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先天性黄疸又称为体质性黄疸,是由于胆红素代谢功能缺陷所致的慢性黄疸。有时诊断较为困难或与其他原因引起的肝内胆汁郁积性黄疸相混淆,因此,对此类黄疸必须有明确认识,以便与其他类型黄疸加以鉴别。先天性黄疸根据胆红质代谢缺陷的机制不同分为四类型:1.先天性非溶血性黄疸间接胆红质增高Ⅰ型(Gilbert氏综合征);2.先天性非溶血性黄疸间接胆红质增高Ⅱ型(CriglerNajjar氏综合征);3.先天性非溶血性黄疸直接胆红质增高Ⅰ型(Dubin Johnson氏综合征);4.先天性非溶血性黄疸直接胆红质增高Ⅱ型(Rotor氏综合征)。上述类型黄疸的特点为家族遗传性,除Crigler-Najjar氏综合征外,均有长期持续性或波动性黄疸存在,临床症状轻微,肝功能无明显损害。
Congenital jaundice, also known as physical jaundice, is a chronic jaundice caused by a defect in bilirubin metabolism. Sometimes the diagnosis is more difficult or with other causes of intrahepatic cholestasis of jaundice confused, therefore, such jaundice must have a clear understanding, in order to be identified with other types of jaundice. Congenital jaundice according to the mechanism of bilirubin metabolism defects are divided into four types: 1. Congenital non-hemolytic jaundice indirect hypercholesterolemia type Ⅰ (Gilbert’s syndrome); 2. Congenital non-hemolytic jaundice indirect bile red Quality increased type Ⅱ (CriglerNajjar’s syndrome); 3 congenital non-hemolytic jaundice direct hypercholesterolemia type Ⅰ (Dubin Johnson’s syndrome); 4 congenital non-hemolytic jaundice direct bilirubin type Ⅱ ( Rotor’s syndrome). The above types of jaundice are characterized by family heritability. Except Crigler-Najjar’s syndrome, there are long-term persistent or fluctuating jaundice, with mild clinical symptoms and no obvious impairment of liver function.