Duchnowska A, Wach K, Poradowska W
Probl Med Wieku Rozwoj. 1979;8:84-91.
Lipoprotein-X (LP-X) in the serum of infants with persistent jaundice is indicative of cholestasis. In early infancy biliary atresia and biliary agenesis are the most common cause of cholestasis, whereas neonatal hepatitis is a less frequent cause of cholestasis. The authors introduced and described the qualitative and quantitative methods of LP-X determination for diagnostic purposes. LP-X estimations were carried out in 9 children with persistent jaundice. LP-X was found to be present in 4 infants-in 2 with complete absence of extrahepatic biliary tracts, in 1 with extrahepatic biliary atresia and in 1 with hypoplastic extrahepatic biliary tract. LP-X was also found in a 5 year old boy with mechanical occlusion of bile ducts caused by a malignant tumor ( rhabdomyoblastoma ), and in 3 year old girl with inborn enzymatic liver dysfunction. In this case LP-X concentration was estimated before and after 3 week course of cholestyramine, after which there was a 35% decrease in the LP-X concentration. In a 4 month old child LP-X was not found in spite of the absence of extra and intrahepatic biliary tracts. This finding may be explained by the far advanced hepatic cirrhosis. The authors stress the importance of introducing of LP-X estimation in the differential diagnosis of jaundice in early infancy.
持续性黄疸婴儿血清中的脂蛋白-X(LP-X)提示胆汁淤积。在婴儿早期,胆道闭锁和胆管发育不全是胆汁淤积最常见的原因,而新生儿肝炎是胆汁淤积较不常见的原因。作者介绍并描述了用于诊断目的的LP-X测定的定性和定量方法。对9例持续性黄疸患儿进行了LP-X评估。在4例婴儿中发现了LP-X,其中2例肝外胆管完全缺如,1例肝外胆道闭锁,1例肝外胆管发育不良。在1例因恶性肿瘤(横纹肌肉瘤)导致胆管机械性梗阻的5岁男孩以及1例先天性酶性肝功能障碍的3岁女孩中也发现了LP-X。在这种情况下,在服用消胆胺3周疗程前后对LP-X浓度进行了评估,之后LP-X浓度下降了35%。在1例4个月大的儿童中,尽管肝内外胆管均缺如,但未发现LP-X。这一发现可能是由于晚期肝硬化所致。作者强调在婴儿早期黄疸的鉴别诊断中引入LP-X评估的重要性。