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Objective To compare the early efficacy and safety of Da Vinci robot-assisted Kasai versus traditional open surgery for biliary atresia (BA). Methods From January 2021 to June 2022, retrospective analysis was performed for 9 children undergoing robotic portoenterostomy (RP) and another 21 children open portoenterostomy (OP) within the same period. General profiles, perioperative biochemical parameters, postoperative complications and hospitalization stay were compared. Results In RP group, there were 8 boys and 1 girl with a body weight of 5.4(5.0-6.5) kg and an average age of 65(56-90) day; In OP group, 13 boys and 8 girls with a body weight of 4.9 (4.5-5.8) kg and an average age of 59(49, 85) day. No significant differences existed among the above parameters (P>0.05). In RP and OP groups, preoperative total bilirubin [179.6(116.2-224.5) mmol/L vs. 170.3(129.6-208.2) mmol/L], postoperative total bilirubin [115.0(69.3-143.4) mmol/L vs. 120.3(88.5-160.7) mmol/L], preoperative direct bilirubin[83.4(79.0-155.7) mmol/L vs. 97.6(82.5-147.4) mmol/L], postoperative direct bilirubin [71.6(47.6-100.2) mmol/L vs. 82.1(64.3-117.5) mmol/L], preoperative alanine aminotransferase [175(114-234) U/L vs. 147(94-218) U/L], postoperative alanine aminotransferase [144(119-310) U/L vs. 183(150-253) U/L], preoperative aspartate aminotransferase [212(164-164) U/L vs. 129 (96-157) U/L] and postoperative aspartate aminotransferase [167(90-191) U/L vs. 120 (80-155) U/L]. There were no differences of statistical significance (P>0.05). Compared with those before operation, the biochemical indexes of children in all groups after operation were decreased except for the increase of alanine aminotransferase after operation in OP group, with statistical significance (P < 0.05). Postoperative hospitalization stay [15(12.5-21.5) day vs. 15(12-17.5) day], postoperative complication rate (2/9 vs. 3/21), cholangitis rate (7/9 vs. 15/21) and jaundice clearance (3/9 vs. 12/21) showed no inter-group differences of statistical significance (P>0.05). Conclusions Early curative efficacy of Da Vinci robotic surgery and open surgery is comparable for BA. [ABSTRACT FROM AUTHOR]

Objective To explore the middle/long-term native liver survival and examine its influencing factors of Kasai portoenterostomy for type III biliary atresia (BA). Methods From January 2011 to December 2020, single-center retrospective analysis was performed for 234 type III BA children undergoing Kasai portoenterostomy. Gender, operative age, liver function test, blood routine test, cytomegalovirus (CMV) IgM examination and aspartate aminotransferase-to-platelet ratio index (APRi) were recorded. Operative age ≤ eerativewas defined as early operation group while operative age > 60 days as late operation group. IgM antibodies against CMV were detected by immunoenzyme-linked reaction. They were assigned into two groups of IgM positive and IgM negative according to the detection results. Ratio index of aspartate aminotransferase-to-platelet = AST/(up- per limit of AST) x 100/PLT(109/L). Aspartate aminotransferase and platelets were measured at the same time 1 week pre-Kasai. They were divided into two groups of APRi < 0.6 and APRi of. Total bilirubin ≤ 2 mg/ dL. being more than once post-Kasai within 6 mouths was defined as jaundice-eliminating group and the rest as jaundice group. And 5/10-year native liver survival was calculated by Kaplan Meier method. Univariate analysis was performed by Log rank test and multibariate analysis by Cox regression. Results There were 118 boy and 116 girls with an average operative age of (72.7 ± 23) days. The 5/10-year native liver survival rate was 41.2% and 38.9% respectively. Univariate Kaplan-Meier liver survival and Log Rank test analysis revealed that gender (x² = 0.01,P=0.91), CMV (x² = 1.3,P = 0.25) and APRi (x² = 0,P = 0.98) had no effect on native liver survival. Compared with late operative age group, native liver survival rate spiked markedly in early operative age group (x² =7.6,P=0.006). Compared with total bilirubin level greater than or equal to 2 mg/dL, native liver survival rate rose significantly in < 2 mg/dL of total bilirubin (x² = 74.6,P<0.001). Multivariate Cox regression analysis showed that only total bilirubin < 2 mg/dl. within 6 months post-Kasai was an independent influencing factor of middle/long-term liver survival. Conclusions Total bilirubin < 2 mg/dL within 6 months after Kasai portoenterostomy is an independent influencing factor of middle/long-term liver survival. [ABSTRACT FROM AUTHOR]

Neonatal biliary atresia is one of the most common fatal diseases in neonates, with higher incidence rates in Asia than in other parts of the world. Early detection and treatment of neonatal biliary atresia are crucial, yet the lack of professional pediatricians and auxiliary diagnostic and treatment methods can cause parents to miss the best treatment window. To address this issue, this paper develops a predictive algorithm with practical application value that uses neonatal stool pictures to predict whether the newborn has neonatal biliary atresia and reminds parents to visit a doctor in time. To achieve higher recognition rates in practical scenarios, the algorithm in this paper is developed based on a real-scene dataset of newborn fecal images. First, we designed a self-attention network model BANet (Biliary Atresia Network), which will combine shallow features and deep features of pictures to get better classification. To address issues as dark or overexposed images, we developed an automatic brightness adjust-ment algorithm by analyzing the brightness distribution of the dataset. Furthermore, we added a shadow data enhancement method duiring training to mitigate the inference of shadows on recognition results. In order to verify the effectiveness of the algorithm proposed in this paper, we design a comparison test with doctors. The results show that BANet out performed doctors in objective evaluation indicators such as the recognition rate of four classifications, the recognition rate of two classifications, specificity and sensitivity. The proposed BANet can effectively use the color, abnormal points and other information in the picture, by compensating the brightness of the picture, the accuracy and robustness of the overall algorithm are improved and good results have been achieved in practical application scenarios. [ABSTRACT FROM AUTHOR]

Objective To explore the effect of total cholesterol (TC) on bile duct injury in children with biliary atresia (BA) and investigate the immunological mechanism. Methods Peripheral blood TC levels were retrospectively collected from 118 BA children diagnosed by intraoperative cholangiography,80 infants of the same age with infantile hepatitis syndrome ( IHS) and 165 normal control (NC) children of the same age without any evidence of hepatopathology and liver function abnormalities admitted to Guangzhou Women and Children's Medical Center during the period of 2011 - 2020. The correlation between peripheral blood TC and various indicators of bile duct injury or multiple immune cells associated with BA formation was statistically analyzed respectively. Cholesterol was added into the co-culture system of CD177+ neutrophils and biliary epithelial cells ( BECs) in vitro, and its effect on BEC apoptosis was observed. Simultaneously, the changes of related indicators in CD177+ neutrophils after cholesterol stimulation were observed. BA children were divided into high-cholesterol BA group and normal-cholesterol BA group according to the detection value of peripheral blood TC. Through RNA sequencing analysis, the differences in gene expression of CD177+ neutrophils between the high-cholesterol BA group and the normal-cholesterol BA group were compared. Results The peripheral blood TC level of [ 4. 9 ( 4. 0, 6. 1)] mmoVL in BA children was higher than that of [ 3. 7 (3. 1, 4. 1)] mmoVL in NC group and [ 4. 2 ( 3. 3, 5. 4) ] mmoVL in IHS children, and the difference was statistically significant ( P < 0. 05). The peripheral blood TC level of [ 4. 9( 4. 0, 6. 1)] mmoVL in BA children was positively correlated with gamma-glutamyltransferase ( GGT) level of [ 497. O( 298. 5, 843.0) ] U/L, an indicator of bile duct injury ( r = 0. 401, P < 0. 05), as well as positively correlated with direct bilirubin ( DBIL) and total bile acid (TBA) ( r = 0. 338, P < 0. 05 ; r = 0. 235, P < 0. 05). In terms of the proportion of apoptotic cholangiocytes in liver tissue, the high-cholesterol BA group was 0. 724 ±0. 146, the normal-cholesterol BA group was 0. 232 ±0. 141, the NC group ( children with portal vein cavernous transformation of similar age) was 0. 242 ± 0. 228. There was a statistically significant difference between high-cholesterol BA group and the other two groups ( P < 0. 05 ). Peripheral blood TC content [ 4. 4 ( 3. 1, 5. 5) ] mmoVL in BA children was positively correlated with peripheral blood CD177+ neutrophil ratio ( 26. 5 ± 6. 4) % ( r = 0. 503, P < 0. 05), while there was no significant correlation between TC and peripheral blood monocytes [ 1. 0 ( 0. 8, 1. 2) ] X 109 /L or neutrophils ( 3. 2 ± 1. 4) X 109 /L ( P > 0. 05). The peripheral blood TC level of BA children ( 4. 7 ± 1. 6) mmoVL was significantly positively correlated with the number of peripheral blood T cells ( 4 952. 1 ± 1 985. 0) cells/µL( r = 0. 488, P <0. 05) and had no correlation with the number of peripheral blood B cells [971. 0( 664. 8,1 636. 1)] cells/µL (P > 0. 05). Compared with the normal-cholesterol BA group, the expression of various mitochondrial oxidative phosphorylation-related genes ( CYBB, CALR, MT-CYB, MT-ND5, MT-ND]) was significantly up-regulated in high-cholesterol BA group, and the difference was statistically significant ( P < 0. 05 ). Under cholesterol stimulation, the mean fluorescence intensity ( MFI) of TOMM20 and DNA 8-OHdG, the indicators of mitochondrial oxidative phosphorylation, were 73. 47 ± 6. 36 and 159. 07 ± 10. 17, respectively, and the differences were statistically significant as compared with those before stimulation (P <0. 05). The CD177+ neutrophil extracellular trap indicators, MPO and H2B were 60. 51 ±4. 26 and 157. 46 ±9. 40 respectively, and the differences were statistically significant (P <0. 05) as compared with those before stimulation. Meanwhile, in the co-culture system of CD177+ neutrophils and BECs, the viability of BECs after cholesterol stimulation was statistically significantly different from that before stimulation ( P < 0. 05). Conclusion Mitochondrial oxidative phosphorylation in CD177+ neutrophils is up-regulated by cholesterol in BA, promoting the release of neutrophil extracellular traps and causing bile duct injury. [ABSTRACT FROM AUTHOR]

Copyright of China Tropical Medicine is the property of China Tropical Medicine Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To investigate the expression of CD163 in children with biliary atresia (BA) and its relationship with liver fibrosis. Methods A total of 30 children with BA (the BA group, 14 patients with liver fibrosis grade Ⅰ-Ⅱ, 16 patients with liver fibrosis grade Ⅲ-Ⅳ) and 10 children with choledochal cyst (the CC group) were collected. Samples of liver tissue and blood were obtained from the 2 groups. The expression levels of CD163 in liver tissue and blood of the two groups were detected by immunohistochemistry and flow cytometry. The expression of sCD163 was detected by enzyme-linked immunosorbent assay (ELISA), and the value of sCD163 in evaluating BA liver fibrosis was analyzed by receiver operating characteristic curve (ROC). Results Immunohistochemical staining and flow cytometry indicated that CD163 expression levels in liver tissue and blood were significantly higher in the BA group than those in the CC group (P＜0.01), and which increased with the increased severity of liver fibrosis (P＜0.01). The expression of sCD163 also showed the same change. sCD163 predicted BA with an area under the ROC curve of 0.927, a best threshold value of 8.323 µg/L, a sensitivity of 86.67% and a specificity of 100%. Conclusion The expression of CD163 is up-regulated in children with BA, which accelerates the process of liver fibrosis. sCD163 can accurately reflect the severity of liver fibrosis in children with BA. [ABSTRACT FROM AUTHOR]

Objective To explore the application value of ultrasonic measurement of hilar cyst size in differentiating between cystic biliary atresia (CBA) and congenital biliary dilatation (CBD). Methods Retrospective review was conducted for clinical data of 34 hospitalized children with hilar cysts aged < 150 days from January 2013 to January 2022.According to the findings of intraoperative cholangiography, they were assigned into two groups of CBA (n=14) and CBD (n=20).General profiles, biochemical parameters, preoperative ultrasonic characteristics, cholangiographic and intraoperative findings between two groups were compared. And receiver operating characteristic curve (ROC) was plotted for examining the diagnostic value of CBA. Results The serum levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBIL) and direct bilirubin (DBIL) differed significantly between two groups (P < 0.05).ROC results indicated that maximal cyst width had the highest diagnostic efficiency. Optimal cut-off value was 2.4 cm, area under the ROC curve (AUC) 0.98 and sensitivity and specificity were 91.0% and 95.2% respectively. Optimal cutoff value of maximal cyst length was 3.1 cm, AUC 0.95 and sensitivity and specificity were 91.0% and 85.2% respectively. Optimal cutoff value for maximal cyst length was 0.7 cm, AUC 0.95 and sensitivity and specificity were 91.0% and 89.6% respectively. Optimal cutoff value of maximum cyst width was 2.3 cm, AUC 0.92 and sensitivity and specificity were 80.2% and 85.9% respectively. Optimal cutoff value of maximum cyst length was 3.1 cm, AUC 0.89 and sensitivity and specificity were 90.2% and 78.8% respectively. Conclusion Ultrasonic measurement of maximal cyst width may aid in diagnosing CBA in jaundiced children with postnatal hilar cyst. If ultrasonic examination reveals that cystic width is≤2.3 cm, CBA highly probable. Early cholangiography and surgery are necessary. [ABSTRACT FROM AUTHOR]

Objective To explore the risk factors and outcomes of recurrent cholangitis after Kasai in biliary atresia (BA) patients. Methods Retrospective review was conducted for clinical and follow-up data of 60 BA children with cholangitis after Kasai procedure from January 2018 to September 2021.According to whether the frequency of cholangitis episodes was more than or equal to 3 times in half a year, they were divided into two groups of recurrent cholangitis (n=20) and occasional cholangitis (n=40).Gender, preoperative body mass index (BMI), cytomegalovirus (CMV) infection, age at Kasai surgery, liver fibrosis grade, serum levels of liver function parameters at preoperation and Day 14 postoperation and the occurrence of early cholangitis were compared between two groups.Cox regression analysis was performed for estimating the risk factors of recurrent cholangitis.Postoperative jaundice clearance (JC) rate and postoperative native liver survival rate were also compared between two groups. Results No significant differences existed in gender, preoperative BMI, CMV infection, age at Kasai or liver fibrosis grade (P>0.05).Serum total bile acid level at preoperation was significantly lower in recurrent cholangitis group than that in occasional cholangitis group (Z=-2.070, P < 0.05).Alanine transaminase(ALT)[(169.47±93.53)U/L], Aspartate trans aminase(AST)[(173.96±96.25)U/L], [242.50(169.68, 296.13)ratio 244.50(148.50, 297.00)U/L], gamma-glutamyl transferase(GGT)[572.00(310.75, 895.75)than 388.00(245.50, 695.25)U/L], total bilirubin, (219.61±73.08)ratio(207.38±58.63)μmol/L), direct bilirubin(DBIL)ratio(122.70(104.50, 163.25)ratio 116.70(81.42, 158.35)μmol/L]and albumin, Alb[(36.27±2.91)vs.(37.88±4.90)g/L]at preoperation between 2 groups had no significant difference(P>0.05).ALT[(211.23±144.87)to(229.87±104.76)U/L]and AST[162.50(108.17, 225.75)to 196.50(160.75, 235.43)U/L], GGT[(742.85±438.09)vs.(584.05±385.31)U/L], TBIL[136.20(86.05, 190.23)vs.145.50(95.73, 213.90)μmol/L], DBIL[79.85(60.88, 122.83)vs.90.00(61.30, 129.30)μmol/L]and Alb[(37.92±5.55)vs.(39.22±4.55)g/L]at day 14 postoperation between 2 groups were not significantly different(P>0.05).The incidence of early cholangitis was significantly higher in recurrent cholangitis group than that in occasional cholangitis group (χ2=9.909, P < 0.05).Cox regression analysis revealed that early cholangitis was risk factor for recurrent cholangitis; JC rate of recurrent cholangitis group was lower than that of occasional cholangitis group at Month 3/6 postoperation.However, the difference was not statistically significant (P>0.05).JC rate at Month 12 postoperation was significantly lower in recurrent cholangitis group than that in occasional cholangitis group (χ2=5.179, P < 0.05).No significant inter-group difference existed in native liver survival rate at 1 year postoperation (P>0.05). Conclusion Early cholangitis is an independent risk factor for recurrent cholangitis after Kasai in BA children.And recurrent cholangitis affects the postoperative jaundice clearance. [ABSTRACT FROM AUTHOR]

Objective To explore the correlation between age of Kasai portoenterostomy and early prognosis to provide references for timing of Kasai surgery in biliary atresia (BA) children. Methods Retrospective review was conducted for clinical data of hospitalized BA children from January 2019 to December 2021.General profiles (gender & operative age) and parameters of liver function, coagulation function and blood ammonia were collected. According to the jaundice clearance at 3 months post-operation, they were assigned into two groups of non-jaundice clearance (n=47) and jaundice clearance (n=66).The cut-off value of operative age was calculated. Then two groups were divided according to operative age. The prognostic parameters of Kasai portoenterostomy at 3 months post-operation were compared, including alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST), direct bilirubin (DBIL), postoperative DBIL/ preoperative DBIL, gamma-glutamyltransferase (GGT), total bile acid (TBA), total bilirubin (TBIL), platelet count (PLT), aspartate aminotransferase-to-platelet ratio index (APRI) and international normalized ratio (INR). Results A total of 113 cases were collected, including 49 males and 64 females. The operative age was 59.00 (40.50, 69.00) days. Univariate analysis of the preoperative indexes of the non-jaundice clearance group and the jaundice clearance group showed that the age of operation(64 (52, 79) d vs. 55 (33, 67) d), AST (202.00 (138.00, 332.00) U/L vs. 147.50 (100.00, 242.00) U/L), indirect bilirubin (IBIL) ((70.60±20.31)μmol/L vs. (79.96±27.13)μmol/L), total protein(TP) ((55.88±6.59)g/L vs. (53.13±4.71)g/L) between the two groups were statistically significant (P < 0.05). The indexes with P < 0.1 in univariate analysis [age of operation, albumin (ALB), ALT, AST, IBIL, TP, activated partial thromboplastin time(APTT)] were included in the multivariate Logistic regression analysis, and the results showed that the age of operation (OR=0.974, 95%CI: 0.951-0.997, P=0.030) was an independent factor influencing the jnundice clearance at 3 months after Kasai protoentrostomy. According to the cut-off value (46.5d) of the operative age group, the results showed that the levels of ALP(365.00 (316.00, 514.75) U/L vs. 492.00 (343.00, 684.00) U/L), ALT ((76.35±81.33) U/L vs. (134.09±97.45)U/L), AST (67.50 (41.50, 99.50) U/L vs. 112.00 (79.00, 168.00) U/L), DBIL (2.55 (1.78, 6.95) μmol/L vs. 9.80 (3.60, 36.80) μmol/L), postoperative DBIL/ preoperative DBIL(0.05 (0.03, 0.09) vs. 0.12 (0.05, 0.54)), TBA (45.50 (25.93, 92.93) μmol/L vs.106.20 (41.20, 187.50) μmol/L), TBIL (9.65 (6.98, 15.43) μmol/L vs. 21.50 (10.40, 66.90) μmol/L) and APRI (0.32 (0.22, 0.48) vs.0.62 (0.41, 0.93)) in the operation age ≤46.5 d group were significantly lower than those in the operation age > 46.5 d group, the difference was statistically significant (P < 0.05). Conclusion Early prognostic parameters are superior in group with operative age≤46.5 days to group with operative age >46.5 days. And early Kasai portoenterostomy may improve the prognosis of BA. [ABSTRACT FROM AUTHOR]

As a serious disease of hepatobiliary system in neonates and infants, biliary atresia (BA) is characterized by progressive inflammation and fibrosis of intra-and extrahepatic bile ducts. Kasai operation has remained a preferred treatment option. However, around 60% of children with postoperative liver fibrosis may progress further and eventually liver transplantation is often required for saving lives. Our current understandings of the etiology and pathogenesis of BA are insufficient. And there is no proper way of preventing postoperative complications. Though as a subject of much controversy, Laparoscopic Kasai procedure has achieved satisfactory outcomes. This review focused upon current hot issues of researchers to provide rationales for future researches of BA. [ABSTRACT FROM AUTHOR]

Through continuous explorations of pediatric surgeons in different countries, biliary atresia (BA) has gradually shifted from a deadly disease to one effectively managed by palliative surgery or liver transplantation. It is imperative for clinicians to standardize the diagnostic and therapeutic procedures of BA children to smoothly transition from children to adults. As promulgated by Japanese Biliary Atresia Society (JBAS) in 2021, English version of the Guidelines was elaborated for serving as references for managing BA children in China. [ABSTRACT FROM AUTHOR]

Objective To explore the impact of cytomegalovirus (CMV) infection on the prognosis of Kasai operation in children with biliary atresia (BA). Methods From January 2018 to December 2020, 91 BA children undergoing Kasai operation at Tianjin Children's Hospital and Shanxi Children's Hospital were divided into two groups of CMV positive (n=35) and CMV negative (n=56).CMV positive children were divided into antiviral treatment and non-treated groups. Follow-ups were conducted for (2-34) months until October 10, 2021.The impact of CMV infection on jaundice clearance, cholangitis and autologous liver survival after Kasai operation were examined. Results In CMV positive group, there were 26 cases in treatment group and 9 cases in non-treated group. Operative age was significantly older in positive group. Significant difference existed in proportion of white blood cell (WBC).In positive group, aspartate aminotransferase (AST) spiked sharply[244.00(195.00, 341.00) vs. 185.00(126.25, 281.00), P < 0.05].No significant difference existed in jaundice clearance rate, incidence of cholangitis or survival of autologous liver after Kasai operation. The incidence rate of early cholangitis declined markedly after antiviral treatment(11.54% vs. 55.56%, P < 0.05). Conclusion CMV infection has no significant impact on the prognosis of BA children. And antiviral therapy lowers the incidence of early cholangitis. It hints that antiviral therapy has a positive effect on BA children with CMV infection. [ABSTRACT FROM AUTHOR]

Objective To evaluate the efficacy of currently popular Nomogram prediction model for biliary atresia (BA) through a single-center data model. Methods Retrospective review was preformed for 631 children undergoing surgical explorations for infantile obstructive jaundice from 2009 to 2020.According to the findings of intraoperative cholangiography, they were assigned into two groups of BA (n=500) and non-BA (n=131).Gender, body weight, ALP (alkaline phosphatase), GGT (gamma glutamyl transferase) and DB (direct bilirubin) were input into the model for estimating the predicted risk probability of BA in the same child. They were grouped according to operative year and day age at surgery. The predicted probability of BA and other liver function parameters were plotted as receiver operating characteristic curve (ROC) curve in the same coordinate system. And area under the curve (AUC), sensitivity and specificity for diagnosing BA were obtained. Also the accuracy of model for an early diagnosis of BA was evaluated. Results Nomogram predictive model had higher AUC value and sensitivity for diagnosing BA than single parameter diagnosis. However, AUC value for diagnosing BA in children aged≤50 day or > 80 day was lower than that for GGT alone. Thus the prediction of pediatric BA using this model might not be accurate enough. Conclusion Nomogram predictive model has higher AUC value and sensitivity for diagnosing BA than single parameter diagnosis. However, AUC value for diagnosing BA in children aged≤50 day or > 80 day is lower than that for GGT alone. [ABSTRACT FROM AUTHOR]

Biliary atresia is a rare congenital disease of children with unknown pathogenesis and biliary obstruction, which is mainly manifested with progressive inflammation and fibrous obstruction of the biliary system inside and outside the liver, and subsequently develop into cholestatic cirrhosis and liver failure. Although biliary atresia is rare, it is the most common cause of cholestasis during the infancy period. If surgical treatment were not performed (such as Kasai operation and liver transplantation), children may die in a short period of time. Kasai operation can improve biliary drainage, whereas it fails to change the outcomes of children with biliary atresia. Most of them will still suffer from persistent liver injury due to cholestasis, and eventually require liver transplantation. At present, there is no consensus on whether Kasai operation should be performed prior to liver transplantation. In this article, research progress on the diagnosis and surgical treatment of biliary atresia was reviewed, aiming to provide reference for clinical diagnosis and treatment of biliary atresia and improve the survival of children with biliary atresia. [ABSTRACT FROM AUTHOR]

Copyright of Chinese Journal of Contemporary Pediatrics is the property of Xiangya Medical Periodical Press and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Imaging Science & Photochemistry is the property of Imaging Science & Photochemistry Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Imaging Science & Photochemistry is the property of Imaging Science & Photochemistry Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

For children with biliary atresia, early diagnosis and timely surgical intervention are of great importance to improve the survival rate of autologous liver. With reference to the authors' clinical experience and recent research advances, this article briefly reviews the methods for rapid diagnosis of biliary atresia and analyzes the advantages and disadvantages of different diagnostic method, so as to provide a reference for the establishment of clinical diagnostic thinking. [ABSTRACT FROM AUTHOR]

Cholangitis is among the most common complication after Kasai procedure in children with biliary atresia, which has great impact on the quality of life and native liver survival of children. The liver condition during Kasai operation can affect the postoperative bile drainage. The incidence of cholangitis after Kasai operation is associated with bile drainage. The proliferation of bacteria within the bile conduit of jejunum due to the change of intestinal environment maybe the main cause of cholangitis. Intestinal content reflux after Kasai operation may also induce the occurrence of cholangitis. In addition to the direct retrograde infection, enteric bacteria can also break through the intestinal mucosa and enter the liver through the portal vein to induce cholangitis. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is characterized by progressive inflammatory obstruction of intrahepatic and extrahepatic bile ducts and hepatic fibrosis, which is one of the common causes of obstructive jaundice in infants. Kasai surgery is the first choice for children with BA. Timely and effectively relieving inflammatory obstruction of bile duct and curbing liver fibrosis are the key to prolong or realize autologous liver survival in children with BA. GATA binding protein 6 (GATA6) has the function of regulating transcriptional activity. Studies have shown that it can inhibit the activation of hepatic stellate cells (HSCs) by regulating cell proliferation and differentiation, up-regulating the expression of ferritin and participating in mitogen-activated protein kinase-dependent signal pathway, promote HSCs senescence, reduce liver fibrosis, and play a certain role in the process of liver repair after BA liver injury. This article intends to review the relationship between GATA6 and BA liver fibrosis from the aspects of functional characteristics and pathways of action. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a progressive disease involving both intrahepatic and extrahepatic bile ducts. Kasai portoenterostomy is the most widely used treatment strategy for BA. However, about 60% of the patients treated by Kasai operation have poor postoperative outcomes and still need liver transplantation in the future. Although the factors affecting the postoperative survival of children with BA are closely related to the BA classification, the surgical method (laparoscopic or traditional open), the age at the time of surgery, and liver lesions at the time of surgery, the adjuvant therapy after BA is still an area that can be improved to a great extent. In recent years, there have been many reports on postoperative BA, such as hormones, bile acid metabolism drugs, antibiotics and probiotics, etc. In this paper, the research progress of adjuvant therapy after BA is reviewed to further explore the prognostic value of adjuvant therapy in children with BA. [ABSTRACT FROM AUTHOR]

Objective To investigate the expression levels of glypican 3 (GPC3) and its association with liver fibrosis in children with biliary atresia (BA). Methods A total of 41 infants with BA (BA group), 9 infants with choledochal cyst (CC group) and 12 healthy infants with normal liver function and no other hepatobiliary diseases by physical examination (NC group) were collected. Serum GPC3 levels were measured by enzyme-linked immunosorbent assay (ELISA) in the 3 groups. The receiver operating characteristic (ROC) curve was used to evaluate the auxiliary diagnostic value of GPC3 for BA. Liver tissue samples from the BA group and the CC group were retained to analyze the relationship between GPC3 and the grade of liver fibrosis in children with BA by measuring the expression levels of GPC3 by immunohistochemical staining and qPCR. Results (1) GPC3 levels were significantly higher in the BA group than those in the CC group and the NC group (χ2= 24.170, P＜0.01). There were no significant differences in the serum levels of GPC3 in children aged≤30 d (n = 21) and children aged＞30 d (n = 20) in the BA group, but which were higher than those in the CC group and the NC group (χ2= 24.210, P＜0.01). There were no significant differences in GPC3 levels between grades I to II and grades III to IV children in the BA group, but which were higher than those in the CC group and the NC group (χ2=24.390, P＜0.01). The area under the ROC curve (AUC) of GPC3 for the diagnosis of BA was 0.878 (95%CI: 0.792-0.965, P＜0.01), with a sensitivity of 82.93%, a specificity of 80.95%, and an optimal cut-off value of 0.639 μg/L. (2) The results of liver tissue immunohistochemistry showed that GPC3 expression in liver tissue was significantly increased in the BA group compared to that of the CC group (Z=3.565, P＜0.01), and the content of GPC3 in liver tissue was positively correlated with the grade of liver fibrosis (rs=0.619, P＜0.01). The GPC3 expression was upregulated in the BA group compared to that of the CC group (Z=7.361, P＜0.05). Conclusion The serum GPC3 has clinical value in the diagnosis and differential diagnosis of BA. GPC3 expression is upregulated in BA liver tissue, and its expression level is positively correlated with the grade of liver fibrosis in biliary atresia [ABSTRACT FROM AUTHOR]

Objective To study the effects of portoenterostomy (Kasai surgery) on liver transplantation (LT) for children with biliary atresia (BA). Methods The clinical data of 880 children who received liver transplantation for BA in our hospital were retrospectively analyzed. According to whether Kasai surgery was performed before liver transplantation, they were divided into the Kasai group (n=542) and the non-Kasai group (n=338). The differences in general information, incidence of postoperative complications, graft survival rate and patient survival rate were compared between the two groups. Results The months of age at the time of transplantation, height, body weight, graft cold ischemia time, graft quality and operation time of children were higher or longer in Kasai group than those in non-Kasai group, while the graft-recipient mass ratio, preoperative Child score and pediatric model of end-stage liver disease (PELD) score were lower in the Kasai group than those in the non-Kasai group (P＜0.05). There were no significant differences in gender, graft hot ischemia time, blood loss/body weight, intraoperative blood transfusion volume, duration of no-liver phase, duration of ventilator use, ICU hospitalization and postoperative hospitalization duration between the two groups. There were no significant differences in the outflow tract obstruction after operation, portal vein anastomotic stenosis, portal vein thrombosis, hepatic artery thrombosis, biliary leakage, biliary tract stenosis, lymphatic leakage, gastrointestinal fistula, intestinal obstruction, acute rejection, cytomegalovirus and Epstein-Barr virus infection between the two groups. There were no significant differences in graft survival rate and cumulative survival rate at 1 and 5 years after operation between the two groups. Conclusion Liver transplantation is a safe and effective surgical method for the treatment of children with BA. Kasai surgery can postpone the time of liver transplantation in children, and have no effects on liver transplantation, postoperative graft survival, survival rate of children and the occurrence of major postoperative complications. [ABSTRACT FROM AUTHOR]

Objective To investigate the expression level of interleukin-8 (IL-8) in children with biliary atresia (BA) and its relationship with cholangitis after Kasai procedure, and to explore the upstream signaling pathway regulating IL-8. Methods (1) A total of 33 children with BA (18 cases with cholangitis and 15 cases without cholangitis after Kasai procedure), 6 children with biliary dilatation and 36 healthy children were included in this study. Serum samples were collected from children with BA and healthy infants. The serum expression levels of IL-8 and epidermal growth factor (EGF) were determined by enzyme-linked immunosorbent assay (ELISA). The activation of extracellular signal-regulated kinase 1/2 (ERK1/2) was detected by immunohistochemical staining in liver tissue samples from 6 children with BA and 6 children with biliary dilatation. (2) Human normal hepatocytes L-02 were cultured in vitro, and treated with phosphate buffer (PBS) and 25, 50 and 100 μg/L EGF for 24 h. The level of IL-8 was detected by quantitative polymerase chain reaction (qPCR). Cell lines HIBEpic, L-02 and LX-2 were treated with PBS and 100 μg/L EGF for 24 h, and the levels of IL-8 were detected by qPCR. L-02 cells were treated with 100 μg/L EGF and/or 5 mmol/L ERK pathway inhibitor KO-947, and the activation of ERK1/2 and the expression level of IL-8 were detected. Results (1) The serum level of IL-8 was significantly higher in the BA group than that in the normal control group (P＜0.01), and the serum level of IL-8 was significantly higher in the cholangitis group than that in the non-cholangitis group (P＜0.01). Serum EGF was positively correlated with IL-8 in the BA group (rs=0.422, P＜0.01). Immunohistochemical staining showed that p-ERK1/2 was highly expressed in liver tissues of BA group. (2) The IL-8 expression in L-02 cells increased in a concentration-dependent manner after PBS and 25, 50, 100 μg/L EGF intervention. There were no significant differences in the expression levels of IL-8 in HIBEpic and LX-2 cells after 100 μg/L EGF treatment (P＞0.05), but the expression of L-8 was increased in L-02 cells (P＜0.01). EGF at 100 μg/L also induced the expression of p-ERK1/2 in L-02 cells, and KO-947 could reverse the changes. Conclusion The serum IL-8 expression is significantly increased in children with BA before surgery, which is closely related to the postoperative cholangitis of Kasai. EGF could promote the expression of IL-8 in hepatocytes through ERK1/2 signaling pathway. [ABSTRACT FROM AUTHOR]

Objective To investigate the value of gamma-glutamyltransferase (GGT) combined with direct bilirubin (DBIL) and aspartate aminotransferase-to-platelet ratio index (APRI) in the diagnosis of biliary atresia (BA). Methods The clinical data of 283 cases of infantile cholestatic hepatopathy were retrospectively analyzed. A total of 138 cases of BA confirmed by biliary exploration, cholangiography and liver biopsy were included in the BA group, and 145 cases of jaundice eliminated during operation or subsided after medical treatment were included in the non-BA group. The general information and the results of the first simultaneous hematological examination were collected, including age, sex, body weight, GGT, DBIL, indirect bilirubin (IBIL), total bile acid (TBA), aspartate aminotransferase (AST), alanine aminotransferase (ALT) and platelet count (PLT). The APRI was calculated. ROC curve was used to evaluate the diagnostic value of each index for BA. Results Values of body weight, GGT, TBA, DBIL, IBIL, AST, ALT and APRI were significantly higher in the BA group than those in the non-BA group (P＜0.05). ROC curve showed that GGT had the largest area under the curve (AUC) for BA diagnosis, which was 0.845 (95%CI: 0.801-0.890). When GGT was 264 U/L, its sensitivity and specificity were 0.732 and 0.814, respectively. The AUC of GGT+DBIL and GGT+APRI for BA were 0.880 (95%CI: 0.840-0.919) and 0.871 (95%CI: 0.830-0.912), respectively. Conclusion GGT has better diagnostic value for BA, and GGT combined with DBIL and APRI has better diagnostic value than GGT alone. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

To evaluate the effect of liver transplantation on intestinal microflora in children with biliary atresia. Methods The fecal samples and liver function indexes of 16 children with biliary atresia before and 6 months after liver transplantation were collected, and 10 healthy children were selected as the healthy controls. DNA extraction and metagenome sequencing were carried out in the fecal samples. Statistical analysis was performed by software packages, such as R language. The changes of species structure and functional composition of intestinal microflora after liver transplantation were analyzed. The recovery of intestinal microflora in children with biliary atresia after liver transplantation was assessed. The relationship between intestinal microflora and liver function indexes was investigated. Results Following liver transplantation, the number of species of intestinal microflora in children with biliary atresia was increased. The opportunistic pathogens were the dominant species of intestinal microflora in children with biliary atresia before liver transplantation. The abundance of opportunistic pathogens was decreased and the abundance of shortchain fatty acid-producing bacteria was increased after liver transplantation (all P<0.05). Following liver transplantation, lipid metabolism, amino acid metabolism, carbohydrate metabolism, energy metabolism, metabolism of cofactors and vitamins were enhanced, whereas infectious diseases of bacterial, immune diseases and drug resistance were weakened. Compared with the healthy control group, there were no statistically significant differences in the diversity and structure of intestinal microflora in the post-liver transplant group, but different species were observed between two groups. The liver function indexes of children with biliary atresia after liver transplantation tended to decline (all P<0.000 1). The abundance of beneficial intestinal microflora was negatively correlated with liver function indexes, whereas the abundance of opportunistic pathogens was positively correlated with liver function indexes (all P<0.05). Conclusions Liver transplantation may significantly improve the structure and functional composition of intestinal microflora in children with biliary atresia. [ABSTRACT FROM AUTHOR]

Copyright of Imaging Science & Photochemistry is the property of Imaging Science & Photochemistry Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Imaging Science & Photochemistry is the property of Imaging Science & Photochemistry Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective To explore the mechanism of BMP-9 in biliary atresia (BA) liver fibrosis. Methods Fourteen samples of BA cases were selected as the BA group and 5 samples of congenital biliary dilatation (CBD) cases were selected as the CBD group. Liver fibrosis was evaluated by HE staining, immunohistochemical staining was used to detect the expression levels of BMP-9 and p-SMAD1/5, and qPCR was used to detect the expression levels of BMP-9 and ID1 mRNA in liver. Human hepatic stellate cells (LX-2) were cultured and treated with rTGF-β1 and different concentrations of rBMP-9. The protein expression levels of α-SMA, SMAD1/5, p-SMAD1/5 and ID1 in cells were detected by Western blot assay, and the mRNA expressions of α-SMA and ID1 in cells were detected by qPCR. Results The expression levels of BMP-9 and p-SMAD1/5 proteins, BMP-9 and ID1 mRNA in liver were higher in the BA group than those of the CBD group. In the BA group, the expressions levels of BMP-9 protein, BMP-9 and ID1 mRNA were significantly higher in children with moderate and severe liver fibrosis than those in children with mild liver fibrosis (P＜0.05). After treatment with rTGF-β1 and rBMP-9, the expression levels of α-SMA protein and α-SMA mRNA were increased in LX-2 cells (P＜0.05). The expressions of p-SMAD1/5, SMAD1/5, ID1, α-SMA protein and ID1, α-SMA mRNA in the downstream pathway were increased after adding different concentrations of rBMP-9 in LX-2 cells compared with those of 0 μg/L group (P＜0.05). Conclusion The expression levels of BMP-9, p-SMAD1/5 and ID1 in the liver of BA children are increased than those of CBD children, which are positively correlated with the degree of liver fibrosis. BMP-9 can activate LX-2 cells through SMAD/ID1 signaling pathway and promote the progress of fibrosis. [ABSTRACT FROM AUTHOR]

Objective: To study the relationship between serum glypican 3 (GPC3), transforming factor β1 (TGF-β1) and vascular endothelial growth factor (VEGF) levels in children with biliary atresia (BA) and liver stiffness and liver function . Methods: 62 children with BA who underwent Kasai surgery in our hospital from March 2016 to March 2019 were selected as the study subjects (BA group), and were divided into children with jaundice (total bilirubin) according to their total bilirubin levels. Children ≥34.2μmol/L) and without jaundice (total bilirubin<34.2μmol/L); 50 healthy children who received physical examination in our hospital during the same period were selected as the control group. The serum GPC3, TGF-β1, VEGF and liver function indexes albumin (ALB), alanine aminotransferase (ALT), aspartate aminotransferase (AST ), γ-glutamyl transpeptidase (GGT) levels and liver stiffness values, using Pearson correlation to analyze the correlation between serum GPC3, TGF-β1 and VEGF levels, liver stiffness values ​​and liver function. Results: The serum GPC3, TGF-β1, VEGF, ALT, AST, GGT levels and liver stiffness values ​​of children in the BA group were significantly higher than those of the control group (P<0.05), and there was no significant difference in serum ALB levels compared with the control group ( P>0.05); the serum GPC3, TGF-β1, VEGF, ALT, AST, GGT levels and liver hardness values ​​of children with jaundice in the BA group were significantly higher than those of children without jaundice, and the serum ALB level was significantly lower than that of children without jaundice (P<0.05); serum GPC3, TGF-β1, VEGF levels in the BA group were positively correlated with serum ALT, AST, GGT levels and liver stiffness values ​​(P<0.05), and negatively correlated with serum ALB levels (P<0.05) . Conclusion: The increase of GPC3, TGF-β1 and VEGF in children with BA is related to the progression of liver fibrosis and the destruction of liver function. It may become a target for studying the mechanism of liver fibrosis after BA in the future. [ABSTRACT FROM AUTHOR]

Objective To investigate the ultrasonographic features and its diagnostic value in portal vein stenosis (PVS) after pediatric liver transplantation. Methods Clinical data of 84 pediatric recipients undergoing liver transplantation who were followed up by routine ultrasound were retrospectively analyzed. According to ultrasound and digital subtraction angiography (DSA) results, all recipients were divided into the normal group (n=57) and PVS group (n=27). The incidence of PVS was assessed by ultrasound. The measurement parameters consisted of diameter of portal vein anastomosis, flow velocity of portal vein anastomosis, hepatic artery velocity, resistance index (RI) of hepatic artery and maximum diameter of the spleen, etc. The ultrasound parameters were statistically compared between the PVS and normal groups. The diagnostic value of ultrasound parameters for PVS after pediatric liver transplantation was evaluated. Results The diameter of portal vein anastomosis in the normal group was significantly larger than that in the PVS group[(0.44±0.08) cm vs. (0.27±0.10) cm], and the flow velocity of portal vein anastomosis in normal group was significantly lower than in the PVS group[(43±12) cm/s vs. (119±58) cm/s] (both P<0.001). The hepatic artery velocity, RI of hepatic artery and maximum diameter of the spleen did not significantly differ between two groups (all P>0.05). The diameter of portal vein anastomosis for the optimal diagnosis of PVS in pediatric liver transplantation, pediatric liver transplantation from organ donation after citizen's death and living-related donor pediatric liver transplantation was 0.35 cm, 0.35 cm and 0.33 cm, respectively. The corresponding area under curve (AUC) was 0.906, 0.916 and 0.906, the sensitivity was 0.947, 0.951 and 0.938, and the specificity was 0.852, 0.833 and 0.889, respectively. The flow velocity of portal vein anastomosis for the optimal diagnosis of PVS was 62.7 cm/s, 69.6 cm/s and 61.2 cm/s. The AUC was 0.990, 0.993 and 1.000, the sensitivity was 1.000, 1.000 and 1.000, and the specificity was 0.930, 0.951 and 1.000. Conclusions Ultrasound features of the pediatric recipients with PVS after liver transplantation include the smaller diameter of portal vein anastomosis and faster anastomotic flow velocity, which possess high diagnostic value. [ABSTRACT FROM AUTHOR]

Objective To investigate the changes and significance of serum γ-glutamyltranspeptidase (GGT) and direct bilirubin (DB) in children with biliary atresia and cholestasis. Methods Eighty children with biliary atresia (biliary atresia group) and 80 children with cholestasis (cholestasis group) were selected, and meanwhile, 50 healthy iufants were randomly chosen as the control group. The serum GGT and DB levels were detected by enzyme-linked immunosorbent assay. Pearson product moment correlation was used to analyze the correlation between serum GGT, DB and total bilirubin (TB), total bile acid (TBA), alanine aminotransferase (ALT), and aspartate aminotransferase (AST). Receiver operating characteristic (ROC) curve was drawn to evaluate the value of serum GGT and DB in the differential diagnosis of biliary atresia and cholestasis. Results Serum GGT, DB, TB, TBA, ALT, and AST in the biliary atresia group and choles tasis group were higher than those in the control group (all P < 0. 05), and the above indexes in the biliary atresia group were higher than those in the cholestasis group (all P < 0. 05). Pearson product moment correlation analysis showed that the serum GGT and DB were positively correlated with TB, TBA, ALT and AST in children with biliary atresia and cholestasis (all P < 0. 05). ROC curve analysis showed that serum GGT and DB had differential diagnosis value for biliary atresia and cholestasis, and the combined detection had higher differential diagnosis value, with the AUC of 0. 915 (95% CI : 0. 870 - 0. 945), the sensitivity, specificity, and accuracy of 91. 25%, 86. 25%, and 88. 75%, respectively. Conclusion Serum GGT and DB levels in children with biliary atresia and cholestasis increase, especially in children with biliary atresia; early combined detection can assist the clinical differential diagnosis of the two diseases. [ABSTRACT FROM AUTHOR]

Objective: To investigate the expression of transforming growth factor-1 (transforming growth factor-β1, TGF-β1) and leukocyte differentiation antigen 34 (cluster of differentiation, CD34) in the liver tissues of children with biliary atresia and the effect of TGF-茁1 and CD34 in liver fibrosis, and to verify the diagnostic value of serum concentration of TGF-β1 in liver fibrosis. Methods: 66 children with biliary atresia admitted to our hospital from April 2016 to January 2019 were selected as the research group, and 30 children with biliary dilatation as the control group. The immunohistochemical detection results of TGF-β1 and CD34 in liver tissues of the two groups were then compared, the expression of TGF-β1 and CD34 in children with different grades of liver fibrosis in research group and the correlation between TGF-β1 or CD34 with the grade of liver fibrosis were respectively detected. The serum TGF-β1 concentration of the two groups were compared, the serum TGF-β1 concentration in children with different grades of liver fibrosis in research group and the correlation between it with the grade of liver fibrosis were detected, and at last the diagnostic value of serum TGF-β1 concentration in children with liver fibrosis caused by biliary atresia were measured by ROC curve. Results: (s): The TGF-β1 IOD and expression area of CD34 in liver tissues of the research group were significantly higher than those of the control group (P<0.05). The positive expression rates of TGF-β1 or CD34 increased with the aggravation of liver fibrosis in the research group (P<0.05), and respectively had positive correlation with the degree of liver fibrosis(ρ=0.594 and P<0.05, ρ=0.616 and P<0.05). The serum concentration of TGF-β1 in the research group was significantly higher than that of the control group (P<0.05), increased with the aggravation of liver fibrosis in the research group (P<0.05), and had positive correlation with the degree of liver fibrosis (ρ=0.944 and P<0.05). The serum concentration of TGF-β1 had diagnostic significance for liver fibrosis in children with biliary atresia (AUC=0.917, P<0.001), and the best diagnostic threshold is 436.4 ng/L, of which the sensitivity is 0.879 and the specificity is 0.867. : The expression of TGF-β1 and CD34 is significantly increased in liver tissues of children with biliary atresia, which suggest that inhibiting the expression of TGF-β1 or CD34 may prevent or delay the further development of liver fibrosis induced by biliary atresia. The serum concentration of TGF-β1 can be used to diagnose the liver fibrosis caused by biliary atresia in children. [ABSTRACT FROM AUTHOR]

To explore the relationship between the expressions of T lymphocyte surface antigens (CD4 and CD8)，macrophage-specific antigen (CD68) and Kasai postoperative cholangitis. Methods Twenty-seven children with biliary atresia who underwent liver transplantation after Kasai operation were divided into frequent cholangitis group (10 cases), early cholangitis group (7 cases) and non-cholangitis group (10 cases) according to the previous outbreak of cholangitis. The general clinical data were compared between groups. The hilar liver tissues of each group were stained with HE to observe the degree of liver fibrosis, bile duct hyperplasia, inflammatory cell infiltration and bile plug. Immunohistochemical staining was used to detect the expressions of CD4, CD8 and CD68 proteins. Results There were no significant differences in sex, age, age of Kasai operation, native liver survival rate, white blood cells, C-reactive protein and liver function before liver transplantation between the three groups. HE staining showed that there were no significant differences in liver fibrosis, biliary duct hyperplasia and bile plug between the three groups. But inflammatory cell infiltration was more serious in the confluent area of the frequent cholangitis group than that in the non-cholangitis group and the early cholangitis group. Immunohistochemical staining showed that no significant difference in CD4 protein expression between the three groups. The expressions of CD8 and CD68 proteins were significantly higher in frequent cholangitis group than those in the non-cholangitis group and the early cholangitis group (P＜0.05). Conclusion The main pathological changes of hepatic cholangitis after Kasai operation are inflammatory cell infiltration in portal area, which are an inflammatory reaction involving CD8+ T cells and CD68+ macrophages. The increased expressions of CD8 and CD68 may be the risk factors of the recurrence of cholangitis. [ABSTRACT FROM AUTHOR]

Objective To analyze the clinical characteristics, pathogenic causes and therapeutic experience of right diaphragmatic hernia after pediatric living donor liver transplantation. Methods Clinical data of 3 recipients with right diaphragmatic hernia after pediatric living donor liver transplantation were retrospectively analyzed. The clinical characteristics, diagnosis and treatment process and therapeutic experience were analyzed and summarized. Results The primary diseases of 3 children with diaphragmatic hernia after living donor liver transplantation were biliary atresia. The diaphragmatic hernia occurred at 4-6 months after liver transplantation. The contents of diaphragmatic hernia included the intraperitoneal and interperitoneal tissues and organs. Diaphragmatic defects were all located in the posterior medial area of the right diaphragm. The primary stage intermittently suturing repair was performed during intraoperative period. No diaphragmatic hernia recurred during long-term follow-up. Conclusions The clinical manifestations of right diaphragmatic hernia after pediatric living donor liver transplantation are diverse. The risk factors include malnutrition, low body weight, surgical trauma, chemical erosion caused by bile leakage, focal infection and pleural-peritoneal pressure gradient, etc. Surgical intervention is the preferred treatment strategy for diaphragmatic hernia after liver transplantation. [ABSTRACT FROM AUTHOR]

Biliary atresia is a kind of progressive obstruction of intrahepatic and extrahepatic bile ducts, which can lead to cholestatic cirrhosis and liver failure. It is one of the most important digestive surgical diseases in the field of pediatric surgery. After the diagnosis of biliary atresia, Kasai operation should be completed as soon as possible. If the window period is exceeded, severe cirrhosis will inevitably occur. In the special period of outbreak, unnecessary exposure should be minimized for children. Preoperative screening and postoperative follow-up can be conducted through wechat or official hospital network. During surgery for patients with NCP, the prevention and control rules framed by the local health administration or local medical institution should be strictly followed. All members of the surgical team must function in a coordinated fashion, class 2 or 3 prevention measures should be provided to all personnel. The operation should be performed in a negative-pressure operating room or infection room. If the local hospital does not have the above capability, the patients with NCP requiring surgery should be transferred immediately to a hospital with appropriate services. [ABSTRACT FROM AUTHOR]

bjective To investigate the value of liver stiffness measurement( LSM) by transient elastography,aspartate aminotransferase-to-platelet ratio index( APRI),and fibrosis-4( FIB-4) in the diagnosis of liver fibrosis in children with biliary atresia. Methods A total of 110 children with biliary atresia who underwent Kasai operation in Department of Neonatal Surgery,Hunan Children’s Hospital,from January 1,2016 to December 31,2018 were enrolled. Liver biopsy specimens and related clinical data were collected,including routine blood test results,liver function,and transient elastography results. The chi-square test was used for comparison of categorical data between groups. The Kruskal-Wallis H test was used for comparison of non-normally distributed continuous data. MedCalc software was used to plot the receiver operating characteristic( ROC) curve,and the ROC curve was used to evaluate the diagnostic efficiency of LSM,APRI,and FIB-4 in evaluating the degree of liver fibrosis. The Spearman correlation analysis was also performed. Results The ROC curve analysis showed that in the diagnosis of biliary atresia with marked liver fibrosis( F≥2),LSM had an area under the ROC curve( AUC) of 0. 874( 95% confidence interval [CI]: 0. 778-0. 970) at the cut-off value of 9. 250 kPa,APRI had an AUC of 0. 636( 95% CI: 0. 362-0. 911) at the cut-off value of 0. 680,and FIB-4 had an AUC of 0. 622( 95% CI: 0. 363-0. 880) at the cut-off value of 0. 047; in the diagnosis of biliary atresia with progressive liver fibrosis( F≥3),LSM,APRI,and FIB-4 had an AUC of 0. 781( 95% CI: 0. 689-0. 873),0. 519( 95% CI: 0. 401-0. 636),and 0. 506( 95% CI: 0. 389-0. 623),respectively,at the cut-off value of 10. 75 k Pa,0. 70,and 0. 05,respectively; in the diagnosis of biliary atresia with liver cirrhosis( F≥4),LSM,APRI,and FIB-4 had an AUC of0. 855( 95% CI: 0. 769-0. 942),0. 701( 95% CI: 0. 599-0. 803),and 0. 717( 95% CI: 0. 609-0. 825),respectively,at the cut-off value of 11. 85 kPa,0. 82,and 0. 09,respectively. The correlation analysis showed that LSM was positively correlated with aspartate aminotransferase level( r = 0. 258,P = 0. 007) and was negatively correlated with platelet count( r =-0. 248,P = 0. 009). Conclusion Transient elastography has a high accuracy in determining the grade of liver fibrosis in children with biliary atresia,with a higher clinical value than APRI and FIB-4 in evaluating the degree of liver fibrosis. [ABSTRACT FROM AUTHOR]

Objective To study the value of serum gamma-glutamyl transpeptidase (GGT) combined with direct bilirubin (DB) in the diagnosis of biliary atresia. Methods A total of 667 infants with cholestasis who were hospitalized and treated from July 2010 to December 2018 were enrolled as subjects. According to the results of intraoperative cholangiography and follow-up, they were divided into biliary atresia group with 234 infants and cholestasis group with 433 infants. The two groups were compared in terms of age of onset, sex, and serum levels of total bilirubin (TB), DB, alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bile acid (TBA), and GGT. A receiver operating characteristic (ROC) curve analysis was performed for indices with statistical significance, and the area under the ROC curve (AUC) and the optimal cut-off value for diagnosis were calculated. Results The biliary atresia group had a significantly younger age of onset than the cholestasis group (P<0.001). There were no significant differences in sex, ALT, and AST between the two groups (P>0.05), while the biliary atresia group had significantly higher serum levels of TB, DB, TBA, and GGT than the cholestasis group (P<0.05). GGT combined with DB had the highest AUC of 0.892 (95% confidence interval: 0.868-0.916) in the diagnosis of biliary atresia. At the optimal cut-off values of 324.0 U/L for GGT and 115.1 μmmol/L for DB, GGT combined with DB had a sensitivity of 79.8% and a specificity of 83.2% in the diagnosis of biliary atresia. Conclusions GGT combined with DB has high sensitivity and specificity in the diagnosis of biliary atresia and can be used as an effective indicator for diagnosis of biliary atresia in infants. [ABSTRACT FROM AUTHOR]

BACKGROUND: Biliary atresia is not suitable as a method for early diagnosis because it mainly depends on intraoperative diagnosis, and the operation is complicated, and there is a great injury to the child. Therefore, finding simple and effective blood biochemical diagnostic indicators will improve early diagnosis efficiency.OBJECTIVE: To measure the plasma alpha-fetoprotein level in children with biliary atresia and to investigate the application value of plasma alpha-fetoprotein level and blood biochemical indicators.METHODS: Blood samples were harvested from 48 infants with biliary atresia, 20 infants with neonatal hepatitis, and 9 healthy controls.Plasma alpha-fetoprotein level was measured by enzyme-linked immunosorbent assay. The correlation between plasma alpha-fetoprotein level and blood biochemical indicators was analyzed to establish a potential diagnosis model. This study was approved by the Ethics Committee of Xinhua Hospital, China(approval No. XHEC-D-2013-026).RESULTS AND CONCLUSION: Plasma alpha-fetoprotein level in infants with biliary atresia and neonatal hepatitis was significantly higher than that in the healthy controls(P < 0.001), but no significant difference in plasma alpha-fetoprotein level was observed between infants with biliary atresia and neonatal hepatitis(P > 0.05). There was no significant difference in plasma alpha-fetoprotein level between infants with different stages of cirrhosis(P > 0.05). Blood biochemical indexes indicated severe liver injury in infants with biliary atresia and neonatal hepatitis. There were significant differences in plasma prealbumin(P < 0.05) and γ-glutaminyl transpeptidase levels between infants with biliary atresia and neonatal hepatitis(P < 0.01). Plasma alpha-fetoprotein level was negatively correlated with γ-glutaminyl transpeptidase level(r =-0.516, P < 0.01). A potential diagnosis model was established based on plasma alpha-fetoprotein, prealbumin,γ-glutaminyl transpeptidase levels, age and sex of infant patients, with a sensitivity of 81.82%, specificity of 91.67%, and area under the curve of 0.939. Results suggest that plasma alpha-fetoprotein level increased in infants with biliary atresia. Establishing a diagnosis model based on blood biochemical indicators such as plasma alpha-fetoprotein is of certain reference value for clinical diagnosis of biliary atresia. [ABSTRACT FROM AUTHOR]

Objective To analyze the relationship between bile ductal plate malformation (DPM) and autologous liver survival in children with biliary atresia (BA). Methods A total of 40 BA children underwent Kasai operation in Tianjin Children’s Hospital and liver transplantation (LT) in Tianjin First Central Hospital from January 2008 to December 2017 were collected. Hepatic tissue samples at the time of Kasai operation and liver transplantation were stained with HE and CK19 immunohistochemistry. According to the existence of DPM in different periods during Kasai operation, patients were divided into without DPM group (n=27) and with DPM group (n=13), and without DPM group (n=17) and with DPM group (n= 23) during liver transplantation. The differences of autologous liver survival time were compared between different stage groups of children. Results The autologous liver survival time was 230.0 (190.5, 276.5) d and 336.0 (218.0, 757.0) d respectively in BA children with and without DPM during Kasai operation. The autologous liver survival time was 230.0 (185.0, 285.0) d and 527.0 (288.0, 817.0) d respectively in BA children with and without DPM during liver transplantation. The autologous liver survival time was significantly shorter in patients with DPM than that in patients without DPM either during Kasai operation or LT (P＜0.05). Conclusion Children with biliary atresia associated with bile ductal plate malformation can shorten their autologous liver survival time. [ABSTRACT FROM AUTHOR]

Objective To explore the influencing factors of the survival time of autologous liver after Kasai procedure for biliary atresia(BA), and to find out the risk factors of early liver transplantation, so as to provide data of evidence-based medicine for BA treatment. Methods Data of 167 BA children underwent liver transplantation due to hepatic failure after Kasai procedure were retrospectively analyzed. According to the operation interval between Kasai procedure and liver transplantation, they were divided into G1 (≤6 months), G2 (＞6 months - 2 years) and G3 (>2 years) groups. Data of patient gender, patient age of Kasai procedure, jaundice clearance, cholangitis after Kasai procedure and liver function indexes before liver transplantation were compared between three groups. The χ2 test, variance analysis, Kruskal-Wallis H test and multiple logistic regression analysis were used to find out the influencing factors for the survival time of autologous liver, that is, the influencing factors of early liver transplantation. Results There were no significant differences in gender and age of Kasai procedure between the three groups. There was statistical difference in the clearance of jaundice between the three groups (P＜0.01), showing a gradually increase from G1 to G3. There was significant difference in the early cholangitis between the three groups (P＜0.05), which was lower in G3 group than that of G1 group. There was significant difference in the late cholangitis after surgery between the three groups (P＜0.01), which was higher in G2 group than that of G1 group. There was statistical difference in the incidence of frequent cholangitis between the three groups (P＜0.05), which was lower in G3 group than that of G2 group. Multiple logistic regression analysis showed that persistent jaundice was the risk factor for native liver survival after Kasai procedure. Conclusion Persistent jaundice and early cholangitis after Kasai procedure can reduce the native liver survival time, which is related to the early liver transplantation. Persistent jaundice is a risk factor for early liver transplantation after Kasai procedure. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a common cause of obstructive jaundice in the infantile period, and also a severe hepatobiliary disease leading to liver failure. At present, the research on the etiology and pathogenesis of BA is continuing. But various signs show that perinatal viral infection plays an important role in the occurrence and development of BA, which may be the exact cause of BA. Therefore, this paper mainly discussed the feasibility of preventing BA from the view of virus vaccination, in order to provide a new perspective for reducing the incidence of BA. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a serious disease of the hepatobiliary system in infants and children, which quickly worses into irreversible liver fibrosis and eventually leads to hepatic failure and death. Controling the liver fibrosis of BA patients with BA timely and effectively is the key to prolong or achieve their survival with autologous liver. Exosomes, as nano-scale vesicles containing special lipids, proteins and nucleic acids, have certain physiological and pathological functions, and regarded as a new way of communication between cells. In recent years, with increasing the research about exosomes, their function of regulating liver fibrosis of BA has been gradually concerned. And exosomes affect the fibrosis process as a carrier of the intercellular signal transmission: transfering or influencing fiber connective tissue growth factor or transforming growth factor beta 1, promoting the secretion of IL-17 indirectly, participating in Notch and Hedgehog (Hh) pathway relating to the process of liver fibrosis and regulating hepatic stellate cells’migration. In particular, it was reported that exosomes from adipose tissue-derived mesenchymal stem cells and human umbilical cord mesenchymal stem cells play a role in inhibiting liver fibrosis in vitro. This paper aims to review the relationship between exosomes and liver fibrosis of BA and provide a new direction for clinical therapy. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a severe hepatobiliary disease in the infantile period characterized by progressive inflammation of intrahepatic and extrahepatic bile duct and fibrous obstruction, which leads to cholestasis and progressive liver fibrosis. Current researches mainly focus on complication and native liver survival rate after Kasai procedure of BA children, while the value of nutrition condition of BA children in the perioperative period is always ignored. This article discussed the effect of nutrition condition on complication and native liver survival rate in the perioperative period after Kasai procedure and liver transplantation of BA children. We hope that it can be helpful to the clinical treatment of BA. [ABSTRACT FROM AUTHOR]

Objective To investigate the relationship of matrix metalloproteinase-7 (MMP-7) in liver tissue of biliary atresia (BA) with liver fibrosis and bile duct reaction, and explore the role of MMP-7 in the process of liver fibrosis and its clinical significance. Methods Data of 15 BA patients with liver biopsy in Kasai operation in our hospital from June 2017 to April 2018 were collected (G1 group)，and data of 41 patients with liver biopsy who underwent liver transplantation after Kasai operation from January 2015 to January 2016 were enrolled as transplantation group. The transplantation group was divided into two groups according to the interval time between Kasai operation and liver transplantation: G2 group (n=28, the interval time was less than 2 years) and G3 group (n=13, the interval time was more than 2 years). Hematoxylin-Eosin (HE) staining was used to observe the degree of liver fibrosis. Immunohistochemical staining was used to detect the expressions of MMP-7 and CK-19. Results (1) Immunohistochemical results showed that MMP-7 was mainly expressed in bile duct epithelial cells, hyperplastic bile ducts and hepatocytes around the portal area. With the progress of hepatic fibrosis, the expressions of MMP-7 were increased in biliary duct cells and hepatocytes, and which were strongly expressed in biliary duct cells than those of hepatocytes. Semi-quantitative analysis showed that the expression levels of MMP-7 were (0.068± 0.017) vs. (0.093±0.017) vs. (0.084±0.016) in the three groups (F=10.541，P＜0.001). CK-19 staining showed that bile duct reactions of three groups were (0.119±0.036) vs. (0.157±0.040) vs. (0.110±0.044) (F=7.296，P＜0.001). The expression levels of MMP-7 and CK-19 protein were significantly higher in G2 group than those of other two groups (P＜0.05). There was no significant difference between G1 and G3 groups (P＞0.05). (2) The liver fibrosis in the three groups were 2 (2, 3) vs. 4 (3, 4) vs. 4 (2, 4) (H=17.785, P＜0.001), and the fibrosis was significantly higher in the G2 group than that of other two groups (P＜ 0.017). (3) Correlation test showed that the expression of MMP-7 protein was positively correlated with the degree of liver fibrosis (rs=0.609, P＜0.001), but there was no correlation with bile duct reaction (r=0.007, P=0.962). Conclusion The expression of MMP-7 protein is highly expressed in liver tissue after Kasai operation, and it increases with the severity of liver fibrosis. MMP-7 may participate in the process of BA liver fibrosis and suggest that the survival of autologous liver is poor. [ABSTRACT FROM AUTHOR]

Objective: This study focuses on Na(+)-taurocholate cotransporting polypeptide (NTCP) deficiency to analyze and investigate the value of the serum bile acid profile for facilitating the diagnosis and differential diagnosis. Methods: Clinical data of 66 patients with cholestatic liver diseases (CLDs) diagnosed and treated in the Department of Pediatrics of the First Affiliated Hospital of Jinan University from early April 2015 to the end of December 2021 were collected, including 32 cases of NTCP deficiency (16 adults and 16 children), 16 cases of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), 8 cases of Alagille syndrome, and 10 cases of biliary atresia. At the same time, adult and pediatric healthy control groups (15 cases each) were established. The serum bile acid components of the study subjects were qualitatively and quantitatively analyzed by ultra-high performance liquid chromatography-tandem mass spectrometry. The data were plotted and compared using statistical SPSS 19.0 and GraphPad Prism 5.0 software. The clinical and bile acid profiles of children with NTCP deficiency and corresponding healthy controls, as well as differences between NTCP deficiency and other CLDs, were compared using statistical methods such as t-tests, Wilcoxon rank sum tests, and Kruskal-Wallis H tests. Results: Compared with the healthy control, the levels of total conjugated bile acids, total primary bile acids, total secondary bile acids, glycocholic acid, taurocholic acid, and glycochenodeoxycholic acid were increased in NTCP deficiency patients ( P < 0.05). Compared with adults with NTCP deficiency, the levels of total conjugated bile acids and total primary bile acids were significantly increased in children with NTCP deficiency ( P < 0.05). The serum levels of taurochenodeoxycholic acid, glycolithocholate, taurohyocholate, and tauro-α-muricholic acid were significantly increased in children with NTCP deficiency, but the bile acid levels such as glycodeoxycholic acid, glycolithocholate, and lithocholic acid were decreased ( P < 0.05). The serum levels of secondary bile acids such as lithocholic acid, deoxycholic acid, and hyodeoxycholic acid were significantly higher in children with NTCP deficiency than those in other CLD groups such as NICCD, Alagille syndrome, and biliary atresia ( P < 0.05). Total primary bile acids/total secondary bile acids, total conjugated bile acids/total unconjugated bile acids, taurocholic acid, serum taurodeoxycholic acid, and glycodeoxycholic acid effectively distinguished children with NTCP deficiency from other non-NTCP deficiency CLDs. Conclusion: This study confirms that serum bile acid profile analysis has an important reference value for facilitating the diagnosis and differential diagnosis of NTCP deficiency. Furthermore, it deepens the scientific understanding of the changing characteristics of serum bile acid profiles in patients with CLDs such as NTCP deficiency, provides a metabolomic basis for in-depth understanding of its pathogenesis, and provides clues and ideas for subsequent in-depth research.

Objective: To explore the clinical identification value of color doppler ultrasound in infant hepatitis syndrome (HS) and biliary atresia (BA). Methods: A total of 53 infants with HS (including 32 infants with cholestatic type HS, and 21 infants with icteric type HS) and 39 infants with BA, who were treated in Zigong Maternal and Child Health-Care Hospital of Sichuan Province from March 2014 to March 2017, were selected, and at the same time, 50 infants without jaundice disease were randomly chosen as control group. All the subjects were examined by color doppler ultrasound. Then the results(including liver and spleen size, shape, internal echo, detectable property of left and right hepatic duct and extrahepatic bile duct and changes of hemodynamic indexes) were compared among the children patients. Results: The normal shape rates of fasting gallbladder in cholestatic type HS group and icteric type HS group were higher than those in BA group, but the three groups were all lower than control group (all P<0.05). The average time of gallbladder contraction rate＞50% in cholestatic type HS group and icteric type HS group was shorter than that in the BA group, but the three groups were all longer than control group(all P<0.05). The detectable property rates of left and right hepatic duct and extrahepatic bile duct in cholestatic type HS group, icteric type HS group and control group were higher than those in BA group(P<0.01). The cystic echo and fibrous plaque rates of hepatic portal in cholestatic type HS group,icteric type HS group and control group were lower than those in BA group(P<0.05). The mean flow velocity of portal vein(VPV) level in BA group was lower than that in the other three groups,while its congestion index of PV(CIPV), anteroposterior diameter of hepatic artery(DHA), resistance index of HA(RIHA), flow velocity radio of HA/PV(A/P), and the RI of splenic artery (RISPA) were higher than those in the other three groups (all P<0.05). Conclusion: The changes of tissue pathology and hemodynamic indexes (VPV, CIPV, DHA, RIHA, A/P, RISPA) can be examined by color doppler ultrasound to identify infant HS and BA. [ABSTRACT FROM AUTHOR]