Liver disorders are more diverse in children than in adults, and autoimmune liver diseases also develop in childhood, although rarely. The autoimmune diseases in children comprise autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). The pathology of AIH and PSC is described. Although AIH in children is rare, it occurs in early childhood, and some elementary school students have been reported to develop cirrhosis. The histology of AIH in children is essentially the same as that in adults. We analyzed eight patients with childhood AIH. Four of these patients had a high AIH score, with typical histological features of AIH, that is, interface hepatitis with infiltration of lymphocytes and plasma cells, a severe necroinflammatory reaction and rosette formation of hepatocytes. Multinucleated hepatocytes were observed in three patients. This finding seems characteristic of childhood AIH, although rarely observed in adult AIH. Clinically, the distinction between AIH and PSC is often difficult in childhood, and the overlapping of both has also been reported. PSC-like histological features may be observed in some pediatric patients with AIH. In patients with acute onset of AIH, they show a pronounced necroinflammatory reaction in zone 3 (central area). Because an autoimmune phenomenon may occur in the early stage of childhood PSC, it is difficult to differentiate it from AIH in some patients. Some patients are diagnosed with AIH in the early stage, but with PSC during long-term follow up. The histopathological findings of childhood PSC are the same as those of adult PSC, and are characterized by biliary-type portal fibrosis and onion-like periductal fibrosis in medium-sized portal tracts.

译文

儿童的肝脏疾病比成人更加多样化,自身免疫性肝病也在儿童时期发展,尽管很少。儿童自身免疫性疾病包括自身免疫性肝炎 (AIH) 和原发性硬化性胆管炎 (PSC)。描述了AIH和PSC的病理。虽然儿童的AIH很少见,但它发生在儿童早期,据报道一些小学生会发展为肝硬化。儿童AIH的组织学与成人基本相同。我们分析了8例儿童AIH患者。这些患者中有4例具有较高的AIH评分,具有典型的AIH组织学特征,即界面肝炎伴淋巴细胞和浆细胞浸润,严重的坏死性炎症反应和肝细胞玫瑰花结形成。在三名患者中观察到多核肝细胞。这一发现似乎是儿童AIH的特征,尽管在成人AIH中很少观察到。临床上,AIH和PSC之间的区别在儿童时期通常很困难,并且也有报道称两者重叠。在某些小儿AIH患者中可能会观察到类似PSC的组织学特征。在AIH急性发作的患者中,他们在3区 (中央区域) 显示出明显的坏死性炎症反应。由于自身免疫现象可能发生在儿童PSC的早期,因此在某些患者中很难将其与AIH区分开。一些患者在早期被诊断为AIH,但在长期随访中被诊断为PSC。儿童PSC的组织病理学发现与成人PSC相同,其特征是胆道型门静脉纤维化和中型门静脉道的洋葱状导管周围纤维化。

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