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急性间歇性卟啉病亦称肝性卟啉病,吡咯卟啉病,易被误诊,应引起临床医生重视。现将我院1958～1982年收治的男8例,女3例急性间歇性卟啉病讨论如下。卟啉病(旧称紫质病),大多是因遗传上的缺陷引起卟啉代谢中有关酶的发生异常或控制血红素合成的调节功能异常,造成卟啉代谢紊乱而发生的疾病。急性间歇性卟啉病是显性常染色体遗传。尿外胆原合成酶部分缺乏是本病生化方面的缺陷。本病发作时腹痛和神经系统症状的发生机理现在还不清楚,是否因ALA(δ-氨基酸乙酰丙酸)及外胆原的毒性作用引起这种症状,尚不能排除。
Acute intermittent porphyria also known as hepatic porphyria, pyrroloporphyrin disease, easily misdiagnosed, should cause clinicians attention. Now in our hospital from 1958 to 1982 admitted to 8 males and 3 females acute intermittent porphyria discussed below. Porphyrinosis (formerly called purpura), mostly due to genetic defects caused by the metabolism of porphyrin enzyme abnormalities in the regulation or control of hemoglobin synthesis dysfunction, resulting in disorders of porphyria metabolism and disease. Acute intermittent porphyria is dominant autosomal inheritance. Partial lack of urinary excretion of bile is a biochemical deficiencies of the disease. The pathogenesis of this disease when abdominal pain and neurological symptoms is still unclear, whether due to ALA (δ-amino acid levulinic acid) and the toxicity caused by the role of external gallbladder can not be ruled out.