Horie Y, Okano J, Kitaoka S, Tajima F, Kawasaki H
2nd Department of Internal Medicine, Tottori University School of Medicine.
Nihon Rinsho. 1995 Jun;53(6):1456-62.
There are seven porphyrias which are caused by defective functions of the enzymes in the haem biosynthesis. The clinical classification of porphyrias are divided into three types which are neuroporphyria, neurocutaneous porphyria and cutaneous porphyria. For acute porphyric attack of neuroporphyria and neurocutaneous porphyria, the treatments of choice are administration of glucose and/or hematin, haem arginate and tinprotoporphyrin. Porphyria cutanea tarda in cutaneous porphyria is controlled by removal of iron by phrebotomies or low-dose chloriquine. A novel approach of intravenous administration of interferon may be useful to control the associated case of chronic hepatitis type C. Skin symptoms in erythropoietic protoporphyria can be alleviated with beta carotene. Hepatic failure due to protoporphyria may need a liver transplantation. Cimetidine, a H2-receptor antagonist, may be useful in the treatment of acute porphyric attack and in remission stage in neuroporphyrias, neurocutaneous porphyrias and cutaneous porphyrias such as porphyria cutanea tarda and protoporphyria.
有七种卟啉病是由血红素生物合成中酶的功能缺陷引起的。卟啉病的临床分类分为三种类型，即神经卟啉病、神经皮肤卟啉病和皮肤卟啉病。对于神经卟啉病和神经皮肤卟啉病的急性卟啉发作，首选的治疗方法是给予葡萄糖和/或血红素、精氨酸血红素和锡原卟啉。皮肤卟啉病中的迟发性皮肤卟啉病通过放血或低剂量氯喹去除铁来控制。静脉注射干扰素的一种新方法可能有助于控制丙型慢性肝炎的相关病例。红细胞生成性原卟啉病的皮肤症状可用β-胡萝卜素缓解。原卟啉病导致的肝衰竭可能需要进行肝移植。H2受体拮抗剂西咪替丁可能有助于治疗急性卟啉发作以及在神经卟啉病、神经皮肤卟啉病和皮肤卟啉病（如迟发性皮肤卟啉病和原卟啉病）的缓解期。
