Prednisone or prednisolone are the mainstay drug treatments for autoimmune hepatitis in children. However, long-term use of corticosteroid is associated with the risk of steroid-induced toxicities, and this situation requires newer immuno-suppressive agents for the treatment of autoimmune hepatitis, especially in growing children. An 11-yr-old Korean girl with type-1 autoimmune hepatitis discontinued prednisolone due to toxicities, i.e., hirsutism, buffalo hump, and skin striae, and remained clinical and biochemical remission under replacement of deflazacort and ursodeoxycholic acid combination therapy. A follow-up liver biopsy after 19 months of deflazacort and ursodeoxycholic acid treatment showed histologic remission.
Ursodeoxycholic Acid/therapeutic use ; Treatment Outcome ; Pregnenediones/*therapeutic use ; Korea ; Immunosuppressive Agents/therapeutic use ; Humans ; Hepatitis, Autoimmune/*drug therapy ; Female ; Drug Therapy, Combination ; Cholagogues and Choleretics/therapeutic use ; Child

No direct comparison of tauroursodeoxycholic acid (TUDCA) and ursodeoxycholic acid (UDCA) has yet been carried out in the treatment of liver cirrhosis in China. We designed a double-blind randomized trial to evaluate the potential therapeutic efficacy of TUDCA in liver cirrhosis, using UDCA as parallel control. The enrolled 23 patients with liver cirrhosis were randomly divided into TUDCA group (n=12) and UDCA group (n=11), and given TUDCA and UDCA respectively at the daily dose of 750 mg, in a randomly assigned sequence for a 6-month period. Clinical, biochemical and histological features, and liver ultrasonographic findings were evaluated before and after the study. According to the inclusion criteria, 18 patients were included in the final analysis, including 9 cases in both two groups. Serum ALT, AST and ALP levels in TUDCA group and AST levels in UDCA group were significantly reduced as compared with baseline (P<0.05). Serum albumin levels were significantly increased in both TUDCA and UDCA groups (P<0.05). Serum markers for liver fibrosis were slightly decreased with the difference being not significant in either group. Only one patient in TUDCA group had significantly histological relief. Both treatments were well tolerated and no patient complained of side effects. It is suggested that TUDCA therapy is safe and appears to be more effective than UDCA in the treatment of liver cirrhosis, particularly in the improvement of the biochemical expression. However, both drugs exert no effect on the serum markers for liver fibrosis during 6-month treatment.
Adult ; Cholagogues and Choleretics ; therapeutic use ; Double-Blind Method ; Female ; Humans ; Liver Cirrhosis ; diagnosis ; drug therapy ; Male ; Middle Aged ; Taurochenodeoxycholic Acid ; therapeutic use ; Treatment Outcome ; Ursodeoxycholic Acid ; therapeutic use

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is various from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibody and histologic findings of nonsuppurative destructive cholangitis. Although the diagnosis is straightforward, it could be underdiagnosed because of its asymptomatic presentation, or underrecognition of the disease. UDCA in a dose of 13-15 mg/kg is the widely approved therapy which can improve the prognosis of patients with PBC. However, one-third of patients does not respond to UDCA therapy and may require liver transplantation. Every effort to diagnose PBC in earlier stage and to develop new therapeutic drugs and clinical trials should be made.
Autoantibodies/blood ; Autoimmunity/immunology ; Cholagogues and Choleretics/therapeutic use ; Humans ; Liver Cirrhosis, Biliary/*diagnosis/pathology/*therapy ; Liver Transplantation ; Ursodeoxycholic Acid/therapeutic use

Primary biliary cirrhosis (PBC) is a chronic cholestatic autoimmune liver disease that predominantly affects middle-aged women. It is characterized by slowly progressive destruction of the small intrahepatic bile ducts together with portal inflammation, and this initially leads to fibrosis and later to cirrhosis. It is currently accepted that the pathogenesis of PBC is multifactorial with genetic and environmental factors interplaying to determine the disease onset and progression. In addition to antimitochondrial antibody (AMA), which is the hallmark of PBC and is detected in at least 90% of the patients, other autoantibodies (antinuclear antibody, anti-smooth muscle antibody and rheumatoid factor, etc.) may also be found in the patients. There is no correlation between the titer of AMAs and the disease severity. Most patients are diagnosed either during the asymptomatic phase of PBC or after presenting with non-specific symptoms. Pruritus and fatigue are the most common symptoms of PBC. The prognosis of PBC has improved significantly during the last few decades. Patients are now diagnosed earlier in its clinical course, they are more likely to be asymptomatic at diagnosis and they are more likely to receive medical treatment. A wide variety of drugs have been assessed for the treatment of this condition: such immunosuppressive agents as corticosteroids, cyclosporine and azathioprine have a weak effect on the disease's natural history. Ursodeoxycholic acid (UDCA) is the only currently approved medical treatment. For PBC patients with end-stage liver disease or an unacceptable quality of life, liver transplantation is the only accepted therapeutic option. Early diagnosis and treatment of PBC are important because effective treatment with UDCA has been shown to delay disease progression and improve rate survival in the early stage.
Autoimmune Diseases/*diagnosis/*drug therapy/epidemiology ; Cholagogues and Choleretics/*therapeutic use ; Cholestadienes/administration & dosage/therapeutic use ; Cholic Acids/administration & dosage/therapeutic use ; Female ; Humans ; Liver Cirrhosis, Biliary/*diagnosis/*drug therapy/epidemiology ; Male ; Middle Aged ; Prevalence ; Rifampin/administration & dosage/therapeutic use

To evaluate the efficacy and safety of ursodeoxycholic acid (UDCA) with oral solubilized formula in amyotrophic lateral sclerosis (ALS) patients, patients with probable or definite ALS were randomized to receive oral solubilized UDCA (3.5 g/140 mL/day) or placebo for 3 months after a run-in period of 1 month and switched to receive the other treatment for 3 months after a wash-out period of 1 month. The primary outcome was the rate of progression, assessed by the Appel ALS rating scale (AALSRS), and the secondary outcomes were the revised ALS functional rating scale (ALSFRS-R) and forced vital capacity (FVC). Fifty-three patients completed either the first or second period of study with only 16 of 63 enrolled patients given both treatments sequentially. The slope of AALSRS was 1.17 points/month lower while the patients were treated with UDCA than with placebo (95% CI for difference 0.08-2.26, P = 0.037), whereas the slopes of ALSFRS-R and FVC did not show significant differences between treatments. Gastrointestinal adverse events were more common with UDCA (P < 0.05). Oral solubilized UDCA seems to be tolerable in ALS patients, but we could not make firm conclusion regarding its efficacy, particularly due to the high attrition rate in this cross-over trial.
Administration, Oral ; Amyotrophic Lateral Sclerosis/*drug therapy ; Cholagogues and Choleretics/pharmacology/therapeutic use ; Cross-Over Studies ; Double-Blind Method ; Female ; Humans ; Male ; Middle Aged ; Placebo Effect ; Severity of Illness Index ; Ursodeoxycholic Acid/pharmacology/*therapeutic use ; Vital Capacity/drug effects

OBJECTIVETo realize the clinical features of autoimmune hepatitis-primary biliary cirrhosis (AIH+PBC) overlap syndrome.

METHODSWe analyzed and compared the biochemistry, autoimmune antibodies, and liver biopsy results of 129 autoimmune hepatic disease cases retrospectively, using the international criteria to see which could be diagnosed as AIH/PBC overlap syndrome.

RESULTSOur 35 AIH+PBC overlap syndrome patients were mainly women, with a sex ratio of 1 female: 10 male, and a median age of 50.79+/-11.27 (20 to 70 years old). They had AIH characteristics such as flare of ALT, AST and elevated immunoglobulin G (IgG), gamma-immunoglobulin. There were also antinuclear antibodies (74.3%); moderate or severe periportal or periseptal lymphocytic infiltration, piecemeal necrosis, and florid bile duct lesions, high serum levels of ALP, presence of mitochondrial antibodies (68.6%) and M2 antibodies (45.7%), and features of PBC.

CONCLUSIONSAIH+PBC overlap syndrome is not rare. It should be diagnosed in time and to find effective treatments for it.

Adult ; Autoantibodies ; blood ; Cholagogues and Choleretics ; therapeutic use ; Female ; Hepatitis, Autoimmune ; complications ; diagnosis ; drug therapy ; Humans ; Immunoglobulin G ; blood ; Liver Cirrhosis, Biliary ; complications ; diagnosis ; drug therapy ; Male ; Middle Aged ; Retrospective Studies ; Ursodeoxycholic Acid ; therapeutic use

BACKGROUND/AIMS: Chronic hepatocellular damage is closely associated with hepatic fibrosis and fatal complication in most liver diseases. The aim of this study is to compare the efficacy and safety of biphenyl dimethyl dicarboxylate (DDB) and ursodeoxycholic acid (UDCA) in patients with abnormal ALT. METHODS: One-hundred thirty-five patients with elevated ALT were randomized to receive either 750 mg/day of DDB or 300 mg/day of UDCA for 24 weeks in 4 referral hospitals. Ninety-three (69%) patients had non-alcoholic steatohepatitits, 27 (20%) had alcoholic hepatitis, and 15 (11%) had chronic hepatitis. The primary end point was the rate of ALT normalization at week 24. The secondary endpoints were changes in AST, liver stiffness, and the incidence of adverse events. RESULTS: A total of 101 patients completed 24 weeks of therapy. ALT normalization at week 24 was observed in 44 (80.0%) patients in DDB group and 16 (34.8%) in UDCA group (p<0.001). Higher mean reduction of ALT levels from baseline to 24 weeks was seen in DDB group compared with UDCA group (-70.0% vs. -35.9%, p<0.001). Normalization of AST level (p=0.53) and change in the liver stiffness (p=0.703) were not significantly different between the two groups. Severe adverse drug reaction occurred in 1 patient in DDB group but the subject continued therapy during the study period. CONCLUSIONS: DDB was not inferior to UDCA for normalizing ALT level. Furthermore it was safe and well tolerated by patients with abnormal ALT.
Adolescent ; Adult ; Aged ; Alanine Transaminase/*blood ; Cholagogues and Choleretics/*therapeutic use ; Dioxoles/*therapeutic use ; Double-Blind Method ; Drug Administration Schedule ; Female ; Follow-Up Studies ; Hepatitis, Alcoholic/*drug therapy ; Hepatitis, Chronic/*drug therapy ; Humans ; Male ; Middle Aged ; Non-alcoholic Fatty Liver Disease/*drug therapy ; Tertiary Care Centers ; Treatment Outcome ; Ursodeoxycholic Acid/*therapeutic use ; Young Adult

No abstract available.
Antiviral Agents/therapeutic use ; Ascites/diagnosis/prevention & control/therapy ; Cholagogues and Choleretics/therapeutic use ; Fatty Liver/diagnosis/diet therapy ; Fatty Liver, Alcoholic/diagnosis/drug therapy ; Hemorrhage/prevention & control/therapy ; Hepatic Encephalopathy/diagnosis/prevention & control/therapy ; Hepatitis B, Chronic/diagnosis/drug therapy ; Hepatitis C, Chronic/diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/drug therapy/pathology/*therapy ; Liver Cirrhosis, Biliary/drug therapy ; Vasodilator Agents/therapeutic use

Nonalcoholic steatohepatitis (NASH) can progress into liver cirrhosis; however, no definite treatment is available. Omega-3 polyunsaturated fatty acid (omega-3) has been reported to alleviate experimental NASH, although its beneficial effect was not evident when tested clinically. Thus, this study aimed to investigate the additive effect of omega-3 and ursodeoxycholic acid (UDCA) on diet-induced NASH in mice. C57BL/6 mice were given a high-fat diet (HFD) for 24 weeks, at which point the mice were divided into three groups and fed HFD alone, HFD with omega-3 or HFD with omega-3 in combination with UDCA for another 24 weeks. Feeding mice an HFD and administering omega-3 improved histologically assessed liver fibrosis, and UDCA in combination with omega-3 further attenuated this disease. The assessment of collagen alpha1(I) expression agreed with the histological evaluation. Omega-3 in combination with UDCA resulted in a significant attenuation of inflammation whereas administering omega-3 alone failed to improve histologically assessed liver inflammation. Quantitative analysis of tumor necrosis factor alpha showed an additive effect of omega-3 and UDCA on liver inflammation. HFD-induced hepatic triglyceride accumulation was attenuated by omega-3 and adding UDCA accentuated this effect. In accordance with this result, the expression of sterol regulatory binding protein-1c decreased after omega-3 administration and adding UDCA further diminished SREBP-1c expression. The expression of inducible nitric oxide synthase (iNOS), which may reflect oxidative stress-induced tissue damage, was suppressed by omega-3 administration and adding UDCA further attenuated iNOS expression. These results demonstrated an additive effect of omega-3 and UDCA for alleviating fibrosis, inflammation and steatosis in diet-induced NASH.
Animals ; Cholagogues and Choleretics/pharmacology/*therapeutic use ; Diet, High-Fat/adverse effects ; Drug Synergism ; Fatty Acids, Omega-3/pharmacology/*therapeutic use ; Fibrosis/drug therapy/etiology/immunology/pathology ; Inflammation/drug therapy/etiology/immunology/pathology ; Liver/*drug effects/immunology/pathology ; Male ; Mice, Inbred C57BL ; Non-alcoholic Fatty Liver Disease/*drug therapy/etiology/immunology/pathology ; Ursodeoxycholic Acid/pharmacology/*therapeutic use

Biliary cast describes the presence of casts within the biliary tree. It is resultant sequel of cholangitis and hepatocyte damage secondary to bile stasis and bile duct injury. Biliary cast syndrome was first reported in patient undergone liver transplantation. The pathogenesis of biliary cast is not clearly identified, but proposed etiologic factors include post-transplant bile duct damage, ischemia, biliary infection, or post-operative biliary drainage tube. Although biliary casts are uncommon, most of biliary cast syndrome are reported in the liver transplant or hepatic surgery patients. A few reports have been published about non-transplant or non-liver surgery biliary cast. We report two cases of biliary cast syndrome in non-liver surgery patients.
Acute Disease ; Ascariasis/diagnosis ; Bile Duct Diseases/*diagnosis/ultrasonography ; Bile Ducts/ultrasonography ; Cholagogues and Choleretics/therapeutic use ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects ; Female ; Gallstones/diagnosis ; Humans ; Liver Cirrhosis, Biliary/diagnosis/drug therapy ; Male ; Middle Aged ; Pancreatitis/etiology ; Tomography, X-Ray Computed ; Ursodeoxycholic Acid/therapeutic use