POSTINFANTILE GIANT-CELL TRANSFORMATION IN HEPATITIS
POSTINFANTILE GIANT-CELL TRANSFORMATION IN HEPATITIS
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DOI：
10.1002/hep.1840160208
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发表时间：
1992-08-01
期刊：
影响因子：
13.5
通讯作者：
ISHAK, KG
中科院分区：
文献类型：
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作者：
DEVANEY, K;GOODMAN, ZD;ISHAK, KG
巨细胞肝炎是新生儿常见的肝损伤类型，但在婴儿期后很少见。这些病例被归因于自身免疫性疾病、非甲非乙型肝炎，以及最近的副粘病毒感染。为了更好地确定婴儿后(合体)巨细胞肝炎的实体，我们回顾了来自20名有这种发现的患者的24份活组织标本，无论是单独的还是与其他诊断相结合的。不同标本的多核巨细胞数量差异很大。除一名患者外，所有患者均出现不同程度的门脉炎症，且所有患者均有与肝细胞损伤相关的肝炎性腺泡炎症。纤维化是一种常见的发现，从轻微的门脉周围纤维化到已确诊的肝硬变(33%)。这些变化被解释为25%的急性巨细胞性肝炎，42%的CAH和其余的活动性肝硬变。患者年龄2~80岁，平均35岁，男女之比约为1：1。多数患者出现肝病症状和体征超过1个月。其中7例患者的抗核抗体效价为阳性。三名患者有直接的库姆斯反应和贫血。总体而言，在40%的患者中发现了自身免疫病的证据。一名患者患有累及肝脏的非霍奇金淋巴瘤。只有一名患者有输血史或丙型肝炎的危险因素，没有患者接受了副粘病毒抗体的血清学研究。对一名患者的肝组织进行了超微结构检查，但没有发现病毒颗粒。其中17名患者获得了随访信息。4人死亡(其中一个原因与肝病无关)；其中一名幸存患者接受了成功的原位肝移植。看来，婴儿后巨细胞肝炎最好被认为是一种不寻常的反应模式，在急性和慢性肝炎中都可能发生。在我们的系列中，最常见的潜在原因是自身免疫性疾病，这可能对许多此类患者的治疗具有重要意义。
Giant-cell hepatitis is a frequent pattern of liver injury in the neonate, but it is rare after infancy. Such cases have been attributed to autoimmune disease, to non-A, non-B hepatitis and, most recently, to paramyxovirus infection. To better define the entity of postinfantile (syncytial) giant-cell hepatitis, we reviewed 24 biopsy specimens from 20 patients with this finding, either alone or in combination with other diagnoses. The number of multinucleated giant cells varied greatly from one specimen to another. Varying degrees of portal inflammation appeared in all but one of the patients, and all had hepatitislike acinar inflammation associated with hepatocellular injury. Fibrosis was a common finding, varying from mild periportal fibrosis to established cirrhosis (33%). The changes were interpreted as acute giant-cell hepatitis in 25%, as CAH in 42% and as active cirrhosis in the remainder. The patients ranged in age from 2 to 80 yr, with a mean of 35 yr and a male/female ratio of approximately 1:1. The signs and symptoms of liver disease were present for more than 1 mo in most patients. A positive antinuclear antibody titer was found in seven of the patients. Three patients had a direct Coombs reaction and anemia. Overall, evidence of autoimmune disease was found in 40% of the patients. One patient had non-Hodgkin's lymphoma involving the liver. Only one patient had a history of blood transfusion or risk factors for hepatitis C. No patient underwent serological study for paramyxovirus antibodies. Liver tissue from one patient was examined ultrastructurally, but no viral particles could be identified. Follow-up information was available in 17 of the patients. Four had died (one of causes unrelated to liver disease); one of the surviving patients underwent successful orthotopic liver transplantation. It would appear that postinfantile giant-cell hepatitis is best regarded as an unusual reaction pattern that can occur in both acute and chronic hepatitis. The most frequently identified underlying cause in our series was autoimmune disease, which may have significant implications for treatment of many of these patients.