Day D L, Letourneau J G, Allan B T, Sharp H L, Ascher N, Dehner L P, Thompson W M
AJR Am J Roentgenol. 1987 Aug;149(2):391-3. doi: 10.2214/ajr.149.2.391.
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.
遗传性酪氨酸血症是一种常染色体隐性酶紊乱疾病，可在儿童早期导致微结节性和大结节性肝硬化。约三分之一的患病儿童会发生肝细胞癌。我们评估了5名患有该疾病儿童的影像学检查结果。对所有5例肝脏的病理检查均显示有肝硬化和多个再生结节；5例肝脏中有2例存在肝细胞癌。所有5例患者肝脏内均有高密度或高低密度灶。在4次肝脏超声检查中的3次或2次肝脏核素扫描中的1次中，这些高密度灶并未表现为局灶性病变。血管造影显示1例局灶性肝细胞癌患者存在肿瘤血管，但另1例患有严重肝硬化和多灶性肝细胞癌的患者血管造影结果不明确。酪氨酸血症所致肝硬化患儿可能会出现再生结节，在CT扫描上表现为肝脏高密度灶。在这些患者中，很难将再生结节与多灶性肝细胞癌区分开来。
