tetracycline and Alagille-Syndrome

We encountered a 9-year-old Japanese girl with Alagille syndrome. Her scintigraphic examinations of the liver were performed at the ages of 16 months and 9 years. 99mTc-PMT, a hepatobiliary imaging agent, was distributed homogeneously in the liver at the younger age, but unevenly produced an area of focally increased uptake in the medial segment of the liver surrounded by peripheral atrophy at the older age. 99mTc-GSA, a hepatoreceptor binding agent, was highly accumulated in the area, corresponding to the focally increased uptake of 99mTc-PMT. These imaging findings suggest that the pathophysiological and morphological changes of the liver occurred in our patient during the clinical course.

Topics: Alagille Syndrome; Child; Disease Progression; Female; Humans; Infant; Liver; Organotechnetium Compounds; Pyrrolidines; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Aggregated Albumin; Technetium Tc 99m Pentetate; Tetracycline

A child with Alagille syndrome, characterized by intrahepatic bile duct paucity, developed severe liver cirrhosis and was referred for liver transplantation. In the pre-transplantation evaluation, scintigraphic scans were performed using 99mTc-galactosyl serum albumin (99mTc-GSA) as a hepatoreceptor binding agent and 99mTc-pyridoxyl-5-methyl-tryptophan (99mTc-PMT) as a hepatobiliary agent. These studies demonstrated severe hepatobiliary dysfunction with an area of increased focal uptake in the liver. Histological examination at surgery confirmed that this focal lesion was an area of compensatory hyperplasia in advanced biliary cirrhosis. We present the usefulness of these tracers for detecting the focal hyperplasia of the liver.

Topics: Alagille Syndrome; Child; Humans; Hyperplasia; Liver; Liver Cirrhosis; Male; Organotechnetium Compounds; Pyrrolidines; Radionuclide Imaging; Technetium Tc 99m Aggregated Albumin; Technetium Tc 99m Pentetate; Tetracycline