Fatigue ; etiology ; Humans ; Liver Cirrhosis, Biliary ; complications ; Pruritus ; etiology

Autoantibodies ; Cholangitis, Sclerosing ; complications ; diagnosis ; Hepatitis, Autoimmune ; complications ; diagnosis ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; Syndrome

Autoantibodies ; blood ; Cholangitis, Sclerosing ; complications ; diagnosis ; Hepatitis, Autoimmune ; complications ; diagnosis ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis

Adult ; Factor VII Deficiency ; complications ; Female ; Humans ; Liver Cirrhosis, Biliary ; complications

Biliary Tract Diseases ; complications ; surgery ; Humans ; Hypertension, Portal ; complications ; etiology ; surgery ; Liver Cirrhosis ; complications ; surgery ; Perioperative Care

Cholangitis, Sclerosing ; complications ; diagnosis ; therapy ; Hepatitis, Autoimmune ; complications ; diagnosis ; therapy ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; therapy ; Syndrome

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands leading to a progressive destruction of these glands due to the production of autoantibodies. This disorder is either isolated (primary SS) or associated with other systemic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. The risk of developing NHL, which is equivalent for both primary and secondary SS, was estimated to be 44 times greater than that observed in a comparable normal population. NHLs in SS patients occur preferentially in the salivary glands and in other mucosa-associated lymphoid tissues (MALT). However, it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyelid and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.
Case Report ; Eyelid Neoplasms/pathology ; Eyelid Neoplasms/etiology* ; Female ; Human ; Liver Cirrhosis, Biliary/pathology ; Liver Cirrhosis, Biliary/complications* ; Lymphoma, Mucosa-Associated Lymphoid Tissue/pathology ; Lymphoma, Mucosa-Associated Lymphoid Tissue/etiology* ; Middle Age ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/complications*

OBJECTIVETo analyze the clinical and pathological characteristics of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) overlap syndrome.

METHODSOf our 68 patients with the diagnosis of PBC, we identified 9 overlap syndrome cases strictly using the revised descriptive criteria and scoring system for diagnosis of AIH proposed by the International Autoimmune Hepatitis Group Report. The clinical manifestations and pathological changes shown in liver biopsies from the overlap syndrome and pure PBC were analyzed and compared.

RESULTSThe mean aggregate scores of the 9 cases (13.2%) of the overlap syndrome group and 59 cases of pure PBC (86.8%) were 10.2+/-0.2 and 4.7+/-0.7 respectively among the 68 total. The serum levels of ALT and AST, immunoglobulin G, gammaglobulin, in the overlap syndrome group were significantly higher than those in the pure PBC group, and also there was a frequent presence of antinuclear antibody and/or smooth muscle antibody positivity in the overlap syndrome group. Histopathologically the livers in the overlap syndrome group showed combined features of interface hepatitis and piecemeal necrosis, characterizing the two diseases.

CONCLUSIONThe overlap syndrome group showed combined features of both PBC and AIH. There were differences in clinical aspects, serology and histology between the overlap syndrome and pure PBC groups.

Adult ; Female ; Hepatitis, Autoimmune ; complications ; diagnosis ; pathology ; Humans ; Immunoglobulin G ; blood ; Liver ; pathology ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; pathology ; Male ; Middle Aged ; Retrospective Studies

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

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*