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急性卟啉病为卟啉代谢紊乱病。临床较少见。我国有不少报道。本病分为:1.红细胞生成性卟啉病(先天性骨髓中卟啉代谢紊乱)。2.肝卟啉病(肝内卟啉代谢紊乱)。其中又分为:(1)急性间歇性肝卟啉病;(2)迟发性皮肤型肝卟啉病;(3)混合型肝卟啉病;(4)变异型肝卟啉病。此病有三大症状:1.暴露皮肤可有大疱疹,色素沉着,疤痕,面部多毛,及陪拉格样皮炎。2.突发腹痛,伴恶心呕吐、便秘、白细胞增多,有时误诊为急腹症。3.神经系症状,易与神经衰弱、癔病、上行性瘫痪及多发性神经炎混淆。有神经症状者死亡率高,常有肝肾
Acute porphyria is a disorder of porphyrin metabolism. Clinical less common. There are many reports in our country. The disease is divided into: 1. Erythropoietic porphyria (congenital bone marrow in porphyrin metabolism disorders). 2. Liver porphyria (liver dysfunction of porphyria). Which is divided into: (1) acute intermittent liver porphyria; (2) delayed skin type porphyria; (3) mixed liver porphyria; (4) variant liver porphyria. The disease has three major symptoms: 1. Exposed skin may have herpes, pigmentation, scars, facial hairy, and to accompany langer-like dermatitis. 2. Sudden abdominal pain, with nausea and vomiting, constipation, leukocytosis, sometimes misdiagnosed as acute abdomen. 3. Neurological symptoms, easy and neurasthenia, hysteria, paralysis and multiple neuritis paralysis confusion. Neurological symptoms of high mortality, often liver and kidney