McCormack L R, Liem H H, Strum W B, Grundy S M, Muller-Eberhard U
Eur J Clin Invest. 1982 Jun;12(3):257-62. doi: 10.1111/j.1365-2362.1982.tb01001.x.
Employing a continuous bile collection, we measured the bile secretion of porphyrins, haem (iron protoporphyrin IX regardless of oxidation state) and bilirubin in five healthy subjects. The baseline values for the flow of porphyrins in the bile were: 4.7 +/- 1.9 nmol/h uroporphyrin, 27.3 +/- 3.8 nmol/h coproporphyrin and 39.2 +/- 11.7 nmol/h protoporphyrin. Bile haem flow was 59.7 +/- 12.6 nmol/h, and that of bilirubin 23.8 +/- 8.2 mumol/h. Following haem injection (6.4 mumol/kg) the flow of protoporphyrin but not of the other porphyrins was reduced, and the bile haem flow increased (232 +/- 109.5 nmol/h), while the flow of bilirubin did not increase significantly. A few patients with representative porphyrias showed the expected increase in copro- and protoporphyrin in the bile. The patient with coproporphyria exhibited a bile flow of coproporphyrin of 1470 +/- 133 nmol/h and of protoporphyrin of 334 +/- 29 nmol/h; haem infusion significantly reduced the bile flow of both porphyrins (to 649 +/- 101 for copro- and 215 +/- 36 nmol/for protoporphyrin). The patient with protoporphyria had an increased protoporphyrin flow, yet haem infusion caused no reduction in protoporphyrin flow (106 +/- 7 after v. 81.4 +/- 13 nmol/h before haem). In conclusion, we found that haem and porphyrins are normal constituents of bile, and that injected haem appears in bile. Bile bilirubin did not rise within 12 h after haem infusion a finding which warrants further investigation.
我们采用连续胆汁收集法,测量了5名健康受试者胆汁中卟啉、血红素(不论氧化态的铁原卟啉IX)和胆红素的分泌情况。胆汁中卟啉流量的基线值为:尿卟啉4.7±1.9 nmol/小时,粪卟啉27.3±3.8 nmol/小时,原卟啉39.2±11.7 nmol/小时。胆汁血红素流量为59.7±12.6 nmol/小时,胆红素流量为23.8±8.2 μmol/小时。注射血红素(6.4 μmol/千克)后,原卟啉流量降低,但其他卟啉流量未降低,胆汁血红素流量增加(232±109.5 nmol/小时),而胆红素流量未显著增加。一些具有代表性卟啉病的患者胆汁中粪卟啉和原卟啉出现预期的增加。患粪卟啉病的患者胆汁中粪卟啉流量为1470±133 nmol/小时,原卟啉流量为334±29 nmol/小时;血红素输注显著降低了两种卟啉的胆汁流量(粪卟啉降至649±101,原卟啉降至215±36 nmol/小时)。患原卟啉病的患者原卟啉流量增加,但血红素输注未导致原卟啉流量降低(血红素注射前为81.4±13 nmol/小时,注射后为106±7 nmol/小时)。总之,我们发现血红素和卟啉是胆汁的正常成分,注射的血红素会出现在胆汁中。血红素输注后12小时内胆汁胆红素未升高,这一发现值得进一步研究。