To explore the role of the "Clinical Practice Guidelines" column and others in the Medical Joumnal of Peking Union Medical College Hospital (Med J PUMCH) in promoting diseiplinary development.

Objective:To investigate the influence of curative-intent resection with textbook outcomes of liver surgery (TOLS) on long-term prognosis of gallbladder carcinoma (GBC).Methods:The retrospective cohort study was conducted. The clinicopathological data of 824 patients with GBC in the national multicenter database of Biliary Surgery Group of Elite Group of Chinese Journal of Digestive Surgery, who were admitted to 15 medical centers from January 2014 to January 2021, were collected. There were 285 males and 539 females, aged (62±11)years. According to the evalua-tion criteria of TOLS, patients were divided into those who achieved TOLS and those who did not achieve TOLS. Measurement data with normal distribution were represented as Mean± SD, and com-parison between groups was conducted using the independent sample t test. Measurement data with skewed distribution were represented as M( Q1, Q3), and comparison between groups was conducted using the Mann-Whitney U test. Count data were described as absolute numbers, and comparison between groups was conducted using the chi-square test. Comparison of ordinal data were conduc-ted using the Mann-Whitney U test. The Kaplan-Meier method was used to calculate survival rate and draw survival curve, and the Log-rank test was used for survival analysis. The COX stepwise regression model with backward Wald method was used for univariate and multivariate analyses. Results:(1) Achievement of TOLS. Of the 824 patients undergoing curative-intent resection for GBC, there were 510 cases achieving TOLS and 314 cases not achieving TOLS. (2) Follow-up. Of the 824 patients undergoing curative-intent resection for GBC, after excluding 112 deaths within 90 days after discharge, 712 cases were included for the survival analysis. The median follow-up time, median overall survival time and 5-year overall survival rate of the 510 patients achieving TOLS were 22.1(11.4,30.1)months, 47.6(30.6,64.6)months and 47.5%. The median follow-up time, median overall survival time and 5-year overall survival rate of the 202 patients not achieving TOLS were 14.0(6.8,25.5)months, 24.3(20.0,28.6)months and 21.0%. There was a significant difference in overall survival between patients achieving TOLS and patients not achieving TOLS ( χ2=58.491, P<0.05). (3) Analysis of factors influencing prognosis of patients. Results of multivariate analysis showed that TOLS, carcinoembryonic antigen (CEA), CA19-9, poorly differentiation of tumor, T2 stage of eighth edition of American Joint Committee on Cancer (AJCC) staging, T3 and T4 stage of eighth edition of AJCC staging, N1 stage of the eighth edition of AJCC staging, N2 stage of the eighth edition of AJCC staging, adjuvant therapy were independent factors influencing overall survival time of patients undergoing curative-intent resection for GBC ( hazard ratio=0.452, 1.479, 1.373, 1.612, 1.455, 1.481, 1.835, 1.978, 0.538, 95% c onfidence interval as 0.352-0.581, 1.141-1.964, 1.052-1.791, 1.259-2.063, 1.102-1.920, 1.022-2.147, 1.380-2.441, 1.342-2.915, 0.382-0.758, P<0.05). Conclusion:Patients under-going curative-intent resection for GBC with TOLS can achieve better long-term prognosis.

Objective:To investigate the influencing of preoperative total bilirubin (TBil) on perioperative complications of hepatolithiasis receiving liver resection.Methods:The retrospective cohort study was conducted. The clinical data of 300 patients with hepatolithiasis who were admitted to 2 medical centers from January 2010 to January 2022 were collected. There were 115 males and 185 females, aged (54±13)years. Measurement data with normal distribution were represented as Mean± SD, and the independent sample t test was used for comparison between groups. Measurement data with skewed distribution were represented as M( Q1, Q3), and the Mann-Whitney U test was used for comparison between groups. Count data were expressed as absolute numbers, and the chi-square test was used for comparison between groups. Variables with P<0.10 in the univariate analysis were included into the multivariate analysis. Univariate analysis was conducted using the Logistic regression model,and multivariate analysis was conducted using the Logistic stepwise regression model with backward Wald method. Continuous variables were converted into categorical variables based on commonly reported cutoff values when conducting Logistic regression analysis. Results:(1) Comparison of clinical data of patients with different preoperative TBil. Of 300 patients with hepatolithiasis, there were 252 cases with low level of preoperative TBil as 14.4(11.1,19.7)μmol/L, and there were 48 cases with high level of preoperative TBil as 44.0(31.3,59.8)μmol/L. Of the pati-ents with low level of preoperative TBil, neutrophils percentage was 62%±10%, cases with intra-operative blood transfusion was 29, and cases undergoing anatomical liver resection was 166. Of the patients with high level of preoperative TBil, neutrophils percentage was 70%±11%, cases with intraoperative blood transfusion was 22, and cases undergoing anatomical liver resection was 15. There were significant differences in cases classified as>grade 2 of ASA classification, neutrophils percentage, cases with intraoperative blood transfusion and cases undergoing anatomical liver resection between patients with low and high level of preoperative TBil ( t=5.182, χ2=33.669, 18.775, P<0.05). (2) Comparison of perioperative complications of patients with different preoperative TBil. Of the 252 patients with low level of TBil, there were 151 cases with complications including 35 cases of serious complications, there was 1 case with postoperative liver failure, the duration of postoperative hospital stay was 13.0(10.0,16.0)days. Of the 48 patients with high level of TBil, there were 32 cases with complications including 17 cases of serious complications, there were 6 cases with postoperative liver failure, the duration of postoperative hospital stay was 14.0(10.0,18.8)days. There were significant differences in cases with serious complications and cases with postoperative liver failure between patients with low and high level of preoperative TBil ( χ2=13.041, 20.879, P<0.05). (3) Analysis of factors influencing postoperative serious complications in patients undergoing liver resection. Results of multivariate analysis showed that age, body mass index (BMI), preoperative TBil and volume of intraoperative blood loss were independent factors influencing postoperative serious complications in patients undergoing liver resection for hepatolithiasis ( odds ratio=3.852, 2.358, 2.935, 5.135, 95% confidence interval as 1.478?9.979, 1.110?5.009, 1.398?6.158, 2.088?12.626, P<0.05). Conclusions:Patients with high level of preoperative TBil have a significantly increased risk of postoperative serious complications and liver failure who receive liver resection for hepatolithiasis. Age, preoperative BMI, TBil and volume of intraoperative blood loss are independent factors influencing postoperative serious complications in patients undergoing liver resection for hepatolithiasis.

Background/Aims: Ubiquitination is widely involved in the progression of hepatocellular carcinoma (HCC) by regulating various cellular processes. However, systematic strategies for screening core ubiquitin-related genes, clarifying their functions and mechanisms, and ultimately developing potential therapeutics for patients with HCC are still lacking. Methods: Cox and LASSO regression analyses were performed to construct a ubiquitin-related gene prediction model for HCC. Loss- and gain-of-function studies, transcriptomic and metabolomics analysis were used to explore the function and mechanism of UBE2S on HCC cell glycolysis and growth. Results: Based on 1,423 ubiquitin-related genes, a four-gene signature was successfully constructed to evaluate the prognosis of patients with HCC. UBE2S was identified in this signature with the potential to predict the survival of patients with HCC. E2F2 transcriptionally upregulated UBE2S expression by directly binding to its promoter. UBE2S positively regulated glycolysis in a HIF-1α-dependent manner, thus promoting the proliferation of HCC cells. Mechanistically, UBE2S enhanced K11-linkage polyubiquitination at lysine residues 171 and 196 of VHL independent of E3 ligase, thereby indirectly stabilizing HIF-1α protein levels by mediating the degradation of VHL by the proteasome. In particular, the combination of cephalomannine, a small molecule compound that inhibits the expression of UBE2S, and PX-478, an inhibitor of HIF-1α, significantly improved the anti-tumor efficacy. Conclusions UBE2S is identified as a key biomarker in HCC among the thousands of ubiquitin-related genes and promotes glycolysis by E3 enzyme-independent ubiquitination, thus serving as a therapeutic target for the treatment of HCC.

Objective:To explore the potential immune mechanism of pediatric ABOi-LDLT presenting low humoral immune response to donor specific blood group antigen.Methods:From June 2013 to December 2020, clinical data were retrospectively reviewed for 29 patients of long-term surviving pediatric ABOi-LDLT.There were A to O ABOi-LDLT( n=10)and B to O ABOi-LDLT( n=19). Graft types included left lateral lobe( n=26)and left hemi-liver( n=3). The median age of liver transplantation was 10 months, the median weight 8.0 kg and the median follow-up time 41.9 months.The titers of donor specific blood group antibodies and non-donor specific blood group antibodies(IgG, IgM)were continuously monitored before transplantation and at 1, 3, 6, 12, 24, 36 months post-transplantation.Protocol or event-based liver biopsy was performed to determine whether or not there was antibody-mediated rejection. Results:The titer of donor specific blood group antibody in recipients was significantly lower than that of non-donor specific blood group antibody( P<0.001). Among 18 protocol liver pathological biopsies, two cases were C4d positive for vascular endothelium.Five abnormal event-based liver biopsies were completed and one was C4d positive in bile duct endothelium.No pathological sign of typical blood group antibody mediated antigen-antibody complex mediated cascade immune reaction was detected in liver pathological biopsy.Typical pathological signs of blood group antibody mediated rejection were absent in protocol liver biopsy. Conclusions:Donor specific blood group antibody is expressed at a low level in pediatric ABOi-LDLT recipients.It presents as incomplete immune tolerance to donor specific blood group antigen.

Objective To evaluate the clinical efficacy of liver transplantation in children with Alagille syndrome (ALGS). Methods Clinical data of 12 children with ALGS were collected and retrospectively analyzed. Clinical characteristics of children with ALGS, pathological characteristics of liver tissues, characteristics of liver transplantation, postoperative complications and follow-up of children with ALGS were analyzed. Results JAG1 gene mutation and typical facial features was present in all 12 children. Jaundice was the most common initial symptom, which occurred at 7 (3, 40) d after birth. Upon liver transplantation, the Z scores of height and body weight were calculated as -2.14 (-3.11, -1.83) and -2.32 (-3.12, -1.12). Five children developed severe growth retardation and 4 children with severe malnutrition. Eight of 12 children were diagnosed with cardiovascular abnormalities. Pathological examination showed that the lobular structure of the diseased livers of 4 children was basically maintained, and 8 cases of nodular liver cirrhosis in different sizes including 1 case of single early moderately-differentiated hepatocellular carcinoma. Three children were misdiagnosed with biliary atresia and underwent Kasai portoenterostomy. Eight children underwent living donor liver transplantation, three children underwent cadaveric donor liver transplantation (two cases of split liver transplantation and one case of cadaveric total liver transplantation), and one child underwent domino liver transplantation (donor liver was derived from a patient with maple syrup urine disease). during the follow-up of 30.0(24.5, 41.7) months, the survival rates of the children and liver grafts were both 100%. During postoperative follow-up, the Z scores of height and body weight were calculated as -1.24 (-2.11, 0.60) and -0.83 (-1.65, -0.43), indicating that the growth and development of the children were significantly improved after operation. Conclusions Liver transplantation is an efficacious treatment for children with ALGS complicated with decompensated cirrhosis, severe itching and poor quality of life. For children with ALGS complicated with cardiovascular abnormalities, explicit preoperative evaluation should be delivered, and consultation with pediatric cardiologists should be performed if necessary.

Objective:To evaluate the application effect of palliative care based on multidisciplinary cooperation in patients with heart failure.Methods:Randomized controlled trials of palliative care in patients with heart failure were searched by computer in PubMed, Embase, CINAHL, The Cochrane Library, China Biology Medicine disc, CNKI, Wanfang Database, VIP and other Chinese and English databases. The search time limit was from the establishment of the databases to December 31, 2020. Two researchers independently screened the literature, extracted the data and evaluated the quality. RevMan 5.4 software was used to conduct a meta-analysis of the included studies.Results:A total of 12 studies were included, including 1 402 patients. Meta-analysis results showed that compared with conventional management, palliative care based on multidisciplinary collaboration could improve quality of life of patients with heart failure ( SMD=0.72, 95% CI: 0.32-1.11, P<0.01), reduce the burden of symptoms on patients ( MD= -12.90, 95% CI: -20.25--5.54, P<0.01), and reduce depression level ( MD=-0.38, 95% CI: -3.69--0.08, P<0.05), anxiety level ( MD=-0.35, 95% CI: -0.66--0.05, P<0.05) and rehospitalization rate of patients ( RR=0.70, 95% CI: 0.57-0.86, P<0.01). There was no statistically significant difference in mortality between patients ( RR=1.02, 95% CI: 0.63-1.63, P=0.94) . Conclusions:Palliative care based on multidisciplinary collaboration can improve the quality of life of patients with heart failure, reduce the burden of symptoms, depression level and anxiety level and reduce the rehospitalization rate of patients, but it has no significant effect on reducing the mortality rate of patients. Given that palliative care is still in its infancy in China and is limited by the quality and quantity of included studies, the above conclusions need to be verified by more large-sample and high-quality studies.

To analyzed a case of pediatric patient with propionic acidemia combined with dilated cardiomyopathy retrospectively, who underwent living donor liver transplantation at the Liver Transplantation Center, Beijing Friendship Hospital, Capital Medical University in March 2019.A 2 years and 6 months female child was admitted to hospital for propionic acidemia.The pretransplant echocardiogram showed left ventricular dilatation and systolic dysfunction, and thus dilated cardiomyopathy was considered.A living donor liver transplant was performed using her mother′s left latera-llobe.On the 14 months postoperatively, the child was on a liberated protein diet, but still required levocarnitine supplementation.Her hepatic and cardiac function returned normal, but growth retardation was still present.During the follow-up period, further propionic acidemia-related complications like metabolic decompensation, or any transplant-related complications were not reported.This case report suggested that liver transplantation is effective on pediatric propionic acidemia combined with cardiomyopathy, which reverses cardiomyopathy, improves cardiac function, relieves strict protein restriction, reduces the risk of metabolic decompensation, and significantly improves quality of life.

Objective:To study the hepatic hemodynamics changes and pathophysiological mechanisms of the use of a functional shunt after auxillary liver transplantation to treat portal hypertension associated with a small-for-size graft.Methods:A retrospective analysis of the clinical data of patients with portal hypertension treated with functional shunting of small-volume grafts from a living donor liver at the Beijing Friendship Hospital, Capital Medical University from July 2014 to December 2018, and a total of 6 patients were included as the research objects, including 4 males and 2 females, with a median age of 35.5 (29.0-52.0) years old. Blood flow monitoring data were collected during and after operation, and the characteristics of liver hemodynamics were analyzed.Results:The portal venous blood flow of the remnant native liver gradually decreased to no flow. As a buffer response, the flow velocity of hepatic artery increased. The portal venous blood flow of the graft gradually increased in the early postoperative period and then gradually decreased from post-operation Day 5 to 10 due to gradual increase in portal venous resistance. However, the portal venous perfusion gradually increased from Day 10 after the operation, reached to a level and declined to a stable level about 1 month after the operation. The volume of abdominal drainage slowly decreased after the peak level at Day 5-10 after the operation, and disappeared completely at Day 30 after operation.Conclusions:When using auxiliary liver transplantation for functional shunting to treat portal hypertension, autologous residual liver can act as a guide buffer for the pressure gradient of portal vein hyperperfusion in liver transplantation, and reach a steady state of blood flow distribution about 1 month after surgery, while relying on autologous remnant liver hepatic artery buffer response prevents small liver syndrome.

Objective:To review our experience in the use of "Full right-Full left" split liver transplantation in adult-to adult or adult-to adult-size child.Methods:The clinical data of liver donors to 4 recipients of full right-full left split liver transplantation performed at Beijing Friendship Hospital of Capital Medical University from January to December 2019 were reviewed. The surgical methods of split liver transplantation, cold ischemia time, operation time, intraoperative blood transfusion, and postoperative complications and prognosis were analyzed.Results:The 4 recipients of complete right hepatic-left hepatic split liver transplantation included 3 adults and 1 heavy child (45 kg). Their ages ranged from 14 to 48 years, and body weight ranged from 45 to 61 kg. The end-stage liver disease model score were 21, 12, 41, and 30 points. The ratios of graft mass to recipient's body mass ranged from 0.85% to 1.35%. The cold ischemia time was 457-650 min, and the operation time was 460-575 min. Early liver function recovered smoothly in all the 4 patients after transplantation, and no small liver syndrome occurred. Patients were followed up to 6 months after operation. One patient developed anastomotic biliary leak, which was cured by endoscopic retrograde cholangiopancreatographic treatment. Another patient developed biliary stricture presenting with repeated biliary tract infection despite percutaneous transhepatic puncture biliary drainage. A third patient died six months from lung infection.Conclusion:In properly selected patients, using full right-full left hemiliver by split liver transplantation increased organ utilization and provided patients with increased treatment opportunities.