Disodium gadoxetate uptake in progressive familial intrahepatic cholestasis type I: Enhancing our understanding of the cholestatic disease
نویسندگان
چکیده
Hepatocyte-specific magnetic resonance imaging (MRI) contrast agents are commonly used to depict anatomic hepatobiliary lesions and are also useful in characterizing the kinetics of hepatocyte uptake and excretion. We report a case of a 13-year old female with progressive familial intrahepatic cholestasis (PFIC) type 1 who demonstrated decreased uptake and excretion of gadoxetate disodium contrast material. This case illustrates the challenge of imaging children with cholestasis using hepatobiliary-specific contrast agents; we propose an alternative explanation for the delayed excretion that may be related to the underlying genetic defect of this child.
منابع مشابه
Transcriptional regulation of hepatobiliary transport systems in health and disease: implications for a rationale approach to the treatment of intrahepatic cholestasis.
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...
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