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急性间歇型血卟啉病(Acut intermittent por-phyria,AIP),为一种先天性卟啉代谢障碍性疾病,是血卟啉病中较常见的一种。未病时,体内生成大量卟啉前体—卟胆元,后者可沉着于肝、肾、脊髓、交感神经节而引起各种临床表现。本病极易误诊,我院十年来收治 AIP 病人15例,初诊时全部误诊,后经检查尿卟胆元阳性,方确诊本病。15例中男性6例,女性9例。发病年龄18～60岁,其中30岁和30岁以下10例,占66.6%。其临床主要表现见附表。
Acute intermittent porphyria (Acti intermittent por-phyria, AIP), is a congenital porphyria metabolic disorders, is a more common type of blood porphyria. Not disease, the body generated a large number of porphyrin precursor - porphyrin, which can be deposited in the liver, kidney, spinal cord, sympathetic ganglia and cause a variety of clinical manifestations. The disease is extremely easy to misdiagnosis, 15 cases of AIP patients admitted to our hospital over the past 10 years, all misdiagnosed when the first visit, after examination of urinary porphyrin-positive, side of the diagnosis of the disease. There were 6 males and 9 females in 15 cases. Age of onset of 18 to 60 years, of which 30 years old and 30 years of age in 10 cases, accounting for 66.6%. The main clinical manifestations see schedule.