Chang M H, Hsu H C, Lee C Y, Wang T R, Kao C L
Department of Pediatrics, National Taiwan University, Republic of China.
J Pediatr Gastroenterol Nutr. 1987 Mar-Apr;6(2):203-7. doi: 10.1097/00005176-198703000-00006.
Fifty-six patients with moderate to severe neonatal hepatitis were followed for 12 to 78 months. Two died from causes other than hepatitis itself and were free from liver disease at the time of death. Of the remaining 54 patients, seven died of hepatitis, two are living with chronic liver disease and psychomotor retardation, and 45 are living without liver disease. High peak bilirubin levels and liver histologic findings of periportal fibrosis, moderate to severe portal inflammation, and/or diffuse giant cell transformation appear to be major factors predictive for poor outcome. Cytomegalovirus (CMV) infection was a common associated infection. Evidence of CMV infection was found in 22 (49%) of the 45 patients studied. Three of them died, and one is still living with cirrhosis of the liver. Metabolic disorders such as alpha-1-antitrypsin deficiency, galactosemia, and aminoaciduria and/or aminoacidemia were carefully screened but were not found in these cases. A fatal case had a sibling who had died of a similar disease course. Chinese infants may have metabolic and familial cholestasis diseases requiring further investigation.
56例中重度新生儿肝炎患者接受了12至78个月的随访。2例死于肝炎本身以外的原因，死亡时无肝脏疾病。其余54例患者中，7例死于肝炎，2例患有慢性肝病并伴有精神运动发育迟缓，45例无肝脏疾病存活。高胆红素峰值水平以及门周纤维化、中度至重度门脉炎症和/或弥漫性巨细胞转化的肝脏组织学表现似乎是预后不良的主要预测因素。巨细胞病毒（CMV）感染是常见的相关感染。在研究的45例患者中，22例（49%）发现了CMV感染证据。其中3例死亡，1例仍患有肝硬化。对α-1-抗胰蛋白酶缺乏症、半乳糖血症和氨基酸尿症和/或氨基酸血症等代谢紊乱进行了仔细筛查，但在这些病例中未发现。1例死亡病例有1名死于相似病程疾病的同胞。中国婴儿可能患有需要进一步调查的代谢性和家族性胆汁淤积疾病。
