Bloomer J R, Pierach C A
Hepatology. 1982 Nov-Dec;2(6):817-21. doi: 10.1002/hep.1840020613.
Hepatic damage in protoporphyria appears to be caused by a toxic effect of excess protoporphyrin. Therapy which reduces the formation of excess protoporphyrin may, therefore, be helpful. We examined the effects of hematin administered i.v. to two patients with protoporphyria and decompensated cirrhosis. Neither patient had side effects from the compound or manifested signs of toxicity. The vascular disappearance of hematin in one patient was similar to that in patients with porphyria who do not have structural liver disease. In both patients, biochemical changes occurred that were compatible with a reduced rate of protoporphyrin formation. Thus, hematin administration may be useful in treating patients with protoporphyria who develop liver disease.
原卟啉症中的肝损伤似乎是由过量原卟啉的毒性作用引起的。因此，减少过量原卟啉形成的治疗方法可能会有所帮助。我们对两名患有原卟啉症和失代偿性肝硬化的患者静脉注射血晶素，观察其效果。两名患者均未出现该化合物的副作用或毒性迹象。其中一名患者血晶素的血管内消失情况与无肝脏结构疾病的卟啉症患者相似。两名患者均出现了与原卟啉形成速率降低相符的生化变化。因此，对于患有肝病的原卟啉症患者，注射血晶素可能有效。
