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肝脏遗传性的缺陷分三大类: 一、功能性肝脏缺陷,组织学正常:遗传性高血氨症,急性间歇性卟啉病,Gilbert综合征。二、功能性肝脏缺陷,具特征性,非进行性的组织病理学改变:D-J综合征,醣原累积症。三、遗传性肝脏缺陷,有进行性慢性肝病(并可发展为肝硬化):血色病,Wilson病,α_1-抗胰蛋白酶缺乏,原卟啉病。遗传性高血氨症肝脏的功能和组织学正常,但血氨增高。正常人血氨不超过1微克/毫升、遗传性高氨血症可达2～
Hereditary defects in the liver divided into three categories: First, functional liver defects, histological normal: hereditary hyperacetinosis, acute intermittent porphyria, Gilbert syndrome. Second, functional liver defects, with characteristic, non-progressive histopathological changes: D-J syndrome, glycogenosis. Third, hereditary liver defects, progressive chronic liver disease (and can develop into cirrhosis): hemochromatosis, Wilson’s disease, α_1-antitrypsin deficiency, protoporphyria. Hepatic hereditary hematologic liver function and histology normal, but increased blood ammonia. Normal blood ammonia does not exceed 1 microgram / ml, hereditary hyperacesemia up to 2 ~