Walsh J R
Postgrad Med. 1977 Aug;62(2):71-81. doi: 10.1080/00325481.1977.11714578.
Acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria are hepatic porphyrias due to enzyme defects that are inherited as autosomal dominants. Porphyria cutanea tarda is considered an acquired disorder. Similar drugs or circumstances are precipitants of acute attacks in all three inherited hepatic porphyrias. The respective biochemical abnormalities are identifiable by simple, readily available laboratory tests. Management of patients with any of the inherited hepatic porphyrias is directed primarily toward prevention of attacks through avoidance of precipitants and through a diet high in carbohydrate. Therapy for porphyria cutanea tarda includes interdiction of alcohol use and repeated phlebotomy.
急性间歇性卟啉病、杂合性卟啉病和遗传性粪卟啉病是由于酶缺陷导致的肝性卟啉病，这些酶缺陷以常染色体显性方式遗传。迟发性皮肤卟啉病被认为是一种后天性疾病。相似的药物或情况是所有三种遗传性肝性卟啉病急性发作的诱因。各自的生化异常可通过简单、易于获得的实验室检查来识别。对任何一种遗传性肝性卟啉病患者的治疗主要是通过避免诱因和高碳水化合物饮食来预防发作。迟发性皮肤卟啉病的治疗包括戒酒和反复进行静脉放血。
