technetium-tc-99m-disofenin and Hyperbilirubinemia--Hereditary

Rotor syndrome is a rare, benign familial disorder characterized by chronic fluctuating, nonhemolytic and predominantly conjugated hyperbilirubinemia with normal hepatic histology. In contrast to Dubin-Johnson syndrome, there is no liver pigmentation in Rotor syndrome. A 36-year-old man was admitted due to asymptomatic persistent jaundice. His siblings had jaundice with direct hyperbilirubinemia. Physical examination revealed icteric sclerae without hepatosplenomegaly. Laboratory findings showed increased serum bilirubin with direct bilirubinemia. Hepatic uptake and storage capacity of indocyanine green was markedly reduced, while excretion into bile was slightly suppressed. Markedly decreased hepatic uptake and poor visualization of the gallbladder and biliary tract were shown in 99mTc-DISIDA scan. Histology of the liver showed mild steatosis without pigmentation. We report a case with the review of literature.

Topics: Adult; Coloring Agents; Humans; Hyperbilirubinemia, Hereditary; Indocyanine Green; Jaundice, Chronic Idiopathic; Liver; Liver Function Tests; Male; Radionuclide Imaging; Radiopharmaceuticals; Technetium Tc 99m Disofenin

Topics: Bilirubin; Cholecystography; Female; Hepatitis C; Humans; Hyperbilirubinemia, Hereditary; Infant; Infant, Newborn; Jaundice, Neonatal; Male; Pakistan; Prospective Studies; Radiographic Image Enhancement; Radionuclide Imaging; Risk Assessment; Sensitivity and Specificity; Severity of Illness Index; Technetium Tc 99m Diethyl-iminodiacetic Acid; Technetium Tc 99m Disofenin

The hepatic clearance of Tc-99m labeled iminodiacetic acid (IDA) compounds is believed to be impaired in patients with severe hyperbilirubinemia. Competitive inhibition of hepatocyte uptake of IDA by bilirubin has been demonstrated in vitro, but not by clinical scintigraphy. We present a patient with Crigler-Najjar syndrome without evidence of hepatobiliary damage, who demonstrated normal uptake and excretion of Tc-99m DISIDA despite a serum indirect bilirubin level in excess of 30 mg/dl. It is therefore suggested that a markedly elevated serum bilirubin level per se does not inhibit hepatic uptake of Tc-IDA and does not preclude clinically useful scintigraphic examination.

Topics: Child; Crigler-Najjar Syndrome; Humans; Hyperbilirubinemia, Hereditary; Imino Acids; Liver; Male; Organometallic Compounds; Radionuclide Imaging; Technetium; Technetium Tc 99m Disofenin