title: Hepatic regenerating nodules in hereditary tyrosinemia.
abstract: Hereditary tyrosinemia is an autosomal recessive, enzymatic disorder that results in micro- and macronodular cirrhosis in early childhood. Hepatocellular carcinoma occurs in approximately one-third of affected children. We evaluated the imaging studies performed in five children with this disorder. Pathologic examination of all five of the livers revealed cirrhosis and multiple regenerating nodules; hepatocellular carcinoma was present in two of the five livers. All five patients had high-attenuation or high- and low-attenuation foci within the liver. These high-attenuation foci were not apparent as focal lesions in three of four hepatic sonograms or in one of two hepatic nuclear scans. Angiography showed tumor vascularity in one patient with a focal hepatocellular carcinoma, but was indeterminate in a second patient with severe cirrhosis and multifocal hepatocellular carcinoma. Children with cirrhosis due to tyrosinemia may develop regenerating nodules that appear as high-attenuation hepatic foci on CT scans. It is difficult to differentiate regenerating nodules from multifocal hepatocellular carcinoma in these patients.
authors.forename: D L
authors.initials: DL
authors.lastname: Day
authors.forename: J G
authors.initials: JG
authors.lastname: Letourneau
authors.forename: B T
authors.initials: BT
authors.lastname: Allan
authors.forename: H L
authors.initials: HL
authors.lastname: Sharp
authors.forename: N
authors.initials: N
authors.lastname: Ascher
authors.forename: L P
authors.initials: LP
authors.lastname: Dehner
authors.forename: W M
authors.initials: WM
authors.lastname: Thompson
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D000792:Angiography; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000209:etiology; D008113:Liver Neoplasms / Q000209:etiology; D008297:Male; D011877:Radionuclide Imaging; D014057:Tomography, X-Ray Computed; D014443:Tyrosine / Q000097:blood*; D014463:Ultrasonography
chemical_list: D014443:Tyrosine