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作者对过去15年间因新生儿期开始的胆汁淤积而入院的17名儿童进行了随访,并对可能的病程及发病因素进行了探讨。除1例妊娠36周外,其余16例均足月分娩。男性8例、女性9例。6例出生体重少于3公斤。11例在生后3天内、3例在生后3~10天间、2例在生后3~4周间首次发现黄疸。有1例于6岁时出现搔痒但临床上始终无黄疸。所有儿童均有胆汁淤积的症状和体征,而且绝大部分人具有胆汁淤积生化改变的证据。对其中持续存在胆汁淤积的临床和生化证据之14例儿童进行了削腹探查,手术性胆管造影发现11例从胆囊到十二指肠的肝外胆道系统是通畅的,但有狭窄。4
The authors followed up 17 children hospitalized for cholestasis during the past 15 years in the neonatal period and discussed the possible course of the disease and the risk factors. In addition to a case of 36 weeks of pregnancy, the remaining 16 cases were full-term delivery. 8 males and 9 females. 6 cases birth weight less than 3 kg. 11 cases within 3 days after birth, 3 cases in 3 to 10 days after birth, 2 cases in the first 3 to 4 weeks after birth, jaundice was found. One patient had itch at the age of 6 but no jaundice in the clinic. All children have signs and symptoms of cholestasis, and the vast majority of people have evidence of biochemical changes in cholestasis. Hysterectomy was performed in 14 children with clinical and biochemical evidence of persistent cholestasis. Surgical cholangiography showed that 11 cases of extrahepatic biliary tract from the gallbladder to the duodenum had an open but narrow stenosis. 4