The pathophysiology of Wilson's disease visualized: A human Cu PET study.

Background And Aims: Wilson's disease (WD) is a genetic disease with systemic accumulation of copper that leads to symptoms from the liver and brain. However, the underlying defects in copper transport kinetics are only partly understood. We sought to quantify hepatic copper turnover in patients with WD compared with heterozygote and control subjects using PET with copper-64 ( Cu) as a tracer.

Hepatic bile acid transport increases in the postprandial state: A functional C-CSar PET/CT study in healthy humans.

Background & Aims: It is not known how hepatic bile acids transport kinetics changes postprandially in the intact liver. We used positron emission tomography (PET)/computed tomography (CT) with the tracer [-methyl-C]cholylsarcosine (C-CSar), a synthetic sarcosine conjugate of cholic acid, to quantify fasting and postprandial hepatic bile acid transport kinetics in healthy human participants.

Methods: Six healthy human participants underwent dynamic liver C-CSar PET/CT (60 min) during fasting and from 15 min after ingestion of a standard liquid meal.

Obeticholic acid improves hepatic bile acid excretion in patients with primary biliary cholangitis.

Background & Aims: Obeticholic acid (OCA) is an agonist of the nuclear bile acid receptor farnesoid X receptor, which regulates hepatic bile acid metabolism. We tested whether OCA treatment would influence hepatic transport of conjugated bile acids in patients with primary biliary cholangitis (PBC) who responded inadequately to treatment with ursodeoxycholic acid (UDCA).

Methods: Eight UDCA-treated patients with PBC with alkaline phosphatase ≥1.

Intravenous and oral copper kinetics, biodistribution and dosimetry in healthy humans studied by [Cu]copper PET/CT.

Purpose: Copper is essential for enzymatic processes throughout the body. [Cu]copper (Cu) positron emission tomography (PET) has been investigated as a diagnostic tool for certain malignancies, but has not yet been used to study copper homeostasis in humans. In this study, we determined the hepatic removal kinetics, biodistribution and radiation dosimetry of Cu in healthy humans by both intravenous and oral administration.