Felsher B F, Carpio N M
Gastroenterology. 1975 Jul;69(1):42-7.
Reduction in caloric intake was associated with a greater absolute rise in the serum bilirubin concentration in patients with Gilbert's syndrome and partial hepatic bilirubin uridine diphosphate glucuronyltransferase (UDPG-T) dysfunction compared to patients with hemolytic unconjugated hyperbilirubinemia and normal subjects. Two patients with overt hemolysis but an exaggerated response to caloric deprivation had reduced UDPG-T activities comparable to Gilbert's syndrome. The UDPG-T activities in the other patients with hemolytic jaundice were normal. The combination of fasting and novobiocin in 2 normal subjects produced a greater increase in bilirubin level than either fasting or novobiocin alone. These data suggest that theunderlying UDPG-T dysfunction, rather than the prefasting level of unconjugated hyperbilirubinemia, is responsible for the diet-induced hyperbilirubinemia in Gilbert's syndrome. The diet test appears to differentiate Gilbert's syndrome from hemolytic jaundice as well as from normal subjects, irrespective of the initial serum bilirubin concentration.
与溶血性非结合性高胆红素血症患者和正常受试者相比,热量摄入减少与吉尔伯特综合征患者及部分肝胆红素尿苷二磷酸葡萄糖醛酸基转移酶(UDPG-T)功能障碍患者血清胆红素浓度的绝对升高幅度更大有关。两名明显溶血但对热量剥夺反应过度的患者,其UDPG-T活性降低,与吉尔伯特综合征相当。其他溶血性黄疸患者的UDPG-T活性正常。在两名正常受试者中,禁食和新生霉素联合使用比单独禁食或单独使用新生霉素导致胆红素水平升高幅度更大。这些数据表明,吉尔伯特综合征中饮食诱导的高胆红素血症的原因是潜在的UDPG-T功能障碍,而非禁食前非结合性高胆红素血症的水平。饮食试验似乎可以将吉尔伯特综合征与溶血性黄疸以及正常受试者区分开来,而与初始血清胆红素浓度无关。