Child ; Humans ; Liver Cirrhosis, Biliary/diagnosis*

Objective: To analyze the clinical characteristics and prognostic value of liver function in a large samples of patients with anti-glycoprotein 210 (gp210 antibody) positive primary biliary cholangitis (PBC). Methods: A retrospective study was performed on 931 PBC cases in Beijing You'an Hospital affiliated to Capital Medical University from 2010 to 2019. According to the detection of gp210 antibody, 318 cases were divided into gp210 antibody positive group (positive group) and 613 cases were divided into gp210 antibody negative group (negative group). The differences in demographic, medical history, clinical indicators, B-ultrasound and pathological indicators as well as the histopathological basis were compared between the two groups. SPSS 16.0 software was used for statistical analysis. Measurement data were analyzed by t-test or rank sum test, and enumeration data by χ² test. Multivariate analysis was used for logistic test, and and survival analysis was used for prognosis. Results: The positive and the negative groups were compared. The ratio of male to female was significantly higher in positive than negative group (1:5.35 vs. 1:9.73, P＜0.05), and the difference was statistically significant. The proportion of hormone use in history of past diagnosed and treated was higher in positive than negative group (12.9% vs. 3.47%, P＜0.05), and the difference was statistically significant. The detection of biochemical indexes such as alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBIL), alkaline phosphatase (ALP), glutamyl transpeptidase (GGT) were higher in positive than the negative group (51.1 U/L vs. 41.1 U/L, 62.6 U/L vs. 49.6 U/L, 24.1 μmol/L vs. 17.9 μmol/L, 228.3 U/L vs. 169.6 U/L, 203.9 U/L vs. 147.6 U/L), (P＜0.05), and the differences were statistically significant. Antinuclear antibody (ANA)-positive rate, high titer ratio and immunoglobulin G (IgG) levels were higher in positive than negative group (95.2% vs. 81.6%, 69.7% vs. 48.8%, 17.2 g/L vs. 16.2 g/L), (P＜0.05), and the differences were statistically significant. The incidence of liver failure was higher in positive than negative group (P＜0.05). CK7 and inflammation score were higher in positive group than negative group in liver histopathological observations (0.83±0.53 vs. 0.28±0.47; 1.06±0.39 vs. 0.54±0.65), (P＜0.05), and the differences were statistically significant. Conclusion: The illness condition of patients with gp210 antibody positive PBC is more severe than patients with gp210 antibody negative PBC, and the incidence of liver failure is significantly increased. Cholangiocytes may be the histopathological basis of the clinical characteristics of gp210 antibody positive PBC patients.
Aspartate Aminotransferases ; Autoantibodies ; Female ; Humans ; Liver Cirrhosis, Biliary/diagnosis* ; Liver Failure ; Male ; Retrospective Studies

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen. The presence of this antibody is the most useful index in diagnosing acute hepatitis B (+) by immunoserological test. The final diagnosis of the patient in Good Gang-An Hospital was PBC through additional tests. The patient is receiving outpatient treatment.
Antibodies ; Bile Ducts ; Biopsy ; Cholestasis ; Diagnosis ; Fatigue ; Hepatitis B Core Antigens ; Hepatitis B* ; Hepatitis* ; Humans ; Immunoglobulin M ; Immunoglobulins ; Liver ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Outpatients ; Pruritus ; Republic of Korea*

Bile canalicular antibody (BCA) was first reported in 1969. Many studies of BCA were performed in the 1970s and 1980s and revealed that BCA has a highly positive rate in chronic active hepatitis and primary biliary cirrhosis (PBC). These studies suggested that BCA can be useful in the diagnosis of these liver diseases. However, BCA is almost negative in patients with alcoholic hepatitis. We report a case of BCA in a 50-yr-old woman with a history of heavy alcohol consumption. The patient's serum levels of aspartate transaminase and alanine transaminase were increased, leading to a diagnosis of alcoholic hepatitis. The patient was evaluated for liver disease. Anti-mitochondria antibody, anti-smooth muscle antibody, and anti-liver kidney microsomal antibody tests were conducted, yielding negative results. However, during this testing process, the patient's serum was incidentally found to be positive for BCA at a titer of 1:160. This is the first case report of BCA in Korea.
Alanine Transaminase ; Alcohol Drinking ; Alcoholics* ; Aspartate Aminotransferases ; Bile* ; Diagnosis ; Female ; Hepatitis, Alcoholic* ; Hepatitis, Chronic ; Humans ; Kidney ; Korea ; Liver Cirrhosis, Biliary ; Liver Diseases

OBJECTIVES: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that may progress to end stage liver cirrhosis. Benefits of ursodeoxycholic acid (UDCA) treatment has been investigated through large clinical studies. However, most of the studies were done in western countries and recent increase in prevalence of this relatively uncommon chronic liver disease draws attention in Korea. As early UDCA treatment effectively prevent the grave consequences of PBC progression, early diagnosis and lifelong management with UDCA is important. This study was designed to investigate the clinical features of PBC and response rates of UDCA treatments in Ewha Womans University Medical Center. METHODS: Clinical data of PBC patients diagnosed between 2001 and 2014 at Ewha Womans University Medical Center were analyzed retrospectively. RESULTS: A total of 35 patients with mean follow-up duration of 42 months were enrolled. At the diagnosis, 72.7% of the patients were asymptomatic, 5.7% had decompensated liver cirrhosis. The mean serum alkaline phosphate (ALP) level was 2.65 times upper limit of normal. UDCA was prescribed in 91.4% of the patients (n=32), among which 77.4% exhibited biochemical responses defined as serum ALP less than 2 upper limit of normal at 6 months (Mayo criteria). CONCLUSION: Most PBC patients were asymptomatic at the time of diagnosis and the average biochemical responses rate to UDCA treatment were ranged from 60.0% to 78.9% according to various response criteria. To elucidate the clinical features and courses of Korean PBC patients in detail, larger scale investigations and longer clinical follow up studies are warranted.
Academic Medical Centers ; Diagnosis ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Korea ; Liver Cirrhosis ; Liver Cirrhosis, Biliary* ; Liver Diseases ; Prevalence ; Retrospective Studies ; Ursodeoxycholic Acid

BACKGROUND/AIMS: Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) (AIH-PBC overlap syndrome) is a rare disease that has not been clearly characterized in Korean patients. This study investigated the clinical features of AIH-PBC overlap syndrome compared with those of AIH and PBC alone. METHODS: This retrospective cohort study included 158 consecutive patients who were diagnosed as AIH (n=61), PBC (n=81), or AIH-PBC overlap syndrome (n=9) based on the Paris and the International Autoimmune Hepatitis Group (IAIHG) criteria from 2001 to 2011 in Korea. We compared the clinical features of these three groups retrospectively, including their biochemical characteristics, treatments, responses, and clinical outcomes. RESULTS: The AIH-PBC overlap syndrome patients exhibited biochemical characteristics of both AIH and PBC, and showed a similar response to ursodeoxycholic acid (UDCA) monotherapy as for the PBC patients. However, the response of AIH-PBC overlap syndrome patients to UDCA and steroid combination therapy was worse than the response of AIH patients to steroid-based therapy (P=0.024). Liver cirrhosis developed more rapidly in AIH-PBC overlap syndrome patients than in AIH patients group (P=0.013), but there was no difference between AIH-PBC overlap syndrome patients and PBC patients. The rates of developing hepatic decompensation did not differ significantly between the groups. CONCLUSIONS: The AIH-PBC overlap syndrome patients exhibited a worse response to UDCA and steroid combination therapy and a faster cirrhotic progression compared with AIH patients.
Adult ; Aged ; Cohort Studies ; Drug Therapy, Combination ; Female ; Hepatitis, Autoimmune/complications/*diagnosis ; Humans ; Liver/metabolism/pathology ; Liver Cirrhosis, Biliary/complications/*diagnosis/drug therapy ; Male ; Middle Aged ; Republic of Korea ; Retrospective Studies ; Steroids/therapeutic use ; Treatment Outcome ; Ursodeoxycholic Acid/therapeutic use

OBJECTIVE: To determine features of the clinical manifestation in patients with primary biliary cirrhosis (PBC), and to provide a scientific basis for diagnosis of PBC.
 METHODS: A total of 102 patients with PBC treated in the Second Xiangya Hospital, Central South University, from January 2013 to January 2015 were retrospectively analyzed. The patients' general condition, clinical manifestations, serum biochemical and immunological parameters were detected.
 RESULTS: Of the 102 PBC patients, 91 (89.21%) patients were female. The main symptoms in these patients were fatigue, poor appetite, dry mouth, nausea, vomiting, pruritus, stomachache, and abdominal distension. The major signs were jaundice, splenomegaly, hepatomegaly, edema, and ascites. The main features of serum biochemical parameters in these patients included the increase of alkaline phosphatase and gamma glutamyltranspeptidase (GGT), especially the GGT. The anti-mitochondrial antibodies-M2 (AMA-M2) in 81 and 21 patients was positive and negative, respectively. The differences between the AMA-MA positive and negative groups were not statistically significant (P>0.05). According to clinical manifestation, 102 patients were classified into 2 groups: A non-cirrhosis group (n=56) and a cirrhosis group (n=46). The positive rates in these 2 groups, such as ANA, AMA-M2, anti-gp210, anti-Sp100, anti-Ro52, anti-PML, were 54.35%, 89.13%, 41.30%, 13.04%, 43.38% and 10.87% vs 57.14%, 71.43%, 42.86%, 12.5%, 51.79% and 3.71%, respectively, with no significant difference between them (P>0.05). However, there was significant difference in the positive rate of anti-3E-EPO between the above 2 groups (86.78% vs 58.93%, P<0.05). The positive rates of AMA-M2 and anti-3E-EPO in 30 patients diagnosed by hepatic histopathological examination were higher than those of other antibodies.
 CONCLUSION PBC mainly affects middle-aged women, and its clinical manifestation is various. The autoantibody tests play an important role in diagnosis of PBC. Checking for AMA-A2 and anti-3E-BPO can improve the positive rate of PBC. Liver histopathological examination may provide useful information on disease severity, which can determine the histological stage when the patient's serum autoantibodies are negative.
Alkaline Phosphatase ; metabolism ; Autoantibodies ; blood ; Female ; Humans ; Liver Cirrhosis, Biliary ; diagnosis ; pathology ; Male ; Mitochondria ; Retrospective Studies ; gamma-Glutamyltransferase ; metabolism

BACKGROUND: Anti-mitochondrial antibody (AMA) is a serological hallmark of primary biliary cirrhosis (PBC). AMAs are detected by an immunofluorescence assay (IF), which is subject to errors. We evaluated the diagnostic performances of the AMA ELISA test (the anti-MIT3 antibody) and PBC-associated antinuclear antibody (ANA) tests (the anti-gp210 and anti-sp100 antibodies). METHODS: AMA, anti-gp210, and anti-sp100 were measured in the sera of 130 subjects including patients for whom the AMA test was requested with the clinical suspicion of PBC, patients with other autoimmune diseases, and those undergoing health check-ups. AMA was detected by both IF and ELISA (anti-MIT3 antibodies), and anti-gp210 and anti-sp100 were detected by ELISA. The diagnostic performances of the anti-MIT3, anti-gp210, and anti-sp100 were compared with that of the AMA IF test. Associations between the presence of anti-sp100 or anti-gp210 and the diagnosis and biochemical abnormalities of PBC were investigated. RESULTS: The area under the curve of anti-MIT3 for the diagnosis of PBC was 0.934 (95% confidence interval, 0.877-0.970), and the agreement between anti-MIT3 and AMA IF was 93.8% (kappa, 0.82). The sensitivities of anti-MIT3 and AMA IF were both 100%, and the specificities were 83.1% and 81.4%, respectively, whereas the sensitivities of anti-gp210 and anti-sp100 were 41.7% and 16.7%, and their specificities were 94.9% and 97.5%, respectively. The presence of anti-gp210 was associated with the diagnosis of PBC (P=0.0001), but that of anti-sp100 was not. CONCLUSIONS: The diagnostic performance of anti-MIT3 is comparable to that of AMA IF. Anti-gp210 seems to be complementary to AMA for the diagnosis of PBC.
Antibodies* ; Antibodies, Antinuclear ; Autoimmune Diseases ; Diagnosis* ; Enzyme-Linked Immunosorbent Assay ; Fluorescent Antibody Technique ; Humans ; Liver Cirrhosis, Biliary*

Enbucrilate ; adverse effects ; therapeutic use ; Female ; Humans ; Hypertension, Portal ; diagnosis ; etiology ; Liver Cirrhosis, Biliary ; drug therapy ; Middle Aged ; Pulmonary Embolism ; chemically induced ; diagnosis ; Sepsis ; chemically induced ; etiology

BACKGROUND: We summarize our experience in the pathological diagnosis of late complications of liver transplantation (LT) to better understand the causes of late allograft dysfunction in a population mostly composed of patients with hepatitis B virus (HBV) infection. METHODS: We reviewed 361 post-transplant liver biopsies from 174 patients who underwent LT and first presented with liver function abnormalities 3 months post-procedure. The underlying diseases included HBV-associated liver disease (77%), toxic or alcoholic liver disease (10.3%), hepatitis C virus (HCV)-associated liver disease (8.6%), primary biliary cirrhosis (1.2%), primary sclerosing cholangitis (1.2%), and metabolic disease (1.7%). RESULTS: The three most common late complications were acute rejection (32.5%), recurrent disease (19.1%), and biliary complication (17.1%). Patients who underwent LT for HBV infection or for drug- or alcohol-related liver disease had a lower incidence of recurring disease than those who underwent transplantation for HCV infection. During post-transplantation months 3-12, acute rejection was the most common cause of allograft dysfunction and recurring disease was the leading cause for allograft dysfunction (p=0.039). The two primary causes of late allograft dysfunction have overlapping histological features, although acute rejection more frequently showed bile duct damage and vascular endothelialitis than recurring HBV infection, and recurring HBV infection had more frequent lobular activity and piecemeal necrosis. CONCLUSIONS: The causes of late liver allograft dysfunction are closely associated with the original liver diseases and the period after LT. Careful attention is required for differential diagnosis between acute rejection and recurrent HBV.
Bile Ducts ; Biopsy ; Cholangitis, Sclerosing ; Diagnosis, Differential ; Hepacivirus ; Hepatitis B virus ; Humans ; Incidence ; Liver ; Liver Cirrhosis, Biliary ; Liver Diseases ; Liver Diseases, Alcoholic ; Liver Transplantation ; Metabolic Diseases ; Rejection (Psychology) ; Transplantation, Homologous ; Transplants