Molecular regulation of hepatobiliary transport systems: clinical implications for understanding and treating cholestasis.

[benign recurrent intrahepatic cholestasis]

Hepatobiliary transport systems are responsible for hepatic uptake and excretion of bile salts and other biliary constituents (eg , bilirubin ) into bile . Hereditary transport defects can result in progressive familial and benign recurrent intrahepatic cholestasis . Exposure to acquired cholestatic injury (eg , drugs , hormones , proinflammatory cytokines , biliary obstruction or destruction ) also results in altered expression and function of hepatic uptake and excretory systems , changes that may maintain and contribute to cholestasis and jaundice . Recruitment of alternative efflux pumps and induction of phase I and II detoxifying enzymes may limit hepatic accumulation of potentially toxic biliary constituents in cholestasis by providing alternative metabolic and escape routes . These molecular changes are mediated by bile salts , proinflammatory cytokines , drugs , and hormones at a transcriptional and posttranscriptional level . Alterations of hepatobiliary transporters and enzymes are not only relevant for a better understanding of the pathophysiology of cholestatic liver diseases , but may also represent important targets for pharmacotherapy . Drugs (eg , ursodeoxycholic acid , rifampicin ) used to treat cholestatic liver diseases and pruritus may counteract cholestasis via stimulation of defective transporter expression and function . In addition , therapeutic strategies may be aimed at supporting and stimulating alternative detoxification pathways and elimination routes for bile salts in cholestasis .

Diseases
Validation

Diseases presenting "hormones" symptom

benign recurrent intrahepatic cholestasis

dentin dysplasia

dentinogenesis imperfecta

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