Nordmann Y, Deybach J C
Centre Français des Porphyries, Hôpital Louis-Mourier, Colombes.
Ann Med Interne (Paris). 1993;144(3):165-7.
Severe hepatic porphyrias (acute intermittent porphyria, variegate porphyria and hereditary coproporphyria) are hereditary diseases. Each type of porphyria is the result of a specific decrease in the activity of one of the enzymes of heme biosynthesis. Acute attacks are very serious: the abdominal pains are severe and the neurological manifestations can lead to death or incomplete recovery with irreversible sequelae (usually paralysis). Since 1985, the prognosis of acute attacks has been greatly improved by the introduction of heme-arginate. The 69 acute attacks (30 patients, 4 men and 26 women) that we treated with heme-arginate between 1988 and 1991 are described in this report. All patients were infused with 250 mg/d of heme-arginate for 4 days: the mean duration of abdominal pain was 2.5 days (SD 0.72). For 95 p. 100 of the attacks, the total hospitalization time was 5 days or less; side effects were very minor. In every case, a favorable response was dependent upon the early initiation of heme therapy.
严重肝性卟啉病（急性间歇性卟啉病、混合型卟啉病和遗传性粪卟啉病）是遗传性疾病。每种卟啉病都是血红素生物合成过程中一种特定酶的活性降低所致。急性发作非常严重：腹痛剧烈，神经学表现可导致死亡或不完全恢复并伴有不可逆后遗症（通常为瘫痪）。自1985年以来，通过使用精氨酸血红素，急性发作的预后有了很大改善。本报告描述了我们在1988年至1991年间用精氨酸血红素治疗的69次急性发作（30例患者，4名男性和26名女性）。所有患者均以250mg/d的剂量输注精氨酸血红素，持续4天：腹痛的平均持续时间为2.5天（标准差0.72）。95%的发作，总住院时间为5天或更短；副作用非常轻微。在每种情况下，良好的反应都取决于血红素治疗的早期开始。
