Hepatic regenerating nodules in hereditary tyrosinemia.
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.
Day
D L
DL
Letourneau
J G
JG
Allan
B T
BT
Sharp
H L
HL
Ascher
N
N
Dehner
L P
LP
Thompson
W M
WM
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
D014443:Tyrosine