To systematically evaluate the risks of cardiocerebral vascular events in patients with primary biliary cholangitis(PBC). Methods We carried out a Meta analysis by RevMan 5.3 software to investigate literatureon the risk of cardiocerebral vascular events in patients with PBC and controls. Results Compared with non-PBC controls,PBC patients had significantly higher risk of coronary events(=1.56,=0.0002);however,the risk of cerebrovascular events showed no significant difference between these two groups(=1.01,=0.94).Subgroup analysis demonstrated a significantly lower risk of transient ischemic attack or carotid stenosis in PBC patients(=0.63,=0.03);however,there was no significant difference in the risk of stroke(=1.11,=0.40). Conclusion Patients with PBC have an increased risk of coronary events but may have a lower risk of transient ischemic attack or carotid stenosis.
Carotid Stenosis ; etiology ; Cholangitis ; complications ; Coronary Disease ; etiology ; Humans ; Ischemic Attack, Transient ; etiology ; Liver Cirrhosis, Biliary ; complications ; Risk Factors ; Stroke ; etiology

PURPOSE: The purpose of this study was to define the role of cyclooxygenase-2 inhibitors (COX-2i) in reducing hepatic fibrosis in pediatric patients with chronic liver disease. MATERIALS AND METHODS: From September 2009 to September 2010, patients over 2 years old who visited our outpatient clinic for follow-up to manage their chronic liver disease after Kasai portoenterostomy for biliary atresia, were included in this study. Volunteers were assigned to the study or control groups, according to their preference. A COX-2i was given to only the study group after obtaining consent. The degree of hepatic fibrosis (liver stiffness score, LSS) was prospectively measured using FibroScan, and liver function was examined using serum analysis before and after treatment. After 1 year, changes in LSSs and liver function were compared between the two groups. RESULTS: Twenty-five patients (18 females and 7 males) were enrolled in the study group. The control group included 44 patients (26 females and 18 males). After 1 year, the least square mean values for the LSSs were significantly decreased by 3.91±0.98 kPa (p=0.004) only in the study group. Serum total bilirubin did not decrease significantly in either group. CONCLUSION: COX-2i treatment improved the LSS in patients with chronic liver disease after Kasai portoenterostomy for biliary atresia.
Biliary Atresia/complications/enzymology/*surgery ; Child ; Child, Preschool ; Chronic Disease ; Cyclooxygenase 2 Inhibitors/*therapeutic use ; Female ; Humans ; Liver Cirrhosis/etiology/pathology/*prevention & control ; Male ; *Portoenterostomy, Hepatic ; Thiazines/*therapeutic use ; Thiazoles/*therapeutic use

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

In order to investigate the safety and efficacy of endoscopic retrograde cholangiopancreatograpy (ERCP) in liver cirrhosis patients with common bile duct stones, we retrospectively analyzed data of 46 common bile duct stones patients with liver cirrhosis who underwent ERCP between 2000 and 2008. There were 12 cases of Child-Pugh A, 26 cases of Child-Pugh B, and 8 cases of Child-Pugh C. 100 common bile duct stones patients without liver cirrhosis were randomly selected. All the patients were subjected to ERCP for biliary stones extraction. The rates of bile duct clearance and complications were compared between cirrhotic and non-cirrhotic patients. The success rate of selective biliary cannulation was 95.6% in liver cirrhotic patients versus 97% in non-cirrhotic patients (P>0.05). The bile duct clearance rate was 87% in cirrhotic patients versus 96% in non-cirrhotic patients, but the difference was not statistically significant. Two liver cirrhotic patients (4.35%, 2/46) who were scored Child-Pugh C had hematemesis and melena 24 h after ERCP. The hemorrhage rate after ERCP in non-cirrhotic patients was 3%. The hemorrhage rate associated with ERCP in Child-Pugh C patients was significantly higher (25%, 2/8) than that (3%, 3/100) in non-cirrhotic patients (P<0.01%). There was no significant difference between these two groups in the rate of post-ERCP pancreatitis (PEP) and cholangitis. ERCP is safe and effective for Child-Pugh A and B cirrhotic patients with common bile duct stones. Hemorrhage risk in ERCP is higher in Child-Pugh C patients.
Adult ; Aged ; Aged, 80 and over ; Cholangiopancreatography, Endoscopic Retrograde ; adverse effects ; methods ; Female ; Gallstones ; complications ; surgery ; Humans ; Liver Cirrhosis, Biliary ; complications ; surgery ; Male ; Middle Aged ; Postoperative Hemorrhage ; etiology ; Retrospective Studies

Anatomic variations of the portal vein (PV) and bile duct (BD) are more common on the right lobe as compared with left lobe grafts in living donor liver transplantation (LDLT). We recently experienced a case of LDLT for hepatocellular carcinoma combined with liver cirrhosis secondary to hepatitis B virus and hepatitis C virus infection. The only available donor had right lobe graft with type IV PV associated with type IV BD. The patient underwent relaparotomy for PV stenting due to PV stenosis. Percutaneous transhepatic biliary drainage was done for a stricture at the site of biliary reconstruction. Thereafter, the patient was discharged in good health. Our experience suggests that, the use of right lobe graft with type IV PV accompanied by type IV BD should be the last choice for LDLT, because of its technical difficulty and risks of associated complications.
Bile Ducts ; Biliary Tract* ; Carcinoma, Hepatocellular ; Constriction, Pathologic ; Drainage ; Hepacivirus ; Hepatitis B virus ; Humans ; Liver Cirrhosis ; Liver Transplantation* ; Living Donors* ; Portal Vein* ; Postoperative Complications ; Stents ; Tissue Donors ; Transplants*

OBJECTIVEEndogenous hydrophobic bile acids may be a pathogenetic factor of biliary complications after orthotopic liver transplantation (OLT).This study was designed to investigate the effects of hydrophilic ursodeoxycholic acid (UDCA), when administered early after OLT, on serum liver tests and on the incidence of biliary complications.

METHODSA total of 112 adult patients undergoing OLT were randomly assigned to one of two groups for receipt of UDCA (13 to 15 mg/kg/d for 4 weeks, n=56) or a placebo (n=56). All patients underwent serum liver testing and measurement of serum bile acids during the 4 weeks following OLT.Patients with T-tube underwent measurement of biliary bile acids during the 4 weeks following OLT.Biliary complications, as well as patient and graft survival rates, were analyzed during the follow-up period (mean of 65.6 months).

RESULTSAt post-OLT days 7, 21 and 28, the UDCA-treated patients showed significantly lower levels of alanine aminotransferase, aspartate aminotransferase and gamma glutamyl transpeptidase (all P less than 0.05).In addition, the UDCA-treated patients showed significantly lower incidence of biliary sludge and casts within the first year post-OLT (3.6% vs.14.3%; x2=3.953, P=0.047). However, there were no significant differences for the incidence of other biliary complications at post-OLT years 1, 3 and 5.The graft and patient survival rates were also similar between the two groups.

CONCLUSIONUDCA, when administered early after OLT, improves results from serum liver tests and decreases the incidence of biliary sludge and casts within the first postoperative year.

Alanine Transaminase ; Aspartate Aminotransferases ; Bile ; Bile Acids and Salts ; Biliary Tract Diseases ; drug therapy ; physiopathology ; Humans ; Liver ; physiopathology ; Liver Cirrhosis, Biliary ; Liver Function Tests ; Liver Transplantation ; Postoperative Complications ; physiopathology ; Ursodeoxycholic Acid ; therapeutic use ; gamma-Glutamyltransferase

OBJECTIVETo explore the expression level of corticotropin-releasing hormone receptor type-1 (CRHR-1) in intrahepatic cholestatic placental (ICP) tissue.

METHODSHuman placental samples were collected from 10 ICP patients and 10 healthy controls after parturition at 37-40 weeks of gestation. CRHR-1 protein and mRNA expression was assessed by western blotting and nested-real-time fluorescence quantitative PCR, respectively. Normally distributed data were summarized as mean +/- standard deviation, and intergroup comparisons were made by two-tailed Student's t-test. Non-normally distributed data were presented as median with interquartile range, and intergroup comparisons were made by Wilcoxon test. For all statistical analyses, a two-tailed P-value of less than 0.05 was considered statistically significant.

RESULTSThe CRHR-1 fluorescence intensity was lower in ICP tissues (1.55 +/- 0.28) than in placental tissues from healthy controls (1.60 +/- 0.37), but the difference did not reach statistical significance (t = 0.349, P = 0.732). The CRHR-1 mRNA content was slightly higher in the ICP tissues [0.139(0.268)] than in the placental tissues from healthy controls [0.031(0.245)], but the difference did not reach statistical significance (t = 1.504, P = 0.136).

CONCLUSIONCRHR-1 expression is decreased in ICP tissues, which may lead to a smaller volume of placental lobular villi vessels and restrict the CRH positive feedback loop, ultimately promoting acute hypoxic stress and possible harm to the fetus.

Adult ; Case-Control Studies ; Cholestasis, Intrahepatic ; metabolism ; Female ; Humans ; Liver Cirrhosis, Biliary ; metabolism ; Placenta ; metabolism ; Pregnancy ; Pregnancy Complications ; metabolism ; Receptors, Corticotropin-Releasing Hormone ; metabolism

Drugs, Chinese Herbal ; therapeutic use ; Female ; Hepatitis, Autoimmune ; complications ; drug therapy ; pathology ; Humans ; Liver ; pathology ; physiopathology ; Liver Cirrhosis, Biliary ; complications ; drug therapy ; pathology ; Liver Function Tests ; Middle Aged ; Syndrome

OBJECTIVETo observe the efficacy of ursodeoxycholic acid (UDCA) combined with Tongdan: Decoction () on immunological indices and histopathological changes in patients with primary biliary cirrhosis (PBC) of IIor III histological stage.

METHODSSixty PBC patients were assigned randomly and equally: to the control group treated with UDCA alone and the treatment group treated with UDCA combined with Tongdan Decoction. The immunological indices and histopathological changes were detected before and after 24-week treatment, and the follow-up lasted for 1-3 years.

RESULTSAfter 24-week treatment, CD4(+)CD28(-) in the peripheral blood was lowered and CD4(+)CD25(+) was increased in both groups, and better effect was shown in the treatment group (P<0.01). The levels of IgM, IgG, and IgA decreased markedly after 96-week treatment in the treatment group (P< 0.05, P< 0.01), while in the control group, only the latter two showed significant decrease after 148 week (all P<0.05). At the end of the 3-year follow-up, the medians of histopathological

CONCLUSIONCombined therapy of Tongdan Decoction and UDCA showed a better therapeutic effect: than UDCA monotherapy on PBC, especially in improving immunological indices and histopathological hepatic changes.

Antigens, CD ; blood ; Biomarkers ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Immunoglobulin G ; blood ; Inflammation ; blood ; complications ; Liver Cirrhosis, Biliary ; blood ; drug therapy ; immunology ; pathology ; Male ; Middle Aged ; Ursodeoxycholic Acid ; therapeutic use

Visceral leishmaniasis (VL) is a life-threatening infection caused by Leishmania species. In addition to typical clinical findings as fever, hepatosplenomegaly, and cachexia, VL is associated with autoimmune phenomena. To date, VL mimicking or exacerbating various autoimmune diseases have been described, including systemic lupus erythematosus (SLE), rheumatoid arthritis, and autoimmune hepatitis (AIH). Herein, we presented a patient with VL who had overlapping clinical features with SLE, AIH, as well as antimitochondrial antibody (AMA-M2) positive primary biliary cirrhosis.
Adult ; Autoantibodies/blood ; Bone Marrow/pathology ; Cytological Techniques ; Diagnosis, Differential ; Female ; Hepatitis, Autoimmune/complications/*pathology ; Histocytochemistry ; Humans ; Leishmaniasis, Visceral/complications/*diagnosis/*pathology ; Liver/pathology ; Liver Cirrhosis, Biliary/complications/*pathology ; Lupus Erythematosus, Systemic/complications/*pathology