Biliary atresia is a category of disease caused by biliary cirrhosis due to progressive fibro-inflammatory obstruction of extrahepatic and intrahepatic bile ducts, and eventually progresses into liver failure. It is a common indication for liver transplantation in children. In this article, the pathogenesis, diagnosis of biliary atresia, timing, procedures and postoperative complications of liver transplantation were illustrated. Meantime, current status and development of pediatric liver transplantation were discussed.

Objective To investigate the clinical significance of BRCA1/ 2 mutation in breast cancer patients with different malignant tumor family history. Methods We studied 98 cases of diagnosed breast cancer patients with malignant tumor family history. BRCA1/2 screening was performed by PCR-DHPLC sequencing method. All mutations were confirmed by using direct DNA sequencing. Results The prevalence of BRCA1/2 germline mutation was 20.41%.The BRCA1/2 mutation was 55.6% in patients with family breast and ovarian cancer, and was 20.0% and 17.9% in patients with family breast and in patients with ovarian cancer, respectively. In correspondence to 2 and 3 and 4 people withof the breast or ovarian cancer in family , the BRCA1/2 mutation was 16.25%、33.3%、66.67% ,respectively. Conclusion The BRCA1/2 mutation rate increased in the patients with breast and ovarian cancer family history, and the detection of BRCA1/2 mutation increased with the number of patients with cancer in a family.

Objective To investigate the the level of red blood cell microparticls in patients with acute coronary syndromes and the correlation with severity of coronary artery disease and with its influencing factors.Methods A total of 140 patients were sdected into our study from Jun 2016 to Feb 2017.All patiens were identified through coronary angiography.There were 108 patients with acute coronary syndromes (ACS) mentioned as experimental group,which including 25 patients with single vessel disease,27 patients with double vessel disease,27 patients with triple vessel disease and 29 cases of multi-vessel disease,and 32 patients of non-coronary heart disease group mentioned as control group.Each patient was fasting and drawn the periheral venous blood after the next day of hospitalized then analyzing blood lipids,blood routine and biochemical indicators.The expression of CD235a in erythrocyte derived microparticles was quantitatively analyzed by flow cytometry.The Gensini score was calculated based on the Results of coronary angiography.Results Compared with the non-CHD group,the levels of red blood cells microparticles (44.37％ ± 15.48％),low density lipoprotein cholesterol (3.18 ± 1.16mg/L),lipoprotein a (215.23 ± 183.10mg/L) and high sensitivity C Protein (19.58 ± 26.94mg/L) were significantly increased,and the difference was statistically significant (P ＜ 0.05).The levels of erythrocyte microparticles and low-density lipoprotein cholesterol in the subgroups of acute coronary syndromes were statistically significant (P ＜ 0.05),with erythrocyte microparticles levels and low-density lipoprotein cholesterol levels (P ＜ 0.05).There was a positive correlation between Gensini score and erythrocyte drived microparticls (β =2.157,P ＜ 0.05),and negatively correlated with high density lipoprotein (β =-15.579,P ＜ 0.05).Conclusion The level of Gensini scores were calculated based on the results of coronary angiography.That in patients with acute coronary syndrome is significantly higher than that in patients with non-coronary heart disease.The level of erythrocyte derived microparticls is closely related to the severity of coronary artery disease.The level of erythrocyte derived microparticls has a high value in predicting the severity of coronary artery disease in acute coronary syndromes.

Objective To evaluate the effects of combining immune checkpoint inhibitors,trastuzumab,and chemotherapy on the left and right ventricular function in patients with advanced gastric adenocarcinoma expressing human epidermal growth factor receptor 2(HER2).Methods 25 patients with advanced HER2-positive gastric adenocarcinoma who received HER2-ASTRUM therapy(sruili monoclonal antibody combined with trastuzumab and DOS regimen)underwent echocardiographic examinations at baseline(T1),after completion of 4 cycles(T2),and 6 cycles(T3)of chemotherapy.Left ventricular strain,left ventricular myocardial work,right ventricular strain,and ejection fraction were further analyzed.Results Compared with T1,both T2 and T3 showed a decrease in left ventricular ejection fraction(LVEF),(T1,T2,and T3 were[67.3±2.7]％,[63.0±2.9]％and[61.3±3.2]％,P＜0.05).T3 showed a decrease in right ventricular ejection fraction(RVEF),([59.3±7.8]％vs[43.7±6.6]％,P＜0.05),while T2 showed no significant change in RVEF.Both right ventricular global longitudinal strain(RVGLS),(T1,T2,and T3 were[-25.7±5.7]％,[-22.4±5.3]％and[-19.1±3.6]％,respectively;P＜0.05)and right ventricular free-wall longitudinal strain(RVFWLS),(T1,T2,and T3 were[-20.9±4.7]％,[-18.9±4.1]％and[-16.1±3.9]％,P＜0.05)decreased in all three time points.The global work index(GWI)was lower in T2 and T3 than in T1(P＜0.05),while the global constructive work(GCW),global wasted work(GWW),and global work efficiency(GWE)did not show significant changes.Conclusions Combination of trastuzumab,immune checkpoint inhibitors,and chemotherapy has an impact on left ventricular strain,LVEF,and right ventricular strain,as well as GWI,in HER2-positive patients with gastric adenocarcinoma.Left ventricular global longitudinal strain(LVGLS),RVFWLS,and RVGLS can more sensitively monitor cardiac function and detect cardiac dysfunction related to cancer treatment in a timely manner.

Objective:To investigate the roles of PD-1 and PD-L1,as well as related cytokines TGF-βand IL-10 in patients infected with Brucella.Methods:The expression of PD-1/PD-L1 in peripheral blood mononuclear cell CD 3+T cells,CD4+T cells,CD8+T cells,and dendrites cells was detected by flow cytometry in 60 patients infected with Brucella and 34 patients receiving follow-up cured and 60 healthy controls,the serum levels of TGF-βand IL-10 were detected by CBA.The correlations of PD-1 with TGF-βand IL-10 levels in patients infected with Brucella were analyzed by pearson correlation test .Results: Compared with control,brucellosis patients had the higher PD-1/PD-L1,TGF-βand IL-10 expression (P<0.05),while in the patients receiving follow-up cured,the PD-1/PD-L1,TGF-βand IL-10 were remarkably lower than those in pretherapy (P<0.01),the change of PD-1 was positively correlated with the levels of TGF-β(r=0.817,P<0.01) and IL-10 (r=0.835,P<0.01).Conclusion: High expression of PD-1/PD-L1 and TGF-βand IL-10 in patients with Brucella may be involved in the negative regulation of Brucella on host immune response .

Objective To explore the left and right ventricular myocardial strain and dyssynchrony in patients with Micra AV leadless pacing implantation,and to further analyze the impact of implantation site on myocardial strain and dyssynchrony.Methods A retrospective study was conducted on 43 patients with Micra AV implantation and 20 patients with high-degree atrioventricular block(Ⅱdegree typeⅡandⅢdegree atrioventricular block)at the Department of Cardiology,Zhongshan Hospital from April 2023 to December 2023.The demographic information and clinical characteristics of the patients were collected.Echocardiography and speckle tracking imaging techniques were used to obtain conventional echocardiographic parameters,myocardial strain,and dyssynchrony indices of patients in the two groups,including global longitudinal strain(GLS),free wall longitudinal strain(FWLS),peak strain dispersion(PSD)of 18 left ventricular segments,PSD of 6 right ventricular segments,and other indices.According to the implantation location of Micra AV,the patients were further divided into middle group(right ventricular septum),high group(right ventricular inflow tract),and low group(apical region),and the differences in myocardial strain and dyssynchrony indices among the three subgroups were compared.Results The left ventricular GLS(LVGLS),right ventricular GLS(RVGLS),and right ventricular FWLS(RVFWLS)of the Micra AV group were significantly lower than those of the conduction block group(P＜0.001),and the right ventricular dyssynchrony of the Micra AV group was significantly higher than that of the conduction block group(P＜0.05).Comparison of myocardial strain and dyssynchrony at different implantation sites showed that the LVGLS of the high implantation group was significantly higher than that of the middle and low implantation groups,and the left ventricular dyssynchrony index Yu was significantly lower than that of the middle group(P＜0.05);the right ventricular ejection fraction(RVEF)of the low implantation group was significantly lower than that of the high group,and the right ventricular 6-segment PSD was significantly higher than that of the middle and high implantation groups(P＜0.05).Conclusions Compared with patients with high-degree atrioventricular block,patients with Micra AV leadless pacing have reduced left and right ventricular strain,and the implantation of low position had greater effect.

Objective:To investigate the efficacy of one-stage anterior debridement and bone graft fusion for the treatment of cervical pyogenic spondylodiscitis.Methods:This is a retrospective case series study. Retrospective analysis of clinical data from 23 patients with cervical pyogenic spondylodiscitis treated with one-stage anterior approach debridement and bone graft fusion was performed in the Department of Spinal Surgery, the First Affiliated Hospital of Xinjiang Medical University from January 2015 to January 2020. There were 14 males and 9 females,aged (51.9±12.8) years (range:26 to 82 years). Preoperatively, 14 patients had neurological deficits, classified according to the American Spinal Injury Association (ASIA) impairment scale as follows: grade A in 1 case, grade B in 1 case, grade C in 5 cases, and grade D in 7 cases . All patients underwent the one-stage anterior debridement and fusion procedure. The surgical time, blood loss, hospital stay, fusion time, and surgical complications were documented. Clinical efficacy was assessed using the visual analogue scale (VAS), the neck disability index (NDI), and the ASIA impairment scale. Preoperative and postoperative data were compared using paired sample t tests, repeated measures analysis of variance, and generalized estimating equations. Results:All the 23 patients underwent the operative procedures successfully. The operation time was (102.8±19.8) minutes (range:60 to 140 minutes), blood loss was (84.4±40.2) ml (range:30 to 160 ml), and the length of hospital stay was (17.4±6.0) days (range:10 to 30 days). Blood cultures were positive for the causative pathogen in 14 cases (60.8% positivity rate), while 9 cases had negative results. Irrigation fluid cultures yielded the causative pathogen in 19 cases (82.6% positivity rate), with 4 cases negative. All patients were followed up for more than 12 months, with a follow-up duration of (19.0±5.9) months (range:12 to 36 months). At the final follow-up, VAS improved from (5.9±1.1) points preoperatively to (0.8±0.3) points; NDI improved from (38.3±6.0)% preoperatively to (9.3±3.0)%, with statistically significant differences (both P<0.01). All patients experienced improvement in neurological function, with the exception of one patient in grade C and two in grade D, all other patients recovered to grade E. The C 2-7 Cobb angle and the affected segment Cobb angle were corrected. white blood cell, erythrocyte sedimentation rate and C-reactive protein levels returned to normal. All patients achieved bony fusion, with a fusion time of (8.9±1.9) months (range:6 to 12 months). Two diabetic patients developed postoperative incision infection; no other surgery-related complications occurred in the remaining patients. Conclusion:One-stage anterior debridement and bone graft fusion can correct kyphosis, restore normal alignment, and improve neurological function in the treatment of single and double segment cervical pyogenic spondylodiscitis, representing a viable treatment option for cervical pyogenic spondylodiscitis.

Objective:To investigate the efficacy of one-stage anterior debridement and bone graft fusion for the treatment of cervical pyogenic spondylodiscitis.Methods:This is a retrospective case series study. Retrospective analysis of clinical data from 23 patients with cervical pyogenic spondylodiscitis treated with one-stage anterior approach debridement and bone graft fusion was performed in the Department of Spinal Surgery, the First Affiliated Hospital of Xinjiang Medical University from January 2015 to January 2020. There were 14 males and 9 females,aged (51.9±12.8) years (range:26 to 82 years). Preoperatively, 14 patients had neurological deficits, classified according to the American Spinal Injury Association (ASIA) impairment scale as follows: grade A in 1 case, grade B in 1 case, grade C in 5 cases, and grade D in 7 cases . All patients underwent the one-stage anterior debridement and fusion procedure. The surgical time, blood loss, hospital stay, fusion time, and surgical complications were documented. Clinical efficacy was assessed using the visual analogue scale (VAS), the neck disability index (NDI), and the ASIA impairment scale. Preoperative and postoperative data were compared using paired sample t tests, repeated measures analysis of variance, and generalized estimating equations. Results:All the 23 patients underwent the operative procedures successfully. The operation time was (102.8±19.8) minutes (range:60 to 140 minutes), blood loss was (84.4±40.2) ml (range:30 to 160 ml), and the length of hospital stay was (17.4±6.0) days (range:10 to 30 days). Blood cultures were positive for the causative pathogen in 14 cases (60.8% positivity rate), while 9 cases had negative results. Irrigation fluid cultures yielded the causative pathogen in 19 cases (82.6% positivity rate), with 4 cases negative. All patients were followed up for more than 12 months, with a follow-up duration of (19.0±5.9) months (range:12 to 36 months). At the final follow-up, VAS improved from (5.9±1.1) points preoperatively to (0.8±0.3) points; NDI improved from (38.3±6.0)% preoperatively to (9.3±3.0)%, with statistically significant differences (both P<0.01). All patients experienced improvement in neurological function, with the exception of one patient in grade C and two in grade D, all other patients recovered to grade E. The C 2-7 Cobb angle and the affected segment Cobb angle were corrected. white blood cell, erythrocyte sedimentation rate and C-reactive protein levels returned to normal. All patients achieved bony fusion, with a fusion time of (8.9±1.9) months (range:6 to 12 months). Two diabetic patients developed postoperative incision infection; no other surgery-related complications occurred in the remaining patients. Conclusion:One-stage anterior debridement and bone graft fusion can correct kyphosis, restore normal alignment, and improve neurological function in the treatment of single and double segment cervical pyogenic spondylodiscitis, representing a viable treatment option for cervical pyogenic spondylodiscitis.

Objective:To investigate the clinical features and effect of prognostic factors in patients with different pathological types of non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis.Methods:We collected and analyzed the clinical data of 89 patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis who were treated at Huadong Hospital from March 2013 to May 2020. The data were analyzed via log-rank and Cox multivariate analyses.Results:The median overall survival time of the 89 cases was 10.2 months. Patients with B-cell lymphoma-associated hemophagocytic lymphohistiocytosis did not reach the median overall survival time. The median overall survival times of T-cell lymphoma-associated hemophagocytic lymphohistiocytosis and NK-cell lymphoma-associated hemophagocytic lymphohistiocytosis were 10.2 and 3.0 months, respectively. The pathological type of non-Hodgkin lymphoma (OS: P=0041, PFS: P=0.015) , ECOG score ≥ 3 (OS: P=0.031, PFS: P=0.030) , hematopoietic stem cell transplantation (OS: P=0.005, PFS: P=0.040) , lymphadenopathy (OS: P=0.007, PFS: P=0.012) , and splenomegaly (OS: P=0.276, PFS: P=0.324) were related to the overall survival and progression-free survival of patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis. Splenectomy could improve the prognosis of patients with lymphoma-associated hemophagocytic lymphohistiocytosis, especially T-cell lymphoma-associated hemophagocytic lymphohistiocytosis. Conclusion:The clinical characteristics of patients with different pathological types of non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis were similar but were different in the overall survival rate and the effect of prognostic factors. We suggested that patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis should receive more than combined chemotherapy. To improve the prognosis and survival rate of patients, those with B-cell lymphoma-associated hemophagocytic lymphohistiocytosis and NK-cell lymphoma-associated hemophagocytic lymphohistiocytosis promptly require hematopoietic stem cell transplantation. Moreover, patients with T-cell lymphoma-associated hemophagocytic lymphohistiocytosis should consider splenectomy.

Objective:To explore the dynamic changes of thrombosbondin-1(TSP-1)and transforming growth factor beta(TGF-β)in patients who underwent hepatectomy and their clinical value in predicting post-hepatectomy liver failure(PHLF).Methods:Clinical data of 113 patients who received hepatectomy in the First Affiliated Hospital of Xinjiang Medical University from Janu-ary 2016 to March 2019 were retrospectively analyzed.The cohort comprised 59 males and 54 fe-males,with an average age of(41.00±13.98)years.According to the standard of the International Study Group of Liver Surgery(ISGLS),TSP-1 was divided into the PHLF group(n=40)and the non-PHLF group(n=73).The dynamic changes of plasma TSP-1 and TGF-β plasma levels during peri-operative period were observed.The definition and diagnostic criteria suggested by ISGLS was used to evaluate PHLF.The value of plasma TSP-1 and TGF-β level in predicting PHLF were ana-lyzed by calculating areas under the receiver operating characteristic curves(AUCs).Patients were divided into TSP-1 high group and TSP-1 low groups based upon the ROC cutoff values and their correlation with PHLF was analyzed.T test and Mann-Whitney rank sum test were performed to compare the TSP-1 and TGF-β levels between the two groups.The rate comparison was carried out by Chi-square test or Fisher's exact test.Results:In this study,40 patients experienced vary-ing degrees of PHLF.The plasma levels of TSP-1 on postoperative day I(POD1)and day 7(POD 7)in the PHLF group were significantly higher than those in the non-PHLF group(P＜0.05,P＜0.01,re-spectively).The area under the ROC curve of plasma TSP-1 level on postoperative 1 d in the diag-nosis of PHLF was 0.725,with sensitivity of 0.864 and specificity of 0.647.The area under the ROC curve of plasma TSP-1 level on postoperative 7 d in the diagnosis of PHLF was 0.81,with sensitiv-ity of 0.818 and specificity of 0.765.Conclusion:The incidence of PHLF is related to the extent of liver resection,Child-Pugh grade and TSP-1 levels on postoperative day 1.The concentration level of TSP-1 on POD land 7 might be utilized as an effective marker for predicting PHLF.