ObjectiveTo investigate the role and mechanism of hyodeoxycholic acid （HDCA） in the progression of metabolic associated fatty liver disease （MAFLD）， and to provide a new theoretical basis for further clarifying the pathogenesis of MAFLD. MethodsL02 hepatocytes were used as experimental cells， and palmitic acid was used to induce steatosis in L02 cells. The farnesoid X receptor （FXR） siRNA interference chain technique was used to construct a hepatocyte cell line with low FXR expression. CCK8 assay was used to observe the effect of HDCA on L02 steatosis hepatocytes at different concentrations （0， 100， 200， 300， and 400 μmol/L） and time points （12， 24， 36， and 48 hours）. The method of qRT-PCR was used to measure the mRNA expression levels of FXR， proliferating cell nuclear antigen （PCNA）， Cyclin D1， phosphatidylinositol 3-kinase （PI3K）， and protein kinase-B （AKT）， and Western blot was used to measure the protein expression levels of FXR， Cyclin D1， PCNA， PI3K， phosphorylated PI3K （p-PI3K）， AKT， and phosphorylated （p-AKT）. A one-way analysis of variance was used for comparison of normally distributed continuous data with homogeneity of variance between multiple groups， and the Tukey HSD test was used for further comparison between two groups； the Welch analysis of variance was used for comparison of normally distributed continuous data with heterogeneity of variance between multiple groups， and the Games-Howell test was used for further comparison between two groups. The independent-samples t test was used for comparison between two groups. ResultsCCK8 assay showed a significant reduction in the viability of L02 cells and steatosis hepatocytes treated by 300 μmol/L HDCA （P<0.05）， and qRT-PCR showed a significant increase in the mRNA expression level of FXR and significant reductions in the mRNA expression levels of PCNA， Cyclin D1， PI3K， and AKT （all P<0.05）. Western blot showed a significant increase in the protein expression level of FRX （P<0.05）， and after interference of FXR expression in L02 cells， there were significant increases in the protein expression levels of PCNA， PI3K， p-PI3K， AKT， and p-AKT （all P<0.05）. ConclusionHDCA inhibits the PI3K/AKT signaling pathway by upregulating FXR expression， thereby inducing a reduction in the viability of steatosis hepatocytes.

Objective:To enhance comprehension of undifferentiated embryonal sarcoma of the liver(UESL)in children by analyzing ultra-sound,CT,and MRI imaging features.Methods:A retrospective analysis was conducted on 11 cases of UESL in children,confirmed through surgery and pathology,at the Children's Hospital,Affiliated Capital Institute of Pediatrics from December 2009 to December 2021.We ana-lyzed the ultrasound,CT,and MRI imaging features of all patients and summarized their characteristics.Results:All 11 cases presented with solitary hepatic masses ranging from 11.5 to 19.8 cm in diameter.Imaging manifestations of UESL correlated with component proportion and distribution within the masses.Lesions displayed clear boundaries in all cases.CT scans revealed mixed low density in 11 cases,with ir-regular floc soft tissue density shadows observed at the edge of cystic density areas or around partitions in a few cases.Ultrasound images of all six cases showed solid space-occupying masses,with varying sizes of anechoic regions within the solid mass.MRI T1WI showed mixed low intensity signal in three cases and strip/large high intensity signal areas in the lesion.T2WI revealed mixed high intensity signal and strip low intensity signal areas in 3 lesions.In the arterial phase,lesions displayed slightly to moderately heterogeneous strip/patch enhancement,primarily marginal enhancement in nine cases and thickened,tortuous arterial shadows in eight cases.In the delayed phase,lesions showed continuous uneven enhancement,with enhancement at the edge and peripheral-to-central filling observed in eight cases.Additionally,the enhancement range continuously increased in eight cases,with the false capsule sign identified in eight cases in the delayed stage.Conclu-sions:Imaging features of UESL in children exhibit distinct characteristics.Understanding these features,in conjunction with clinical findings,may aid in early diagnosis.

Objective To investigate the reasons for the inconsistent results between the vertical auto profile(VAP)method and bio-chemical homogeneous phase(BHP)method in detecting plasma low-density lipoprotein cholesterol(LDL-C),and provide experimen-tal basis for the accurate and quantitative detection of plasma LDL-C levels.Methods A total of 360 plasma samples from diabetes mellitus patients combined with carotid plaque admitted to the Department of Endocrinology of Ningbo Yinzhou Hospital of Traditional Chinese Medicine during January,2022 and January,2023 were collected.The LDL-C levels of these samples were detected by the VAP method and BHP method,respectively.The VAP method uses software to automatically calculate the area under the LDL-C curve after centrifugation of the sample as the LDL-C level(LDL-CVAP)and the BHP method directly detects the LDL-C level(LDL-CBHP)by the special surfactant method.360 samples were divided into the consistent group(group A)and inconsistent group(group B)ac-cording to the relative deviation between the LDL-CBHP and LDL-CVAP methods.Group B was further divided into the LDL-CBHP on the high side group(Group B1)and LDL-CBHP on the low side group(Group B2).Groups B1 and B2 were divided into B1-1,B1-2,B1-3 and B2-1 groups based on the degree of relative deviation.The percentages of samples and levels of lipoprotein a cholesterol[Lp(a)-C],intermediate-density lipoprotein cholesterol(IDL-C),Lp(a)-C and IDL-C[Lp(a)-C+IDL-C],very low-density lipo-protein cholesterol(VLDL-C),total cholesterol(TC)and total triglyceride(TG)in each group were compared.Results The LDL-CBHP levels of 360 samples were significantly higher than that of LDL-CVAP(P＜0.01).The percentage of samples in group B was significantly higher than that in group A,and that of group B1 was significantly higher than that of group B2(P＜0.05).The levels of Lp(a)-C,IDL-C and Lp(a)-C+IDL-C in groups B1-1,B1-2,and B1-3 were significantly higher than those in group A(P＜0.01).The relative deviation between LDL-CBHP and LDL-CVAP in 360 samples was significantly positively correlated with the levels of Lp(a)-C,IDL-C,and Lp(a)-C+IDL-C(P＜0.01).The maximum correlation coefficient was found in Lp(a)-C+IDL-C.Conclusion The results of plasma LDL-C in diabetes mellitus patients combined with carotid plaque detected by the BHP method are significantly different from those detected by the VAP method,which mainly shows that the results of the BHP method are on the high side.The higher the level of plasma Lp(a)-C+IDL-C,the greater the relative deviation between the BHP method and VAP method.The reason for the high results of LDL-C detected by the BHP method may be related to the fact that LDL-CBHP contains irremovable Lp(a)-C and cholesterol carried by IDL-C.The VAP method can be used as an accurate method for detecting real LDL-C without Lp(a)-C and IDL-C.

Objective:To investigate the epidemiological characteristics, diagnosis, treat-ment and prognosis of gallbladder cancer in China from 2010 to 2017.Methods:The single disease retrospective registration cohort study was conducted. Based on the concept of the real world study, the clinicopathological data, from multicenter retrospective clinical data database of gallbladder cancer of Chinese Research Group of Gallbladder Cancer (CRGGC), of 6 159 patients with gallbladder cancer who were admitted to 42 hospitals from January 2010 to December 2017 were collected. Observation indicators: (1) case resources; (2) age and sex distribution; (3) diagnosis; (4) surgical treatment and prognosis; (5) multimodality therapy and prognosis. The follow-up data of the 42 hospitals were collected and analyzed by the CRGGC. The main outcome indicator was the overall survival time from date of operation for surgical patients or date of diagnosis for non-surgical patients to the end of outcome event or the last follow-up. Measurement data with normal distribu-tion were represented as Mean±SD, and comparison between groups was conducted using the t test. Measurement data with skewed distribution were represented as M( Q1, Q3) or M(range), and com-parison between groups was conducted using the U test. Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test. Univariate analysis was performed using the Logistic forced regression model, and variables with P<0.1 in the univariate analysis were included for multivariate analysis. Multivariate analysis was performed using the Logistic stepwise regression model. The life table method was used to calculate survival rates and the Kaplan-Meier method was used to draw survival curves. Log-rank test was used for survival analysis. Results:(1) Case resources: of the 42 hospitals, there were 35 class A of tertiary hospitals and 7 class B of tertiary hospitals, 16 hospitals with high admission of gallbladder cancer and 26 hospitals with low admission of gallbladder cancer, respectively. Geographical distribution of the 42 hospitals: there were 9 hospitals in central China, 5 hospitals in northeast China, 22 hospitals in eastern China and 6 hospitals in western China. Geographical distribution of the 6 159 patients: there were 2 154 cases(34.973%) from central China, 705 cases(11.447%) from northeast China, 1 969 cases(31.969%) from eastern China and 1 331 cases(21.611%) from western China. The total average number of cases undergoing diagnosis and treatment in hospitals of the 6 159 patients was 18.3±4.5 per year, in which the average number of cases undergoing diagnosis and treatment in hospitals of 4 974 patients(80.760%) from hospitals with high admission of gallbladder cancer was 38.8±8.9 per year and the average number of cases undergoing diagnosis and treatment in hospitals of 1 185 patients(19.240%) from hospitals with low admission of gallbladder cancer was 5.7±1.9 per year. (2) Age and sex distribution: the age of 6 159 patients diagnosed as gallbladder cancer was 64(56,71) years, in which the age of 2 247 male patients(36.483%) diagnosed as gallbladder cancer was 64(58,71)years and the age of 3 912 female patients(63.517%) diagnosed as gallbladder cancer was 63(55,71)years. The sex ratio of female to male was 1.74:1. Of 6 159 patients, 3 886 cases(63.095%) were diagnosed as gallbladder cancer at 56 to 75 years old. There was a significant difference on age at diagnosis between male and female patients ( Z=-3.99, P<0.001). (3) Diagnosis: of 6 159 patients, 2 503 cases(40.640%) were initially diagnosed as gallbladder cancer and 3 656 cases(59.360%) were initially diagnosed as non-gallbladder cancer. There were 2 110 patients(34.259%) not undergoing surgical treatment, of which 200 cases(9.479%) were initially diagnosed as gallbladder cancer and 1 910 cases(90.521%) were initially diagnosed as non-gallbladder cancer. There were 4 049 patients(65.741%) undergoing surgical treatment, of which 2 303 cases(56.878%) were initially diagnosed as gallbladder cancer and 1 746 cases(43.122%) were initial diagnosed as non-gallbladder cancer. Of the 1 746 patients who were initially diagnosed as non-gallbladder cancer, there were 774 cases(19.116%) diagnosed as gallbladder cancer during operation and 972 cases(24.006%) diagnosed as gallbladder cancer after operation. Of 6 159 patients, there were 2 521 cases(40.932%), 2 335 cases(37.912%) and 1 114 cases(18.087%) undergoing ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) examination before initial diagnosis, respec-tively, and there were 3 259 cases(52.914%), 3 172 cases(51.502%) and 4 016 cases(65.205%) undergoing serum carcinoembryonic antigen, CA19-9 or CA125 examination before initially diagnosis, respectively. One patient may underwent multiple examinations. Results of univariate analysis showed that geographical distribution of hospitals (eastern China or western China), age ≥72 years, gallbladder cancer annual admission of hospitals, whether undergoing ultrasound, CT, MRI, serum carcinoembryonic antigen, CA19-9 or CA125 examination before initially diagnosis were related factors influencing initial diagnosis of gallbladder cancer patients ( odds ratio=1.45, 1.98, 0.69, 0.68, 2.43, 0.41, 1.63, 0.41, 0.39, 0.42, 95% confidence interval as 1.21-1.74, 1.64-2.40, 0.59-0.80, 0.60-0.78, 2.19-2.70, 0.37-0.45, 1.43-1.86, 0.37-0.45, 0.35-0.43, 0.38-0.47, P<0.05). Results of multivariate analysis showed that geographical distribution of hospitals (eastern China or western China), sex, age ≥72 years, gallbladder cancer annual admission of hospitals and cases undergoing ultrasound, CT, serum CA19-9 examination before initially diagnosis were indepen-dent influencing factors influencing initial diagnosis of gallbladder cancer patients ( odds ratio=1.36, 1.42, 0.89, 0.67, 1.85, 1.56, 1.57, 0.39, 95% confidence interval as 1.13-1.64, 1.16-1.73, 0.79-0.99, 0.57-0.78, 1.60-2.14, 1.38-1.77, 1.38-1.79, 0.35-0.43, P<0.05). (4) Surgical treatment and prognosis. Of the 4 049 patients undergoing surgical treatment, there were 2 447 cases(60.435%) with complete pathological staging data and follow-up data. Cases with pathological staging as stage 0, stage Ⅰ, stage Ⅱ, stage Ⅲa, stage Ⅲb, stage Ⅳa and stage Ⅳb were 85(3.474%), 201(8.214%), 71(2.902%), 890(36.371%), 382(15.611%), 33(1.348%) and 785(32.080%), respectively. The median follow-up time and median postoperative overall survival time of the 2 447 cases were 55.75 months (95% confidence interval as 52.78-58.35) and 23.46 months (95% confidence interval as 21.23-25.71), respectively. There was a significant difference in the overall survival between cases with pathological staging as stage 0, stage Ⅰ, stage Ⅱ, stage Ⅲa, stage Ⅲb, stage Ⅳa and stage Ⅳb ( χ2=512.47, P<0.001). Of the 4 049 patients undergoing surgical treatment, there were 2 988 cases(73.796%) with resectable tumor, 177 cases(4.371%) with unresectable tumor and 884 cases(21.833%) with tumor unassessable for resectabi-lity. Of the 2 988 cases with resectable tumor, there were 2 036 cases(68.139%) undergoing radical resection, 504 cases(16.867%) undergoing non-radical resection and 448 cases(14.994%) with operation unassessable for curative effect. Of the 2 447 cases with complete pathological staging data and follow-up data who underwent surgical treatment, there were 53 cases(2.166%) with unresectable tumor, 300 cases(12.260%) with resectable tumor and receiving non-radical resection, 1 441 cases(58.888%) with resectable tumor and receiving radical resection, 653 cases(26.686%) with resectable tumor and receiving operation unassessable for curative effect. There were 733 cases not undergoing surgical treatment with complete pathological staging data and follow-up data. There was a significant difference in the overall survival between cases not undergoing surgical treatment, cases undergoing surgical treatment for unresectable tumor, cases undergoing non-radical resection for resectable tumor and cases undergoing radical resection for resectable tumor ( χ2=121.04, P<0.001). (5) Multimodality therapy and prognosis: of 6 159 patients, there were 541 cases(8.784%) under-going postoperative adjuvant chemotherapy and advanced chemotherapy, 76 cases(1.234%) under-going radiotherapy. There were 1 170 advanced gallbladder cancer (pathological staging ≥stage Ⅲa) patients undergoing radical resection, including 126 cases(10.769%) with post-operative adjuvant chemotherapy and 1 044 cases(89.231%) without postoperative adjuvant chemo-therapy. There was no significant difference in the overall survival between cases with post-operative adjuvant chemotherapy and cases without postoperative adjuvant chemotherapy ( χ2=0.23, P=0.629). There were 658 patients with pathological staging as stage Ⅲa who underwent radical resection, including 66 cases(10.030%) with postoperative adjuvant chemotherapy and 592 cases(89.970%) without postoperative adjuvant chemotherapy. There was no significant difference in the overall survival between cases with postoperative adjuvant chemotherapy and cases without postoperative adjuvant chemotherapy ( χ2=0.05, P=0.817). There were 512 patients with pathological staging ≥stage Ⅲb who underwent radical resection, including 60 cases(11.719%) with postoperative adjuvant chemotherapy and 452 cases(88.281%) without postoperative adjuvant chemotherapy. There was no significant difference in the overall survival between cases with postoperative adjuvant chemo-therapy and cases without post-operative adjuvant chemo-therapy ( χ2=1.50, P=0.220). Conclusions:There are more women than men with gallbladder cancer in China and more than half of patients are diagnosed at the age of 56 to 75 years. Cases undergoing ultrasound, CT, serum CA19-9 examination before initial diagnosis are independent influencing factors influencing initial diagnosis of gallbladder cancer patients. Preoperative resectability evaluation can improve the therapy strategy and patient prognosis. Adjuvant chemotherapy for gallbladder cancer is not standardized and in low proportion in China.

Objective:To understand the current status of rare disease related health information release in WeChat official accounts in China.Methods:We used a series of key words containing "rare diseases" and the names of the top 30 rare diseases in hospitalizations in China to search WeChat official accounts. Eligible articles were selected by systematic sampling. All including WeChat official accounts and articles were evaluated to extract the basic information.Results:No relevant WeChat official accounts were found for 14 rare diseases (46.67 %). Most of the WeChat official accounts (52.17 %) were initiated by patients and patient groups. No significant difference was detected in the total number of articles between the official accounts related with Traditional Chinese Medicine (TCM) and non-TCM related ones, however, the frequency of the monthly information release was significantly higher in TCM related official accounts ( P<0.001), while the average reading number of articles was significantly higher in non-TCM related official accounts ( P<0.001). Nearly 80 % of the WeChat official accounts had navigation menu, and the average reading number of official accounts with menus was larger than those without menus. The top three topics were rare disease diagnosis and treatment knowledge (46.00 %), public welfare activity for rare diseases (12.81 %) and uncorrelated things (8.65 %), while the first three leading topics were cutting-edge information, public welfare activity and patient story, respectively. Conclusions:The scale for rare disease related health information release based on WeChat official accounts in China has been basically formed, but it is still in development stage. Many improvements should be made in their coverage of rare diseases, release frequency, topic and form. It is urgent to establish or recreate some high-quality WeChat official accounts in order to provide precise information and effectively facilitate the prevention and treatment of rare diseases.

Objective To analyze the screening results of yon Willebrand factor among patients before blood transfusion in Ruijin Hospital and discuss von Willebrand factor in ABO blood group and the relationship between age and gender,refine the classification of vWF antigen and activity by reference factors.Methods The von Willebrand factor among 247 cases of patients before blood transfusion in Ruijin Hospital with no clinical manifestations of abnormal blood clots and routine coagulation as laboratory tests for normal surgical patients.The vWF:Ag and vWF:Act were measured by immune turbidimetric method and ABO blood group was identified by blood type serology.Furthermore,the differences between A,B,O,AB different blood groups,sex and high (≥40 years) and low age group (＜40 years) were compared by statistical methods.Results The levels of vWF:Ag in different blood groups were as follows:A blood type:98.5-142.00,B blood type:97.90-160.30,O blood type:82.13-125.45,A B blood type:103.00-135.80.The levels of vWF:Act in different blood groups were as follows:A blood type:76-130.14,B blood type:78.06-144.3,O blood type:60.89-116.11,AB blood type:88.99-124.09.O blood type vWF:Ag and vWF:Act were lower significantly (P＜0.05) than non-O blood type,the difference was.Besides,young vWF:Ag and vWF:Act were lower significantly than in the elderly.There was no significant difference in vWF:Ag and vWF:Act levels between male and female groups.At last,the reference range of four groups of vWF activity (antigen) was obtained.Conclusion Plasma vWF antigen and activity levels were significantly affected by ABO blood type and age,and the refined reference range established for these influencing factors was beneficial for more detailed diagnosis of VWD and predicting vWF levels associated with bleeding and thrombosis risk.

Objective To study the molecular mechanism of Bel subtype caused by mutation p.R168W of glycosyltransferase B.Methods Serological test,SSP-PCR and direct sequence the Exon6 and Exon 7 of the ABO gene.Construct a 3D molecular model and predict the structural impact of GTB protein mutations.Results A antigen or B antigen can't be detected on the surface of the propositus' RBC,and only anti-A antibodies were detected in her serum.But serological test indicated her daughter's blood type was a normal B type.SSP-PCR test indicated propositus' ABO gene type is O1 B.By gene sequencing the Exon 6 and Exon 7 of the ABO gene,a ABO Bel allel(c.502C＞T,p.R168W)was discoverd in both the propositus and her daughter.Through the propositus' daughter coexisted Bel gene with normal B gene,her blood type was a normal B type.Conclusions ABO gene c.502C＞T mutations cause Bel phenotypes in patients by reducing the stability of GTB.

Objective To investigate the CT features of hepatic focal nodular hyperplasia (FNH) in children.Methods Thirteen patients with FNH,which was confirmed by postoperative pathology,were enrolled retrospectively.Plain and contrast CT were performed on all patients before operation.The CT imaging features of FNH including size,shape,density,style of contrast were analyzed retrospectively and compared with pathology.Results There were 13 patients with 16 lesions,8 lesions were found in the right lobe,5 lesions in the left lobe and 3 lesions involving both lobes.The tumor size ranged from 5.5 cm to 11.5 cm (media size 7.5 cm) in diameter.Histologically,2 cases were typical type,11 cases were atypical type.The lesions were regular morphology in 12 cases and 1 case with capsule.On plain CT,the lesions were isodensity (n=1) or slightly low-density (n=12).In 2 typical type lesions,there were slit-like,stellate-shaped low density central scars.Arterial phase demonstrated that 12 cases were significantly enhanced and 1 case showed mild enhancement.The central scar was not enhanced.In 12 cases,thickened and torturous arteries were seen.The enhancement was reduced at the portal venous phase in all the lesions,with 10 cases showing slightly higher density,2cases isodensity and 1 case low-density.Two cases showed mild enhancement of the central scar.The enhancement of the solid portion in all lesions decreased at the delay phase,with 12 cases showing isodensity and 1 case slightly low density.Two cases with central scar showed delayed enhancement with slightly higher density.Conclusion The CT features of FNH in children are diversified but distinctive which are related with postoperative pathological findings.Combining with clinical symptoms and CT features can be helpful for the early diagnosis of FNH in children.

Objective To investigate and analyze the usage of expensive outpatient prescriptions in different hospitals in Longgang area,Shenzhen,and to discuss and provide reference for the rational usage of expensive pre-scriptions.Methods Two primary hospitals,two secondary hospitals and one tertiary hospital in the Longgang area, Shenzhen,were chosen as the research targets.The expensive outpatient prescriptions,defined as the amount of pre-scription was over 150,200 and 300 yuan for primary,secondary and tertiary hospital respectively,were randomly selected and evaluated using international standards for the rational use of drugs.Results The average prescription items for primary,secondary and tertiary hospitals were 2.96,2.61 and 2.61.The largest proportions of prescription costs were 150 to 200 yuan,200 to 250 yuan and 300 to 350 yuan,which were respectively accounted for 49.6%, 56.6% and 37.7%.The frequency of using proprietary Chinese medicine,was 60.08%,68.18% and 41.62%respectively,which was not reasonable.The clinical diagnosis for the prescriptions of primary and secondary hospitals was mainly composed of trauma,bronchitis and vaginitis.The usage of drugs was not reasonable about the use of anti-biotics and infusion.In addition,the clinical diagnosis for the prescription of tertiary hospital was mainly diabetes, hypertension and cerebral infarction,where the abuse of using large dose of adjuvant drug was observed.Conclusion The frequency of using proprietary Chinese medicine is higher at all levels of hospital.In order to reduce the propor-tion of expensive prescription,the medicine institution should establish a sound system for drug use management and strengthen supervision and verification for western medicine physicians using proprietary Chinese medicine in their prescription.Furthermore,we should intervene and restrict the abuse usage of the adjuvant medicine.

Objective To study and summarizes the methods ofthe standardized hospital infection manage -ment technology in order to prevent and control hospital infection scientifically .Methods Taking the chance of im-provingthe execution of the standardized hospital infection management issued by the Ministry of Health ,we realized organizational execution by enhancing the responsibility decomposition , mechanism implementation by standardized completion,ideological implementation by education and training ,the implementation of standards by inspection and monitoring and finally form a team by enhanced communication .Results The technicalstandards of hospital infection management was implemented and the quality of hospital infection control was improved progressively .Conclusion It is an effective way to guarantee medical security to improve the execution of the technical standard of hospital infec -tion management .