CAJ | 학술논문

遗传代谢病常以肝大或肝功能不全为主要表现。溶酶体病及金属代谢异常疾病多伴有肝大或肝功能不全。婴儿胆汁淤积性肝病也是一大类以肝功能损害为主要表现的疾病。加深对这类疾病的认识，有助于早期诊断、早期干预、改善预后。
유전대사병상이간대혹간공능불전위주요표현。용매체병급금속대사이상질병다반유간대혹간공능불전。영인담즙어적성간병야시일대류이간공능손해위주요표현적질병。가심대저류질병적인식，유조우조기진단、조기간예、개선예후。
Hepatomegaly and hepatic insufficiency are commonly seen in inherited metabolic disease. Patients with lysosomal disease and metal metabolic disorder often present hepatomegaly or hepatic insufficien-cy. Infant cholestasis hepatopathy is another type of disease characterized with hepatic functional lesion. There-fore,fully understanding this kind of disease would be helpful for early diagnosis,early intervention and progno-sis improvement.