Diagnosis and treatment of primary biliary cholangitis: Is pathology necessary? / 临床肝胆病杂志

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.

Treatment of primary biliary cholangitis by targeting intestinal flora / 临床肝胆病杂志

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.

Efficacy of transjugular intrahepatic portosystemic shunt in treatment of primary biliary cholangitis with portal hypertension / 临床肝胆病杂志

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.

Comparison of percutaneous transhepatic choledochoscopic lithotripsy and traditional open hepatectomy in the treatment of regional hepatolithiasis with biliary cirrhosis / 中国医师杂志

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.

Fanconi syndrome secondary to primary biliary cholangitis: a case report / 中华肾脏病杂志

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.

Production mechanism and detection value of anti-gp210 antibody in primary biliary cholangitis / 临床肝胆病杂志

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.

Research advances in sodium taurocholate cotransporting polypeptide in hepatobiliary diseases / 临床肝胆病杂志

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.

Liver transplantation for the treatment of iatrogenic bile duct injury / Transplante hepático no tratamento da lesão iatrogênica da via biliar

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.

Diagnosis and treatment of primary biliary cholangitis: Current status and challenges / 临床肝胆病杂志

Primary biliary cholangitis is an autoimmune liver disease often observed in women, with the main features of positive antimitochondrial antibodies in serum and progressive non-pyogenic inflammatory destruction of small intrahepatic bile ducts. At present, primary biliary cholangitis is considered the result of interaction between chronic immune injury and biliary epithelium under the combined effect of inheritance and environment. Ursodeoxycholic acid is the first-line drug for primary biliary cholangitis; for patients who cannot tolerate ursodeoxycholic acid or have suboptimal response, the second-line drug obeticholic acid, an FXR agonist, can be considered, and the peroxisome proliferator-activated receptor agonists fibrates can be regarded as second-line alternative drugs. Baseline characteristics (such as young age, male sex, and advanced disease) and blood biochemical parameters (especially bilirubin and alkaline phosphatase) are used for disease risk stratification and response evaluation. Management of the symptoms such as pruritus and weakness cannot be ignored.

The relationship between primary biliary cholangitis and Sjögren's syndrome / 临床肝胆病杂志

Both primary biliary cholangitis and Sjögren's syndrome belong to chronic autoimmune epithelitis, and they often occur at the same time. This article reviews the general features, clinical manifestations, serological features, pathogenesis, treatment regimens, and common features of primary biliary cholangitis and Sjögren's syndrome and explores the common pathogenesis of the two diseases, so as to provide ideas for developing new therapies and management strategies for patients.

Diagnosis and management of primary biliary cholangitis with osteoporosis / 临床肝胆病杂志

Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease often observed in the middle-aged and elderly women and it can eventually lead to liver cirrhosis or liver failure. Osteoporosis is one of the common complications in PBC patients and is characterized by decreased bone mass and increased susceptibility to fractures. Osteoporosis and fractures caused by osteoporosis seriously affect the quality of life of PBC patients, and with the improvement of PBC treatment strategies and the increase in life expectancy, early diagnosis, prevention, and treatment of PBC with osteoporosis is of particular importance. This article briefly summarizes the epidemiology, pathogenesis, and diagnosis and treatment of patients with PBC and osteoporosis and proposes current challenges and future research directions.

Considerations on the elevation of aminotransferases in primary biliary cholangitis / 临床肝胆病杂志

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.

Research advances in animal models of primary biliary cholangitis / 临床肝胆病杂志

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.

Value and clinical application of multiplex bead-based flow fluorescent immunoassay in the detection of autoantibodies in primary biliary cholangitis / 临床肝胆病杂志

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.

Diagnosis and treatment of primary biliary cholangitis: Current status and challenges / 临床肝胆病杂志

Primary biliary cholangitis is an autoimmune liver disease often observed in women, with the main features of positive antimitochondrial antibodies in serum and progressive non-pyogenic inflammatory destruction of small intrahepatic bile ducts. At present, primary biliary cholangitis is considered the result of interaction between chronic immune injury and biliary epithelium under the combined effect of inheritance and environment. Ursodeoxycholic acid is the first-line drug for primary biliary cholangitis; for patients who cannot tolerate ursodeoxycholic acid or have suboptimal response, the second-line drug obeticholic acid, an FXR agonist, can be considered, and the peroxisome proliferator-activated receptor agonists fibrates can be regarded as second-line alternative drugs. Baseline characteristics (such as young age, male sex, and advanced disease) and blood biochemical parameters (especially bilirubin and alkaline phosphatase) are used for disease risk stratification and response evaluation. Management of the symptoms such as pruritus and weakness cannot be ignored.

The relationship between primary biliary cholangitis and Sjögren's syndrome / 临床肝胆病杂志

Both primary biliary cholangitis and Sjögren's syndrome belong to chronic autoimmune epithelitis, and they often occur at the same time. This article reviews the general features, clinical manifestations, serological features, pathogenesis, treatment regimens, and common features of primary biliary cholangitis and Sjögren's syndrome and explores the common pathogenesis of the two diseases, so as to provide ideas for developing new therapies and management strategies for patients.

Diagnosis and management of primary biliary cholangitis with osteoporosis / 临床肝胆病杂志

Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease often observed in the middle-aged and elderly women and it can eventually lead to liver cirrhosis or liver failure. Osteoporosis is one of the common complications in PBC patients and is characterized by decreased bone mass and increased susceptibility to fractures. Osteoporosis and fractures caused by osteoporosis seriously affect the quality of life of PBC patients, and with the improvement of PBC treatment strategies and the increase in life expectancy, early diagnosis, prevention, and treatment of PBC with osteoporosis is of particular importance. This article briefly summarizes the epidemiology, pathogenesis, and diagnosis and treatment of patients with PBC and osteoporosis and proposes current challenges and future research directions.

Considerations on the elevation of aminotransferases in primary biliary cholangitis / 临床肝胆病杂志

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.

Research advances in animal models of primary biliary cholangitis / 临床肝胆病杂志

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.

Value and clinical application of multiplex bead-based flow fluorescent immunoassay in the detection of autoantibodies in primary biliary cholangitis / 临床肝胆病杂志

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.