Dehner L P, Snover D C, Sharp H L, Ascher N, Nakhleh R, Day D L
Department of Laboratory Medicine and Pathology (Division of Surgical Pathology), University of Minnesota Medical School and Hospital, Minneapolis 55455.
Hum Pathol. 1989 Feb;20(2):149-58. doi: 10.1016/0046-8177(89)90179-2.
Hereditary tyrosinemia type I presents with either acute hepatic failure in the neonatal period or later in infancy with progressive liver dysfunction secondary to cirrhosis. The inevitably fatal outcome in those children with the chronic form has been transformed with the advent of liver transplantation. Native livers from five children who received allografts were studied pathologically and compared with earlier hepatic biopsies in two of these patients that had been performed several years before transplantation. Our findings support the conclusion that a sequence of morphologic changes from the initial micronodular cirrhosis through an intermediate mixed cirrhotic pattern to macronodular cirrhosis occurs. The micronodular phase is transitory, over a period of only a few months, since mixed micronodular macronodular cirrhosis was already present in the livers of children who received transplants by 11 months of age. Focal hepatocellular dysplasia was present in one of the livers with mixed cirrhosis but was not identified in the other two cases. Macronodular cirrhosis accompanied two cases of hepatocellular carcinoma in this study. In order to preclude the latter complication, liver replacement is necessary before the age of 2 years.
I型遗传性酪氨酸血症在新生儿期表现为急性肝衰竭，或在婴儿期后期表现为继发于肝硬化的进行性肝功能障碍。随着肝移植的出现，那些患有慢性型疾病的儿童不可避免的致命结局得到了改变。对5名接受同种异体移植儿童的原生肝脏进行了病理研究，并与其中2名患者在移植前数年进行的早期肝活检进行了比较。我们的研究结果支持以下结论：从最初的小结节性肝硬化到中间的混合性肝硬化模式再到大结节性肝硬化，会发生一系列形态学变化。小结节期是短暂的，仅持续几个月，因为在11个月大时接受移植的儿童肝脏中已经出现了混合性小结节大结节性肝硬化。在其中一个混合性肝硬化的肝脏中存在局灶性肝细胞发育异常，但在其他两个病例中未发现。在本研究中，大结节性肝硬化伴有2例肝细胞癌。为了避免后一种并发症，必须在2岁之前进行肝脏置换。
