Tenhunen R, Mustajoki P
Dept. of Clinical Chemistry, Helsinki University Central Hospital, Finland.
Semin Liver Dis. 1998;18(1):53-5. doi: 10.1055/s-2007-1007140.
The characterization of porphyrias as disorders of heme biosynthesis leading to hepatic heme deficiency and raised formation and secretion of heme precursors laid the basis for heme treatment. Although mild attacks sometimes respond to glucose administration, severe attacks or symptoms that are likely to progress should be treated with heme. The heme compounds used in treatment are hematin and heme arginate. In our opinion, heme arginate is preferable to hematin in the treatment of acute porphyrias because of its better stability, fewer side effects, and better documentation of its benefits.
卟啉症被描述为血红素生物合成紊乱，导致肝脏血红素缺乏以及血红素前体生成和分泌增加，这为血红素治疗奠定了基础。尽管轻度发作有时对给予葡萄糖有反应，但严重发作或可能进展的症状应以血红素治疗。治疗中使用的血红素化合物是氯高铁血红素和精氨酸血红素。我们认为，在急性卟啉症的治疗中，精氨酸血红素优于氯高铁血红素，因为它稳定性更好、副作用更少，且其益处有更充分的文献记载。
