Lachmann D
Wien Klin Wochenschr Suppl. 1977;69:3-28.
Abnormal lipoprotein (LP-X) represents a specific parameter for the presence of obstructive jaundice in the adult. Since LP-X has also been detected in the serum of newborn infants, both full-term and premature, and in early infancy, in the absence of clinical evidence of obstructive jaundice, extensive investigations were undertaken in infants during the neonatal period to clarify this phenomenon. The present study reports the data obtained in over 2000 sera from over 370 infants (mature newborn and premature newborn and young infants), tested more or less continuously by means of the Rapidophor method, initially on a qualitative, and subsequently, on a semi-quantitative basis. LP-X appears within the first fortnight in newborn infants, irrespective of the mode of feeding. The LP-X concentration was correlated to the birth weight. Premature infants displaying signs of immaturity possessed markedly higher LP-X levels than mature newborn infants. LP-X was not correlated to the alkaline phosphatase level, nor to the gammaglutamyl transferase activity; the bilirubin level, likewise, had no connection with the LP-X concentration. Patients with proven obstructive jaundice showed distinctly higher LP-X concentrations (greater than 56 mg/100 ml), whereby the rise in LP-X level in some cases preceded the appearance of the clinical manifestations of obstructive jaundice. The following hypotheses are advanced in order to explain the presence of LP-X during the neonatal period and are discussed on the basis of clinical observations in adults, the physiological conditions in the newborn infant and the results of the present study: The liver, which occupies the central position amongst metabolic organs, also in the case of the lipoproteins, is at a physiological stage of organic and functional maturation during this early period of life. Under these circumstances, a pseudo-obstructive mechanism on the basis of insufficient excretion of biliary lipoproteins, in conjunction with a simultaneous "physiological" deficiency of lecithin: cholesterol acyl transferase could lead to the appearance of LP-X in the serum. Catabolism of the resultant LP-X cannot take place owing to an inadequate activity of lipoprotein lipase. Functional immaturity can be presumed in the case of both enzyme systems during the neonatal period. On attainment of a degree of maturity compatible with the appropriate neonatal stage, the LP-X values become negative between the 7th and the 16th week of life. It is conceivable that the appearance of LP-X in the newborn infant can be ascribed to LP-X1, since the "physiological" LP-X concentrations in the neonatal period (values of up to 20 mg/100 ml) are distinctly lower than the values found in obstructive jaundice. LP-X determination can be rated as a useful supplementary investigation in the differential diagnosis of extrahepatic biliary atresia during the first weeks or months of life...
异常脂蛋白(LP-X)是成人梗阻性黄疸存在的一个特定参数。由于在足月和早产新生儿以及婴儿早期的血清中也检测到了LP-X,而此时并无梗阻性黄疸的临床证据,因此在新生儿期对婴儿进行了广泛研究以阐明这一现象。本研究报告了从370多名婴儿(成熟新生儿、早产新生儿和幼儿)的2000多份血清中获得的数据,这些血清或多或少连续地采用Rapidophor法进行检测,最初是定性检测,随后是半定量检测。LP-X在新生儿出生后的头两周内出现,与喂养方式无关。LP-X浓度与出生体重相关。显示出不成熟迹象的早产儿的LP-X水平明显高于成熟新生儿。LP-X与碱性磷酸酶水平无关,也与γ-谷氨酰转移酶活性无关;胆红素水平同样与LP-X浓度没有关联。经证实患有梗阻性黄疸的患者显示出明显更高的LP-X浓度(大于56mg/100ml),在某些情况下,LP-X水平的升高先于梗阻性黄疸临床表现的出现。为了解释新生儿期LP-X的存在,提出了以下假设,并根据成人的临床观察、新生儿的生理状况以及本研究的结果进行了讨论:在生命的这个早期阶段,肝脏在代谢器官中占据中心位置,在脂蛋白方面也是如此,其处于器官和功能成熟的生理阶段。在这种情况下,基于胆汁脂蛋白排泄不足的假梗阻机制,同时伴有卵磷脂:胆固醇酰基转移酶的“生理性”缺乏,可能导致血清中出现LP-X。由于脂蛋白脂肪酶活性不足,所产生的LP-X无法进行分解代谢。在新生儿期,这两种酶系统可能都存在功能不成熟的情况。在达到与相应新生儿阶段相适应的成熟度时,LP-X值在出生后第7至16周变为阴性。可以想象,新生儿中LP-X的出现可归因于LP-X1,因为新生儿期的“生理性”LP-X浓度(高达20mg/100ml)明显低于梗阻性黄疸中发现的值。在生命的头几周或几个月,LP-X测定可被视为肝外胆道闭锁鉴别诊断中的一项有用的辅助检查……