[急性间歇性卟啉病引起的复发性腹痛]
[Recurrent abdominal pain caused by acute intermittent porphyria].
作者信息
Rosland J H
机构信息
Anestesiavdelingen Vest-Agder sentralsykehus 4604 Kristiansand.
出版信息
Tidsskr Nor Laegeforen. 2001 Oct 10;121(24):2818-20.
BACKGROUND
Acute intermittent porphyria is an inherited metabolic disorder which may remain undiagnosed throughout life unless appropriate biochemical analysis is being performed during an acute attack.
MATERIALS, METHOD AND RESULTS: We describe a 26-year-old woman who had been suffering from recurrent abdominal pain for many years. She had been hospitalized several times; her appendix had been removed, and three diagnostic laparoscopies and several gynaecological procedures had not revealed the cause. During an intractable and increasing abdominal pain episode, porphyrins were detected in her urine. Her pain was then treated effectively with a combination of morphine, glucose and haem-arginat infusion.
INTERPRETATION
In a patient suffering from repeated episodes of unexplainable abdominal colic, paresis or psychic symptoms, acute intermittent porphyria should be considered as a diagnosis.
背景
急性间歇性卟啉病是一种遗传性代谢紊乱疾病，除非在急性发作期间进行适当的生化分析，否则可能终生无法确诊。
材料、方法和结果：我们描述了一名26岁的女性，她多年来一直遭受反复发作的腹痛。她曾多次住院；阑尾已切除，三次诊断性腹腔镜检查和多次妇科手术均未查明病因。在一次腹痛难以控制且不断加重的发作期间，在她的尿液中检测到了卟啉。随后，通过吗啡、葡萄糖和精氨酸血红素输注联合治疗，有效缓解了她的疼痛。
解读
对于患有反复不明原因腹痛、轻瘫或精神症状发作的患者，应考虑诊断为急性间歇性卟啉病。
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