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Objective To investigate the long-term efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of primary biliary cholangitis (PBC) with portal hypertension. Methods A retrospective analysis was performed for 102 patients who received TIPS in Affiliated Drum Tower Hospital of Nanjing University Medical School from January 2015 to August 2021, and these patients were divided into PBC group with 41 patients and viral hepatitis cirrhosis group with 81 patients. Related indicators were collected, including routine blood test results, liver and renal function, coagulation function, portal vein thrombosis, hepatic encephalopathy, and etiology of TIPS treatment shortly after admission, preoperative portal venous pressure, and stents used in surgery, and Child-Pugh score was calculated. Follow-up data were collected and analyzed, including postoperative upper gastrointestinal rebleeding, stent dysfunction, hepatic encephalopathy, and the data on survival and prognosis. The independent samples t -test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of continuous data with skewed distribution between two groups; the chi-square test was used for comparison of categorical data between two groups. The Kaplan-Meier method was used for survival analysis, and the log-rank test was used for survival difference analysis. Results In the PBC group and the viral hepatitis cirrhosis group, the median percentage of reduction in portal venous pressure after surgery was 33.00% and 35.00%, respectively, and there was no significant difference between the two groups ( P > 0.05). At the end of follow-up, there were no significant differences between the PBC group and the viral hepatitis cirrhosis group in stent dysfunction rate (14.63% vs 24.69%, χ 2 =1.642, P > 0.05), upper gastrointestinal rebleeding rate (17.07% vs 24.69%, χ 2 =0.917, P > 0.05), the incidence rate of overt hepatic encephalopathy (12.20% vs 7.41%, χ 2 =0.289, P > 0.05), and disease-specific death rate (14.63% vs 9.88%, χ 2 =0.229, P > 0.05). Conclusion For PBC patients with portal hypertension, TIPS can achieve the same efficacy as the treatment of portal hypertension caused by viral hepatitis cirrhosis and can also effectively reduce portal hypertension without increasing the incidence rate of complications and disease-specific death rate. Therefore, it is a safe and effective treatment method.
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Primary biliary cholangitis (PBC) is a progressive cholestatic liver disease targeting biliary epithelial cells, and the concept of "treating diseases by intervening with the gut" has become a research hotspot in recent years. In the future, probiotics may be used to improve the abundance and distribution of intestinal flora, reshape the intestinal microenvironment, and intervene against the progression of PBC. This article reviews the gut microbiota and the clinical application of probiotics, so as to provide ideas for finding new treatment strategies for PBC.
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Primary biliary cirrhosis/cholangitis is an autoimmune disease. Renal tubular acidosis is a common form in PBC cases, but Fanconi syndrome is rarely reported. The paper reported a 66-year-old female patient with fatigue, renal insufficiency and elevated bile duct enzymes. The patient presented with type 2 proximal renal tubular acidosis and complete Fanconi syndrome. Laboratory examinations showed high-titer-positive anti-mitochondrial antibodies, elevated serum IgM, and type 3 cryoglobulinemia. Renal biopsy revealed interstitial nephritis, and electron micrographs showed abnormal mitochondria in proximal tubular epithelial cells. The patient's renal function ameliorated, and acid-base imbalance and electrolyte disturbances were corrected after high-dose glucocorticoid treatment.
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Objective:To compare the therapeutic effects of percutaneous transhepatic choledochoscopic lithotripsy (PTCSL) and traditional open hepatectomy (OH) on regional hepatolithiasis with biliary cirrhosis.Methods:From January 2020 to August 2022, 110 cases of regional hepatolithiasis complicated with biliary cirrhosis treated in the hepatology department of Hunan Provincial People′s Hospital were retrospectively collected. According to the surgical methods of treating hepatolithiasis, the patients were divided into minimally invasive group and laparotomy group. The minimally invasive group received PTCSL, and the laparotomy group received OH. The clinical data of the two groups were compared and analyzed, and the postoperative exhaust time, gastrointestinal function recovery time, operation time and intraoperative bleeding volume were observed. The levels of alanine aminotransferase (ALT), γ-glutamyltransferase(GGT) and aspartate aminotransferase (AST) before and after operation were compared between the two groups. The incidence of complications and stone removal rate of the two groups were recorded.Results:The postoperative exhaust time (11.12±2.09)h, gastrointestinal function recovery time (25.76±4.28)h, operation time (108.51±16.19)h, intraoperative blood loss (20.16±3.59)ml and postoperative exhaust time (29.35±4.83)h and gastrointestinal function recovery time in the minimally invasive group were less than those in the laparotomy group (36.91±6.35)h, operation time (116.27±21.54)h and intraoperative blood loss (38.03±6.22)ml (all P<0.05). The levels of ALT (77.82±16.25)U/L, GGT (248.16±24.83)U/L and AST (65.42±16.82)U/L in the minimally invasive group after operation were lower than those in the laparotomy group [ALT (102.37±25.64)U/L, GGT (345.45±32.60)U/L and AST (96.30±22.17)U/L] (all P<0.05). The incidence of postoperative complications was 7.27%(4/55) in the minimally invasive group and that in the laparotomy group was 29.09%(16/55), with statistically significant difference ( P<0.05). The stone removal rate was 61.82%(34/55) in the minimally invasive group and 92.73%(51/55) in the laparotomy group, with statistically significant difference ( P<0.05). Conclusions:PTCSL and OH are effective in the treatment of regional hepatolithiasis complicated with biliary cirrhosis. The traditional OH has a high stone removal rate, and PTCSL has little influence on liver function, small complication rate and fast postoperative recovery.
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Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease. This article summarizes and reviews the histopathological features of PBC and the role of pathological examination in the diagnosis and treatment of PBC, as well as the role of pathology in staging and prognosis, the diagnosis of atypical PBC and overlap syndrome, the analysis of reasons for poor response to ursodeoxycholic acid, and identification of diseases or exclusion of other comorbidities, so as to improve the awareness of the role of pathological examination in PBC among clinicians.
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ABSTRACT Objective: to assess the outcomes of our patients who were subjected to LT for iatrogenic bile duct injury. Methods: all patients who underwent LT for treatment of complications of biliary duct injury were included in the study. Medical records and study protocols of these patients were retrospectively analyzed to determine demographic and clinical characteristics, treatment, and outcome of the patients. Results: of a total of 846 liver transplants performed, 12 (1.4%) were due to iatrogenic bile duct injury: 10 (83.3%) occurred during cholecystectomy, 1 (8.3%) following chemoembolization, and 1 (8.3%) during laparotomy to control abdominal bleeding. Cholecystectomy was performed by open access in 8 patients and by laparoscopic access in two . There were 8 female (66.7%) and 4 male (33.3%) with a mean age of 50.6 ± 13.1 years (range 23 to 70 years). All transplants were performed with livers from cadaveric donors. The mean operative time was 558.2 ± 105.2 minutes (range, 400-782 minutes). Biliary reconstruction was performed with Roux-en-Y hepaticojejunostomy in 11 patients and choledochocholedochostomy in one. Seven patients died (58.3%) and five (41.7%) were alive during a mean followed up of 100 months (range 18 to 118 months). Conclusion: liver transplantation in patients with iatrogenic bile duct injury is a complex procedure with elevated morbimortality.
RESUMO Objetivo: avaliar os resultados dos nossos pacientes que foram submetidos a transplante hepático por lesão iatrogênica do ducto biliar. Métodos: todos os pacientes que foram submetidos a transplante hepático para tratamento de complicações da lesão do ducto biliar foram incluídos no estudo. Os prontuários e protocolos de estudo desses pacientes foram analisados retrospectivamente para determinar características demográficas e clínicas, tratamento e desfecho dos pacientes. Resultados: de um total de 846 transplantes hepáticos realizados, 12 (1,4%) foram por lesão iatrogênica de via biliar: 10 (83,3%) ocorreram durante colecistectomia, 1 (8,3%) após quimioembolização e 1 (8,3%) durante laparotomia para controle de sangramento abdominal. A colecistectomia foi realizada por via aberta em 8 pacientes e por via laparoscópica em dois. Haviam 8 mulheres (66,7%) e 4 homens (33,3%), com média de idade de 50,6 ± 13,1 anos (variação de 23 a 70 anos). Todos os transplantes foram realizados com fígados de doadores cadavéricos. O tempo operatório médio foi de 565,2 ± 106,2 minutos (variação de 400-782 minutos). A reconstrução biliar foi realizada com hepaticojejunostomia em Y de Roux em 11 pacientes e coledococoledocostomia em um. Sete pacientes morreram (58,3%) e cinco (41,7%) estavam vivos durante um seguimento médio de 100 meses (variação de 18 a 118 meses). Conclusão: o transplante hepático em pacientes com lesão iatrogênica das vias biliares é um procedimento complexo com elevada morbimortalidade.
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Sodium taurocholate cotransporting polypeptide (NTCP) is not only an important transporter for bile acid absorption into the liver, but also a functional receptor for HBV and HDV, and extensive studies have been performed for its structure, function, gene characteristics, and expression and regulation mechanisms. NTCP is also associated with chronic viral hepatitis, nonalcoholic fatty liver disease, liver fibrosis, primary biliary cholangitis, and hepatocellular carcinoma. This article elaborates on the role of NTCP in various hepatobiliary diseases, so as to provide new direction for the diagnosis and treatment of related diseases.
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Primary biliary cholangitis (PBC) is one of the autoimmune liver diseases, and most patients have no obvious clinical manifestations in the early stage and have reached the advanced stage when symptoms appear. Therefore, it is necessary to make a confirmed diagnosis and evaluate prognosis as early as possible. Anti-gp210 antibody is of great value in the diagnosis of PBC and the prediction of disease progression, and the detection of anti-gp210 antibody can help to optimize the PBC scoring system. This article reviews the production mechanism and detection value of anti-gp210 antibody in PBC.
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ABSTRACT Purpose: To delve into the influence of paeoniflorin (PA) on abating primary biliary cholangitis (PBC)-induced liver fibrosis and its causative role. Methods: Our team allocated the mice to control group, PA group, PBC group and PBC+PA group. We recorded the weight change of mice in each group. We used Masson staining for determining liver fibrosis, immunofluorescence staining for measuring tumor necrosis factor-α (TNF-α) expression, quantitative real-time polymerase chain reaction (qRT-PCR) for assaying related gene expression, as well as Western blot for testing related protein expression. Results: The weight of PBC model mice declined. Twenty-four weeks after modeling, the positive rate of anti-mitochondrial antibody-M2 (AMA-M2) in PBC mice reached 100%. Alkaline phosphatase (ALP), alanine aminotransferase (ALT), aspartate aminotransferase (AST), hydroxyproline (HYP), laminin (LN), procollagen type III (PC III), and malondialdehyde (MDA) contents saliently waxed (p<0.01). Meanwhile, superoxide dismutase (SOD) and glutathione peroxidase (GSH-px) activity patently waned (p<0.01). Liver fibrosis levels were flagrantly higher (p<0.01), and TNF-α, NOD-like receptor protein 3 (NLRP3), caspase-1, interleukin-18 (IL-18), and interleukin-1β (IL-1β) protein or gene expression were manifestly up-regulated (p<0.01). PA could restore the weight of PBC mice, strikingly restrain the positive expression of AMA-M2, and down-regulate serum ALP, ALT, AST, HYP, LN, PC III, MDA in PBC mice (p<0.01). PA could also significantly up-regulate SOD and GSH-px levels (p<0.01), down-regulate IL-1β, IL-18, caspase-1, NLRP3, and TNF-α protein or gene expression in PBC mice (p<0.01) and inhibit liver fibrosis levels (p<0.01). Conclusions: PA can reduce PBC-induced liver fibrosis in mice and may function by curbing the formation of NLRP3.
Subject(s)
Animals , Mice , Monoterpenes/pharmacology , NLR Family, Pyrin Domain-Containing 3 Protein/metabolism , Glucosides/pharmacology , Liver Cirrhosis/prevention & control , Liver Cirrhosis/drug therapy , Aspartate Aminotransferases , Liver/pathologyABSTRACT
Objective:To explore the application of ureteroscope combined with holmium laser in patients with hepatolithiasis complicated with biliary cirrhosis.Methods:The clinical data of 89 patients with hepatolithiasis complicated with biliary cirrhosis treated in the hepatobiliary department of Hunan Provincial People's Hospital from February 2014 to December 2019 were collected and divided into two groups: group A was routine operation group and group B was ureteroscopy and holmium laser technology group. The clinical data of the two groups were compared and analyzed respectively.Results:The operation time in group B was less than that in group A [ (302.6±96.7)min vs (349.2±105.6)min, P<0.05], with statistically significant difference; The amount of intraoperative bleeding in group B was less than that in group A [(227.0±197.3)ml vs (331.4 ± 277.3)ml, P<0.05], with statistically significant difference; The postoperative hospital stay in group B was shorter than that in group A[(11.5±4.1)d vs (13.8±5.1)d, P<0.05], with statistically significant difference; The incidence of postoperative complications in group B was lower than that in group A (2.44% vs 18.75%, P<0.05), with statistically significant difference. The liver resection rate in group B was lower than that in group A (29.27% vs 52.08%, P<0.05). Conclusions:Through the establishment of a reasonable access to the liver, the application of ureteroscope and holmium laser technology in patients with hepatolithiasis complicated with biliary cirrhosis can achieve better diagnosis and treatment results, and this technology can be gradually promoted and applied in the clinic.
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Dyslipidemia is one of the most common complications of primary biliary cholangitis (PBC). This article reviews the latest research on lipid profile, the risk of cardiovascular diseases, and treatment of PBC with hyperlipidemia. Different from other liver diseases, PBC with hyperlipidemia has a unique lipid profile, which changes dynamically with disease progression. It is generally not considered that there are increased risks of atherosclerosis and cardiovascular disease. For those who have indications for treatment, statins are recommended as the first choice. In the future, more in-depth systematic studies are needed to clarify its diagnosis, treatment, and management processes.
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MicroRNA(miRNA) affect various biological processes such as cell differentiation, proliferation, and apoptosis by inhibiting the translation of target genes after transcription and are widely involved in the regulation of immune and inflammatory responses in organisms. Autoimmune liver diseases are a group of chronic inflammatory diseases of the hepatobiliary system mediated by abnormal immunity, and abnormal immune inflammatory response of liver tissue with the involvement of miRNA is closely associated with the development and progression of autoimmune liver diseases. This article reviews the current research advances in miRNA in autoimmune liver diseases.
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The incidence rate of primary biliary cholangitis (PBC) is increasing year by year, but there is still no specific medicine at present and PBC has a complex pathogenesis. Kupffer cells, as the key cells involved in immunoregulation, play an important role in PBC. When hepatocytes are damaged, Kupffer cells will be activated and release a large amount of inflammatory cytokines and chemokines, which participate in the development and progression of PBC. This article briefly reviews the role of Kupffer cells in PBC, so as to provide a theoretical basis for Kupffer cells as a potential target for the treatment of PBC.
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Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease with unknown etiology, and patients with poor response to ursodeoxycholic acid and obeticholic acid may eventually progress to liver cirrhosis and even liver failure. Liver transplantation is the only effective treatment method for PBC at present. This article elaborates on liver transplantation, survival time after liver transplantation, complications, recurrence of PBC after liver transplantation, and prospects and challenges of liver transplantation in patients with PBC, so as to provide a reference for clinical outcome and treatment after liver transplantation for PBC.
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Primary biliary cholangitis (PBC) is a progressive intrahepatic cholestatic autoimmune disease, and pruritus can be seen in 60%-70% of PBC patients and seriously affects patients’ quality of life. Improving the symptoms of pruritus in PBC patients is of great significance for improving their quality of life. This article mainly summarizes the advances in the pathogenesis of PBC and the treatment of PBC-related pruritus.
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ObjectiveTo investigate the diagnostic efficiency of FibroTouch, FibroScan, and acoustic radiation force impulse (ARFI) for liver fibrosis in patients with primary biliary cholangitis (PBC). MethodsA retrospective analysis was performed for the patients who underwent liver biopsy and were then diagnosed with PBC in Beijing Friendship Hospital, Capital Medical University, from September 2014 to October 2018, and the METAVIR scoring system was used to evaluate the degree of liver fibrosis and inflammation. Within 1 week after liver biopsy, FibroTouch, FibroScan, and ARFI were used to measure liver stiffness (LS); with pathological results as the gold standard, the area under the ROC curve (AUC) was used to compare the value of FibroTouch, FibroScan, and ARFI in the diagnosis of liver fibrosis in PBC patients, and related influencing factors were analyzed; Youden index was used to calculate the cut-off values of LS for different degrees of liver fibrosis. The Kruskal-Wallis H test was used for comparison between multiple groups, and P value corrected by the Bonferroni method was used for further comparison between two groups. A Spearman correlation analysis was performed, and a multiple linear regression model was used for multivariate analysis. ResultsA total of 68 patients with PBC were enrolled in the study, among whom 13 had F0 liver fibrosis, 15 had F1 liver fibrosis, 18 had F2 liver fibrosis, 12 had F3 liver fibrosis, and 10 had F4 liver fibrosis. LS obtained by FibroTouch (FT-LS), LS obtained by FibroScan (FS-LS), and LS obtained by ARFI (ARFI-LS) were strongly positively correlated with the degree of liver fibrosis (r=0.798, 0.782, and 0.742, all P＜0.001). FT-LS had AUCs of 0.922, 0.881, and 0.926, respectively, in the diagnosis of F≥2, F≥3, and F=4 liver fibrosis, and the corresponding cut-off values were 10.5 kPa, 15.8 kPa, and 17.5 kPa, respectively; FS-LS had AUCs of 0.918, 0.878, and 0.939, respectively, in the diagnosis of F≥2, F≥3, and F=4 liver fibrosis, and the corresponding cut-off values were 10.1 kPa, 12.9 kPa, and 18.2 kPa, respectively; ARFI-LS had AUCs of 0.904, 0.869, and 0.928, respectively, in the diagnosis of F≥2, F≥3, and F=4 liver fibrosis, and the corresponding cut-off values were 1.45 m/s, 1.83 m/s, and 2.08 m/s, respectively. There was no significant difference in diagnosing the same stage of liver fibrosis between FibroTouch, FibroScan, and ARFI (P＞0.05). The multivariate analysis showed that degree of liver fibrosis (β=0.399, P＜0.001), total bilirubin (β=0.466, P＜0.001), and prothrombin time activity (β=-0.195, P=0.020) were influencing factors for FT-LS; degree of liver fibrosis (β=0370, P＜0.001), aspartate aminotransferase (β=0.450, P＜0.001), prothrombin time activity (β=-0.303, P=0.001), and alkaline phosphatase (β=-0.187, P=0.042) were influencing factors for FS-LS; degree of liver fibrosis (β=0.489, P＜0.001), aspartate aminotransferase (β=0.467, P＜0.001), and platelet count (β=-0.188, P=0.028) were influencing factors for ARFI-LS. ConclusionFibroTouch has similar efficiency to FibroScan and ARFI in the diagnosis of liver fibrosis in PBC patients, with relatively high diagnostic efficiency for significant liver fibrosis (F≥2) and liver cirrhosis (F=4), and therefore, it can be used as a reliable method for the diagnosis of liver fibrosis in PBC patients.
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Primary biliary cholangitis (PBC) is an autoimmune disease. Although PBC has the features of autoimmune disease, it has poor response to immunosuppressants and good response to the drugs participating in bile acid metabolism, such as ursodeoxycholic acid. Studies have shown that the bicarbonate secretion of biliary epithelial cells is impaired in PBC patients, and bile acid not blocked by HCO3- umbrella enters biliary epithelial cells and mediates their damage and apoptosis, leading to the expression of autoantibodies in apoptotic cells and immunologic injury. In order to explore the role of HCO3- umbrella secreted by biliary epithelial cells in the pathogenesis of PBC, this article briefly introduces the physiological function and production mechanism of HCO3- umbrella and the influencing factors for HCO3- secretion, and it is pointed out that reduced HCO3- secretion may be a key link in the pathogenesis of PBC and a potential therapeutic target.
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Objective To investigate the value of multiplex bead-based flow fluorescent immunoassay (MBFFI) in the diagnosis of primary biliary cholangitis (PBC) by analyzing its results in detecting antimitochondrial antibody M2 subtype (AMA-M2), gp210 antibody, and sp100 antibody. Methods A total of 340 patients who attended The Affiliated Hospital of Qingdao University from August 2018 to June 2020 and were diagnosed with PBC were enrolled as PBC group, and 143 patients with other diseases and 117 individuals undergoing physical examination were also enrolled. MBFFI and immunoblotting test (IBT) were used to detect AMA-M2, gp210, and sp100 autoantibodies in serum, and the Kappa-test was used to compare the consistency of the two methods in detecting the same autoantibody; the receiver operating characteristic (ROC) curve was used to evaluate the value of MBFFI detection of three antibodies in the diagnosis of PBC; the chi-square test was used to compare positive. Results In the PBC group, the two methods showed the best consistency in detecting AMA-M2(Kappa=0.874) and showed relatively good consistency in detecting gp210 and sp100 antibodies (Kappa=0.713 and 0.749). MBFFI had the highest sensitivity of 72.06% in detecting AMA-M2; it had a sensitivity of 44.71% in the combined detection of gp210 and sp100 antibodies, which was higher than its sensitivity in detecting each antibody alone; it had a sensitivity of 82.65% in the combined detection of AMA-M2, gp210, and sp100 antibodies, which was higher than its sensitivity in detecting each antibody alone. Combined detection of the three antibodies had the largest area under the ROC curve of 0.907 0 in the diagnosis of PBC. Conclusion MBFFI has good consistency with IBT in detecting autoantibodies associated with PBC, and the combined detection of AMA-M2, gp210, and sp100 antibodies by MBFFI has higher sensitivity and value in the diagnosis of PBC.
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Primary biliary cholangitis (PBC) is an inflammatory and cholestatic liver disease caused by autoimmune response targeting the small- and medium-sized intrahepatic bile ducts. The most specific manifestations of this diseases in clinical practice were positive anti-mitochondrial antibody and selective destruction of small- and medium-sized bile ducts based on liver histology. Since it is difficult to obtain the clinical samples of early-stage PBC, the construction and optimization of mouse models is an important method to investigate the pathogenesis of PBC. An understanding of the modeling principles of PBC animal models and disease features in serology, histology, and cytology not only helps to improve the awareness of PBC, but also helps to design scientific and rational research protocols.
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Primary biliary cholangitis (PBC) is an autoimmune-mediated chronic cholestatic liver disease, with the typical biochemical manifestation of significantly elevated alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT), with or without mild-to-moderate elevation of aminotransferases. ALP and GGT play an important role in the diagnosis and monitoring of PBC. With a deeper understanding of PBC, the significance of elevated aminotransferases in diagnosis and treatment has attracted more and more attention. This article briefly summarizes the significance of elevation of aminotransferases in PBC patients.