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Persistent Cholestatic Hepatitis after Choledocholithiasis Removal: A Rare Case of Vanishing Bile Duct Syndrome.

INTRODUCTION: Vanishing bile duct syndrome (VBDS) is a rare condition involving the progressive loss of intrahepatic bile ducts. CASE PRESE … itored for nearly 3 years, and the patient's transaminase levels consistently trended downward with ursodeoxycholic acid before she ultimately passed away from complications arising from other comorbidities. CONCLUSION: This case served as a rare and valuable example of amoxicillin contributing to VBDS, and with cessation of the offending drug and supportive care wit …

Coxiella burnetii as a possible cause of autoimmune liver disease: a case report.

INTRODUCTION: Q fever is a zoonotic infection that may cause severe hepatitis. Q-fever hepatitis has not yet been associated with autoimmune hepatitis and/or primary biliary cirrhosis. CASE PRESENTATION: We describe a 39-year-old man of Sri Lankan origin with chronic Q-fever hepatitis who developed autoantibodies compatible with autoimmune hepatitis/primary biliary cirrhosis overlap syndrome. Ursodeoxycholic acid in addition to antibiotic therapy markedly improved hepatic enzyme levels suggesting that autoimmunity, potentially triggered by the underlying infection, was involved in the pathoge…

Sclerosing cholangitis.

Primary sclerosing cholangitis in children can mimic autoimmune hepatitis in the absence of inflammatory bowel disease. Most adult patients … ear promising for confirming occult cholangiocarcinoma when present. The chemoprevention effects of ursodeoxycholic acid on colorectal dysplasia await further investigation. Initial results from high-dose ursodeoxycholic acid in halting disease progression, however, suggest a beneficial effect. In contrast, the impact of endoscopic therapy on natural history remains u …

A new cause of progressive intrahepatic cholestasis: 3 beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency.

There have been a few reports of infants with severe neonatal cholestasis related to a defect in primary bile acid synthesis. To assess the … the cases a similar disease was observed in siblings. Liver function returned to normal after oral ursodeoxycholic acid therapy. We conclude that 3 beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency should be considered when idiopathic cholestatic liver disease with clinical features akin to Byler disease is characterized by the association of normal serum g …

EFFICACY OF COMPREHENSIVE TREATMENT OF NONALCOHOLIC FATTY LIVER DISEASE IN PATIENTS WITH PREDIABETES.

OBJECTIVE: The aim: To evaluate the effectiveness of the proposed treatment recommendations, which included lifestyle changes, as well as the treatment with ursode¬oxycholic acid, rosuvastatin, and omega-3 PUFA, on the severity of cytolytic and cholestatic syndromes in patients with NAFLD and prediabetes. PATIENTS AND METHODS: Materials and methods: Fifty-five patients with confirmed prediabetes and concomitant NAFLD underwent a comprehensive clinical examination and were treated with rosuvastatin 10 mg/d, omega-3 PUFA at a dose of 1000 mg/d and ursodeoxycholic acid at a dose of 10 mg/kg/d. …

The role of lipophilic bile acids in the development of cirrhotic cardiomyopathy.

Marked hemodynamic changes occur in humans and experimental animals with cirrhotic liver disease. In the heart, basal contractility, respons … eared to result from altered ECC. beta-Adrenoceptor density and signaling were unaffected. Acutely, ursodeoxycholic acid, a more hydrophilic bile acid, had no effect. Cirrhosis produced a decrease in basal force, depressed beta-adrenoceptor responsiveness, and altered ECC similar to cholic acid. However, cirrhosis also altered beta-adrenoceptor signaling including dec …

Importance of bile acid structure in amelioration of griseofulvin-induced murine protoporphyric hepatopathy.

This study investigated the effects of bile acid structure on griseofulvin-induced murine hepatopathy and explored the mechanism(s) of cholestasis in this model of protoporphyria. Mice were fed pulverized chow with cholate, chenodeoxycholate, or ursodeoxycholate, with or without griseofulvin. After 1 to 4 weeks, bile flow, bile acid excretion and composition, biliary protoporphyrin excretion, hepatic protoporphyrin contents, liver histology, and griseofulvin plasma concentrations were determined. Additionally, bile acid absorption was measured. Griseofulvin induced a progressive increase in li

Наркоманное повреждение печени вторично для ритуксимаба.

DILI: травма печени, вызванная наркотиками, HBV: вирус гепатита B, LFT: тест функции печени, RRMS: повторное излучающее множественный склероз, UDCA: ursodeoxoxolic кислота. Rituximab является человеческим химером моноколональным антителом, нацеленным на CD20 на клетки B. Ежегодно на 100 000 человек приходится 19 случаев травмирования печени в результате ритуксимаба (ДИЛИ). Это заболевание характеризуется быстрым повышением уровней аминотрансферазы и гепатоклеточными повреждениями, главным образом в результате возобновления вируса гепатита В (ВБВ). Тем не менее имели место редкие случаи ДИЛИ, к

Совпадение в аннотации оригинала
DILI: drug-induced liver injury, HBV: Hepatitis B virus, LFT: liver function test, RRMS: Relapsing-remitting multiple sclerosis, UDCA: ursodeoxycholic acid. Rituximab is a human chimeric monoclonal antibody that targets CD20 on B cells. The incidence of rituximab-associated drug-induced liver injury (DILI) is 19 cases per 100 000 people annually. This condit…

[Approaches to the treatment of patients with climacteric disorders complicated with menopausal metabolic syndrome with cholestasis].

RESEARCH OBJECTIVE: Development of the individual comprehensive program of follow treatment of patients with climacteric disorders complicated MMS (menopausal metabolic syndrome) with cholestasis; on the basis of application of low-dose hormone replacement therapy in combination with ursodeoxycholic acid, to improve the quality of life. MATERIALS AND METHODS: We observed 101 woman with climacteric syndrome, obesity and cholestasis; conducted a comprehensive clinical and laboratory examination, ultrasound of the hepatobiliary system, measurement modified menopausal index (MMI), the measurement…

Effective therapy using voglibose for nonalcoholic steatohepatitis in a patient with insufficient dietary and exercise therapy: exploring other treatment possibilities.

A 56-year-old Japanese female with a 10-year history of thyroiditis presented to our institution. The laboratory data and clinical findings suggested that the patient had complicated nonalcoholic fatty liver disease or nonalcoholic steatohepatitis (NASH) with autoimmune hepatitis according to the criteria by the application of the International Autoimmune Hepatitis score. The patient could not manage by herself so dietary- and exercise-based treatment was difficult. Accordingly, ursodeoxycholic acid and ezetimibe therapy was started and continued until the performance of a liver needle biopsy …

Autoimmune hepatitis 2 years after the diagnosis of primary sclerosing cholangitis: an unusual overlap syndrome in a 17-year-old adolescent.

A 15-year-old girl was admitted in April 2004 owing to fatigue and loss of appetite. Her paediatrician had found elevated serum levels for alkaline phosphatase. The endoscopic retrograde cholangiography documented typical signs of primary sclerosing cholangitis with involvement of the small and large ducts. The liver biopsy revealed extensive septal and portal fibrosis. No evidence of inflammatory bowel disease was present. She was started on ursodeoxycholic acid therapy and improved clinically. After 22 months she presented again with rising transaminase levels up to 600 U/l. The second liver…

[Primary biliary liver cirrhosis and overlap syndrome. Diagnosis and therapy].

Primary biliary cirrhosis represents a chronic cholestatic liver disease of unknown etiology. It primarily affects females, is associated wi … t immunosuppressants, colchicine, d-penicillamine and methotrexate are of limited clinical benefit. Ursodeoxycholic acid has few side effects and leads to a biochemical response and a delay of disease progression in most cases. When ursodeoxycholic acid therapy is ineffective an overlap syndrome with autoimmune hepatitis can be present, which can respond to steroid tr …

[Clinical significance of bile acids].

During the last years bile acids have gained more and more clinical importance. They play a decisive part in intestinal fat resorption. Increased bile acid content in the colon will result in diarrhea. By determination of serum bile acids the liver function can be judged exactly. It seems probable that bile acids take part in the pathogenesis of gastritis gastric ulcer and colonic cancer. By administration of chenodeoxycholic acid and ursodeoxycholic acid dissolution of cholesterol stones within the gall bladder is possible.

Hepatic and extrahepatic glucuronidation of bile acids in man. Characterization of bile acid uridine 5'-diphosphate-glucuronosyltransferase in hepatic, renal, and intestinal microsomes.

Microsomal UDP-glucuronosyltransferase activity toward the bile acids (chenodeoxycholic, deoxycholic, ursodeoxycholic, lithocholic, and glyc … le acid structure-activity relationship existed, with highest activity observed for lithocholic and ursodeoxycholic acids, which was about twofold higher compared with chenodeoxycholic or deoxycholic acids. Lowest activity was estimated for glycolithocholic acid. UDP-glucuronosyltransferase activity toward chenodeoxycholic acid was studied in biopsy specimens of liver …

Geoepidemiology and changing mortality in primary biliary cholangitis.

Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic cholestatic disease characterized by an autoimmun … hondrial antibody made it possible to diagnose the disease at an earlier stage, and introduction of ursodeoxycholic acid therapy as the first-line therapy for PBC drastically changed PBC-related mortality. At present, patients with an early histological stage have survival rates similar to those of an age- and sex-matched control population. Although 30% of patients t …

Addition of N-acetylcysteine to aqueous model bile systems accelerates dissolution of cholesterol gallstones.

The organic matrix of cholesterol gallstones contains a macromolecular complex of mucin and bilirubin that may inhibit stone dissolution by limiting contact of desaturated bile with crystalline cholesterol. The goal of this study was to determine if the mucolytic agent N-acetylcysteine could accelerate gallstone dissolution in vitro. Paired gallstones were dissolved in either pure taurocholate (140 mM) or ursodeoxycholate (100 mM), or in bovine bile supplemented with either taurocholate or ursodeoxycholate to achieve the same respective bile-salt concentrations. N-acetylcysteine was added to 1

Current and potential treatments for primary biliary cholangitis.

Up to 40% of patients with primary biliary cholangitis have an incomplete response to first-line treatment with ursodeoxycholic acid. Obeticholic acid was approved by the US Food and Drug Administration in 2016 as a second-line treatment for patients with primary biliary cholangitis who are unresponsive to ursodeoxycholic acid; however, approximately 50% of patients might need additional treatments to reach therapeutic goals. A considerable need exists for effective treatment options to prevent progression to liver transplantation or death in these patients. Drugs that might modulate immunolog…

Progress in the Management of Patients with Cholestatic Liver Disease: Where Are We and Where Are We Going?

Cholestatic liver disease is a group of diseases in which bile acid accumulates in the liver for various reasons, resulting in abnormal liver biochemical indicators and histological damage. Cholestasis can be divided into intrahepatic cholestasis and extrahepatic cholestasis, which will contribute to liver damage and progress to liver fibrosis and cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis are the two most typical cholestatic liver diseases. Ursodeoxycholic acid is currently the first-line treatment for PBC, while obeticholic acid, budesonide and fibrates h…

Primary Biliary Cholangitis: advances in management and treatment of the disease.

Primary Biliary Cholangitis, previously known as Primary Biliary Cirrhosis, is a rare disease, which mainly affects women in their fifth to … n in diseases such as human immunodeficiency virus/Hepatitis C Virus coinfection and breast cancer. Ursodeoxycholic acid is the only treatment available today, but even if effective in counteracting the disease progression for the majority of patients, in approximately 40% is not able to decrease effectively the alkaline phosphatase, a surrogate marker of disease acti …

Obeticholic acid in primary biliary cholangitis.

In a double-blind, randomized, placebo-controlled study including 217 patients with primary biliary cholangitis, the authors show that obeticholic acid (a potent farnesoid X agonist) administered with ursodeoxycholic acid or as monotherapy significantly decreases serum alkaline phosphatase and bilirubin when compared to placebo. Pruritus (and serious adverse effects) was observed more frequently in obeticholic acid-treated patients than in controls, in spite of a decrease in serum bile acid concentration. These results are encouraging, but more studies are needed on clinical efficacy and safet…