Background: Cholangiocarcinoma is an invasive biliary malignancy with poor prognosis. Diagnostic accuracy of conventional methods is low which is mainly due to the specific anatomy of the disease. The aim of this study was to evaluate the diagnostic value of biochemical profile and tumor marker of the bile in patients with malignant cholestasis compared to that of choledocholithiasis. ...

Recurrent jaundice due to intrahepatic cholestasis is reported most often in adults. Some varieties are familial and are associated with failure of excretion of conjugated bilirubin by the liver cell. The original descriptions of these types given by Rotor, Manahan, and Florentin (1948) and Dubin and Johnson (1954) concerned patients with a benign recurrent jaundice. The diseases described by t...

Twelve episodes of acute fatty liver of pregnancy (AFLP) were diagnosed in 11 patients during the past 18 years in a general hospital in Santiago, Chile, with a prevalence of 1 per 15,900 deliveries. Acute fatty liver of pregnancy started between the 31st and 38th weeks of pregnancy, with malaise, vomiting, jaundice, and lethargy as the main clinical manifestations. Polydipsia (in nine episodes...

Autoimmune hepatitis (AIH) has rarely been described as an autoimmune paraneoplastic syndrome of thymoma. This case is the seventh case of AIH revealed by cholestasis few years after the diagnosis of thymoma and the first case treated with chemotherapy alone. We report in this paper a new approach to this rare severe condition. A 29 year-old man presented with chest pain and dyspnea with a hist...

Progressive familial intrahepatic cholestasis (PFIC) includes a group of infrequent genetic diseases with autosomal recessive heredity, characterized by intrahepatic cholestasis, usually in childhood and in adolescence. It is caused by defective bile salt secretion and other bile components. The progression leading to liver failure and cirrhosis usually appears in the first few decades of life....

BACKGROUND Mutations in ATP8B1 gene were identified as a cause of low γ-glutamyltranspeptidase cholestasis with variable phenotype, ranging from Progressive Familial Intrahepatic Cholestasis to Benign Recurrent Intrahepatic Cholestasis. However, only the coding region of ATP8B1 has been described. The aim of this research was to explore the regulatory regions, promoter and 5'untranslated region...

Many patients with biliary atresia (BA) have impaired metabolism of copper (Cu) and zinc (Zn) because of the obstruction of bile ducts. An excessive Cu accumulation is cytotoxic and results in fibrosis in hepatic tissues. Since Zn works antagonistically to Cu, lower Zn concentrations may deteriorate liver damage. In the 1980's, we performed a series of surgeries on BA patients for the construct...

PURPOSE Benign recurrent intrahepatic cholestasis (BRIC) is characterized by episodic cholestasis and pruritus without anatomical obstruction. The aim of this study was to evaluate the safety and efficacy of nasobiliary drainage (NBD) in patients with BRIC refractory to medical therapy and to determine whether the use of NBD prolongs the episode duration. METHODS This was a multicenter retros...