 2020 in Beijing Anzhen Hospital, Capital Medical University. Propensity score matching was used to compare outcomes of percutaneous and surgical groups while controlling for confounders.Results:Among the 276 patients who received femoro-femoral VA-ECMO (62 surgical and 214 percutaneous), propensity-score matching selected 52 pairs of patients with similar characteristics with mean age of(59.6±13.0)years old, in which 26 patients were female. There were a lower ECMO cannulation-associated complication (28.8% vs. 48.1%, P=0.044) and a lower hospital mortality (42.3% vs. 67.3%, P=0.010) in the percutaneous group. The circuit blood flow after ECMO initiation was similar in both groups[(3.3±0.8)L·min -1·kg -1 in percutaneous group vs. (3.2±0.7)L·min -1·kg -1 in surgical group, P=0.738]. The serum lactate was declined in both group after ECMO initiation[(5.4±5.8)mmol/L vs. (9.2±6.9)mmol/L, P<0.001 in percutaneous group; (6.3±6.2)mmol/L vs. (10.5±7.0)mmol/L, P=0.003 in surgical group]. Conclusion:Percutaneous approach is a safe and efficient technique in emoro-femoral VA-ECMO cannulation. Compared with surgical cannulation, percutaneous approach is associated with lower ECMO cannulation-associated complication and lower hospital mortality.

Objective:To explore the risk factors of lymph node metastasis and prognosis in Siewert Ⅱ/Ⅲ adenocarcinoma of esophagogastric junction (AEG) patients.Methods:A total of 134 patients who underwent surgical operation between June 2013 and December 2019 at the Beijing Friendship Hospital, Capital Medical University were retrospectively reviewed, including 112 males and 22 females, with a male to female ratio of 5.5∶1 and an average age of 62.1(27-82 years old). The primary outcomes were lymph node metastasis risk and 3-years overall survival. The secondary outcomes were the rate and pattern of lymph node metastasis. Follow-up methods mainly include outpatient and telephone. During the follow-up period, the patient needs to receive physical examination, laboratory examinations, chest and abdominal CT scan and gastroscopy to evaluate the status of disease. The patients were followed up until January 2020. Chi-square test or Fisher test was used for the comparison between count data group, and rank sum test was used for the comparison between grade data group. Stepwise Logistic regression was used for multivariate analysis, and COX regression risk model was used for survival analysis.Results:Multivariate analysis revealed that the parameters infiltration depth ( OR=4.341, 95% CI: 2.498-7.545, P=0.000), gross type ( OR=3.626, 95% CI: 1.425-9.228, P=0.007) and intravascular cancer embolus ( OR=2.888, 95% CI: 1.106-7.544, P=0.030) correlated with lymph node metastasis. For all patients, the lymph nodes No. 1, 2, 3, 4, 7, 11 indicated higher lymph node metastatic rate in the abdominal cavity. However, No.5 and No. 6 indicated different tendency, which was higher in Siewert Ⅲ AEG and lower in Siewert Ⅱ AEG patients. Mediastinal lymph node metastasis of Siewert Ⅱ AEG mainly occurred in No. 110 and No. 111 cases corresponding to 7.1 and 3.0%, respectively, compared with those noted in Siewert Ⅲ AEG patients. The 3-year overall survival analysis revealed that lymph node metastasis (82.1% vs 46.1%, P=0.046) and chemotherapy (60.6% vs 39.4%, P=0.007) exhibited significant differences. Conclusions:The infiltration depth, gross type and intravascular cancer embolus are independent risk factors of lymph node metastasis. In addition, patients with lymph node metastasis exhibite worse long-term prognosis. The data indicate that perioperative chemotherapy could improve the prognosis of AEG patients.

OBJECTIVE: To explore the clinical characteristics and molecular basis for a Chinese boy affected with jaundiced skin and liver disease. METHODS: The patient was subjected to clinical examination and laboratory tests. Genomic DNA of the patient and his parents was extracted and analyzed by using next generation sequencing (NGS). Suspected mutations were analyzed with bioinformatic software and verified by Sanger sequencing. RESULTS: The patient had jaundice in his eyes and skin. Serum bilirubin was elevated along with hepatomegaly. Next generation sequencing showed that the patient has carried c.18C>A(p.C6X) and c.2556delA mutations in the MRP2 gene, which were respectively inherited from his father and mother. CONCLUSION The missense mutation c.18C>A and frameshift mutation c.2556delA probably account for the disease. NGS has provided a powerful tool for the diagnosis of rare genetic diseases including Dubin-Johnson syndrome.
Asian Continental Ancestry Group ; DNA ; High-Throughput Nucleotide Sequencing ; Humans ; Jaundice, Chronic Idiopathic ; Male ; Mutation

OBJECTIVE: To identify potential mutations of the CLS gene in a Chinese pedigree affected with Coffin-Lowry syndrome. METHODS: Whole exome sequencing was applied to detect potential mutation in the proband, and the result was verified by Sanger sequencing. RESULTS: The proband was found to carry a c.966_967delAA (p.Arg323Thr fs*11) deletional mutation in the RPS6KA3 gene. The same mutation was also found in his mother. CONCLUSION The c.966_967delAA (p.Arg323Thr fs*11) deletional mutation of the RPS6KA3 gene probably underlies the disorder in this pedigree.
Asian Continental Ancestry Group ; China ; Coffin-Lowry Syndrome ; genetics ; Humans ; Mutation ; Pedigree ; Ribosomal Protein S6 Kinases, 90-kDa ; genetics ; Sequence Deletion

OBJECTIVE To identify potential mutations in five infants with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). METHODS The SLC25A13 gene was analyzed by next-generation sequencing. Suspected mutations were confirmed by PCR and Sanger sequencing in the probands and their parents. Impact of novel mutations was predicted with PolyPhen-2 software. RESULTS All neonates have harbored mutations of the SLC25A13 gene. Eight mutations were discovered, which included two novel mutations (c.1357A>G and c.1663dup23). All parents were found to be carriers of the mutations. CONCLUSION Mutations of the SLC25A13 gene probably underlie the NICCD among the five patients, among which 851del4 and 1638-1660dup were the most common ones. This has enriched the spectrum of SLC25A13 mutation in association with NICCD.