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急性卟啉病的急诊表现。

ED presentation of acute porphyria.

作者信息

Liu Yueh-Ping, Lien Wan-Ching, Fang Cheng-Chung, Lai Ting-I, Chen Wen-Jone, Wang Hsiu-Po

机构信息

Department of Emergency Medicine, National Taiwan University Hospital, Taipei, Taiwan.

出版信息

Am J Emerg Med. 2005 Mar;23(2):164-7. doi: 10.1016/j.ajem.2004.03.013.

Abstract

Abdominal pain is a common complaint for visits to ED. Among the causes of abdominal pain, the acute porphyria may confuse emergency physicians. With wide range of unspecific symptoms and signs, acute porphyria is rarely considered as a differential diagnosis of acute abdomen in ED. Some patients even receive unnecessary surgery. There are 32 patients who visited the ED of National Taiwan University Hospital because of acute porphyric attacks over the past 13 years. Ten patients (3 males and 7 females) were diagnosed with acute porphyria for the first time at ED. The onset of age ranged from 17 to 55 years (mean, 32 years). All of our patients presented with abdominal pain but without fever, dermatologic, and neurologic symptoms that are typically presented in acute porphyria. On the average, most of them repeatedly sought for medical help because of persistent symptoms for 4 times before being definitely diagnosed and thus receiving the optimal treatment. Meanwhile, all patients needed at least 2 kinds of analgesic, and most of them needed narcotic analgesia for pain control before diagnosis. The most commonest point of tenderness is over epigastrium (7 of 10 patients). The laboratory and image studies of our patients were of no diagnostic value for acute porphyria, except for Watson-Schwartz test. In summary, our study revealed that when a patient after puberty with repetitive visits because of severe abdominal pain without reasonable causes and needs narcotics for pain control, acute porphyria should be taken into consideration.

摘要

腹痛是急诊就诊的常见主诉。在腹痛的病因中,急性卟啉病可能会让急诊医生感到困惑。由于症状和体征缺乏特异性,急性卟啉病在急诊中很少被考虑作为急腹症的鉴别诊断。有些患者甚至接受了不必要的手术。在过去13年中,有32例患者因急性卟啉病发作到台湾大学医院急诊科就诊。10例患者(3例男性,7例女性)在急诊科首次被诊断为急性卟啉病。发病年龄在17至55岁之间(平均32岁)。我们所有的患者均表现为腹痛,但无急性卟啉病典型的发热、皮肤和神经症状。平均而言,他们中的大多数人因症状持续而反复寻求医疗帮助4次后才被明确诊断并接受最佳治疗。同时,所有患者至少需要2种镇痛药,大多数患者在诊断前需要使用麻醉性镇痛药来控制疼痛。最常见的压痛部位在上腹部(10例患者中有7例)。除了沃-施二氏试验外,我们患者的实验室检查和影像学检查对急性卟啉病均无诊断价值。总之,我们的研究表明,对于青春期后因严重腹痛反复就诊且无合理原因且需要使用麻醉药控制疼痛的患者,应考虑急性卟啉病。

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