Kappas A, Drummond G S, Galbraith R A
Rockefeller University Hospital, New York, NY 10021.
Pediatrics. 1993 Mar;91(3):537-9.
The heme oxygenase inhibitor tin (Sn4+)-mesoporphyrin, administered to two 17-year-old Crigler-Najjar type I patients during a 400-day study to lower plasma bilirubin levels, also produced changes, beginning approximately 50 days after initiation of treatment, in hematological and iron metabolism indices consistent with the development of iron deficiency anemia. These indices were responsive to iron supplementation and reverted to normal after termination of inhibitor treatment. Tin-mesoporphyrin enhances biliary heme excretion and inhibits intestinal heme oxygenase when administered orally or parenterally; the changes in blood indices could thus reflect, in part, blockade of heme catabolism and therefore of uptake of heme-derived iron, by intestinal epithelium. This action of the inhibitor suggests that such agents may facilitate studies involving aberrant metabolism of heme-derived iron in humans and that they merit further investigation with respect to their potential value in enhancing iron disposal in certain disorders such as those related, for example, to transfusion-induced iron overload states.
在一项为期400天的研究中,给两名17岁的I型克里格勒 - 纳贾尔病患者使用血红素加氧酶抑制剂锡(Sn4 +)-中卟啉以降低血浆胆红素水平。从治疗开始约50天后,血液学和铁代谢指标也出现了变化,这些变化与缺铁性贫血的发展一致。这些指标对铁补充剂有反应,在抑制剂治疗终止后恢复正常。锡 - 中卟啉口服或肠胃外给药时可增强胆汁血红素排泄并抑制肠道血红素加氧酶;因此,血液指标的变化可能部分反映了血红素分解代谢的阻断,进而反映了肠道上皮对血红素衍生铁的摄取受阻。抑制剂的这一作用表明,此类药物可能有助于涉及人类血红素衍生铁异常代谢的研究,并且就其在某些疾病(例如与输血引起的铁过载状态相关的疾病)中增强铁排泄的潜在价值而言,值得进一步研究。