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急性间歇性卟啉病。不止是腹痛。

Acute intermittent porphyria. More than just abdominal pain.

作者信息

Holman J R, Green J B

机构信息

Department of Family Practice, Naval Hospital, Camp Pendleton, CA 92055-5008.

出版信息

Postgrad Med. 1989 Oct;86(5):295-8. doi: 10.1080/00325481.1989.11704454.

Abstract

Acute intermittent porphyria is an unusual syndrome that may be very difficult to differentiate from acute intraabdominal processes. Diagnosis requires the documentation of increased urinary porphobilinogen. Failure to perform appropriate tests early in the attack may necessitate diagnostic laparotomy to rule out intraabdominal catastrophies. Once the diagnosis has been made, treatment with intravenous dextrose aborts most acute episodes. Patients should avoid such triggering factors as fasting and certain drugs. Screening of family members is important to identify latent carriers.

摘要

急性间歇性卟啉病是一种不常见的综合征,可能很难与急性腹部疾病相鉴别。诊断需要记录尿卟胆原增加。在发作早期未进行适当检查可能需要进行诊断性剖腹手术以排除腹部灾难情况。一旦确诊,静脉输注葡萄糖治疗可中止大多数急性发作。患者应避免禁食和某些药物等诱发因素。对家庭成员进行筛查对于识别潜在携带者很重要。

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