Gentile S, Ayala F, Orlando C, Santoianni P, Budillon G
Istituto di Medicina Generale e Metodologia Clinica, I Facoltà di Medicina, University of Naples, Italy.
Scand J Clin Lab Invest. 1988 Nov;48(7):641-5.
Nicotinic acid (NA) administration in Gilbert's syndrome (GS) patients promotes an increment of bilirubin and of total iron serum levels, dependent on a defective hepatic bilitranslocase function and on a haemolytic effect of NA. In porphyria cutanea tarda (PCT): (1) the effect of nicotinic acid on bilirubinaemia is superimposable to that in controls; (2) a well documented disturbance of iron metabolism occurs; (3) but relationship between bilirubin and iron under NA load has never been investigated. The administration of 5.9 mumol/kg body weight of NA to 12 PCT patients, 10 GS subjects and nine healthy volunteers of comparable age resulted in: (1) normal behaviour of bilirubin parameters in PCT but higher bilirubinaemic values in GS subjects; (2) normal values of serum iron in GS subjects, but higher baseline values and lower sideraemic effect of nicotinic acid in PCT patients; (3) a normal NA half-life in PCT and enhanced in GS subjects. These findings confirm a defective bilirubin uptake and excretion by the liver of GS subjects with a normal iron metabolism. On the contrary, in our PCT patients a normal clearance of bilirubin occurs, but a complex disturbance of iron metabolism is well evident in baseline conditions as well as after NA administration. The latter being probably the consequence of an enhanced excretion of iron extraproduced by the haemolytic effect of NA.
在吉尔伯特综合征(GS)患者中给予烟酸(NA)会促使胆红素和血清总铁水平升高,这取决于肝脏胆红素转运酶功能缺陷以及NA的溶血作用。在迟发性皮肤卟啉症(PCT)中:(1)烟酸对胆红素血症的影响与对照组相当;(2)铁代谢出现有充分记录的紊乱;(3)但从未研究过NA负荷下胆红素与铁之间的关系。对12例PCT患者、10例GS受试者和9名年龄相仿的健康志愿者给予5.9 μmol/kg体重的NA后,结果如下:(1)PCT患者的胆红素参数表现正常,但GS受试者的胆红素血症值更高;(2)GS受试者的血清铁值正常,但PCT患者的基线值更高且烟酸的降铁效应更低;(3)PCT患者的NA半衰期正常,而GS受试者的半衰期延长。这些发现证实了铁代谢正常的GS受试者肝脏对胆红素的摄取和排泄存在缺陷。相反,在我们的PCT患者中,胆红素清除正常,但在基线状态以及给予NA后,铁代谢的复杂紊乱都很明显。后者可能是NA溶血作用导致铁额外生成增加并排泄增强的结果。