Weinberg A G, Mize C E, Worthen H G
J Pediatr. 1976 Mar;88(3):434-8. doi: 10.1016/s0022-3476(76)80259-4.
A 5 1/2-year-old child with hepatocarcinoma complicating hereditary tyrosinemia is presented. A review of the literature and an attempted follow-up of previously reported patients with the chronic form of hereditary tyrosinemia have disclosed 16 cases of hepatocarcinoma occurring in 43 patients surviving beyond 2 years of age (37%). This incidence is considerably higher than that generally given for the occurrence of hepatoma in adults with macronodular cirrhosis. Females and males are equally at risk. Additional factors beyond the development of cirrhosis are likely operative in the induction of hepatocarcinoma in patients with this metabolic disorder; those surviving beyond infancy are at considerable risk for the development of fatal hepatic neoplasms.
本文报告了一名5岁半患遗传性酪氨酸血症并伴有肝癌的儿童。对文献的回顾以及对先前报道的慢性遗传性酪氨酸血症患者的随访尝试发现，在43名存活超过2岁的患者中有16例发生了肝癌（37%）。这一发病率远高于一般成人巨块型肝硬化患者肝癌的发病率。女性和男性患病风险相同。除了肝硬化的发展之外，其他因素可能在这种代谢紊乱患者肝癌的诱发中起作用；那些存活至婴儿期以后的患者发生致命性肝肿瘤的风险相当高。
