Elevated serum level of bilirubin is a common manifestation which is occurred in several diseases. Hyperbilirubinemia can manifest either conjugated or unconjugated. Conjugated or direct hyperbilirubinemia usually are caused by hepatocellular diseases or cholestatic liver diseases. Merely conjugated hyperbilirubinemia is the main manifestation of two congenital syndromes, including Dubin-Johnso...

Hepatobiliary transport systems mediate hepatic uptake and biliary excretion of bile acids, bilirubin and other biliary constituents. Hereditary or acquired defects of these transporters may cause or maintain cholestasis and jaundice under various clinical conditions including progressive familial intrahepatic cholestasis (PFIC) 1-3 or its milder forms, benign recurrent intrahepatic cholestasis...

Cholestasis, or impaired bile flow, is an important but poorly understood manifestation of liver disease. Two clinically distinct forms of inherited cholestasis, benign recurrent intrahepatic cholestasis (BRIC) and progressive familial intrahepatic cholestasis type 1 (PFIC1), were previously mapped to 18q21. Haplotype analysis narrowed the candidate region for both diseases to the same interval...

BACKGROUND Intrahepatic cholestasis of pregnancy (ICP) affects approximately 0.7% of pregnancies in the UK and is associated with prematurity, fetal distress, and intrauterine death. Homozygous mutations in the ATP8B1 gene cause cholestasis with a normal serum gamma-glutamyl transpeptidase (gamma-GT), and have been reported in two forms of cholestasis: progressive familial intrahepatic cholesta...

INTRODUCTION Cholestasis related pruritus, secondary to intrahepatic and/or extrahepatic biliary obstruction is a common manifestation in chronic liver disease. Pruritus is difficult to treat, and results are often suboptimal. A stepwise medical approach is usually employed, followed by a trial molecular adsorbents recirculation system in medication resistant cases. Pruritus resulting in reduce...

Familial intrahepatic cholestases (FICs) are a heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. Three distinct forms are described: FIC1 and FIC2, associated with low/normal GGT level in serum, which are caused by impaired bile salt secretion due to defects in ATP8B1 encoding the FIC1 protein and...

OBJECTIVE Cystatin C is a very potent inhibitor of cysteine proteinases and, it has been clinically applied as a sensitive marker in monitoring of renal and liver functions. The aim of this study was to reveal whether cystatin C may be a useful marker for distinguishing intra- versus extrahepatic cholestasis. MATERIALS AND METHODS Serum cystatin C concentrations were determined by nephelometr...