患有乳糜泻的患者中的急性间歇性卟啉病（AIP）。
Acute intermittent porphyria (AIP) in a patient with celiac disease.
作者信息
Nunnemann Sebastian, Uibel Christoph, Budig Petra, Mäurer Mathias
机构信息
Department of Neurology, Klinikum Würzburg Mitte gGmbH, Standort Juliusspital, 97070 Würzburg, Germany.
出版信息
Neurol Res Pract. 2020 Jan 20;2:2. doi: 10.1186/s42466-020-0049-6. eCollection 2020.
We present the case of an 18 year old Caucasian with known celiac disease, who suffered a severe first attack of acute intermittent porphyria (AIP) with neuropsychiatric symptoms, severe tetraparesis and respiratory insufficiency. Treatment with heme arginate and high-dose intravenous glucose and rigorous rehabilitation resulted in a slow but almost complete recovery of her motor symptoms. To our knowledge this is the first case of acute intermittent porphyria triggered by malnutrition in the context of celiac disease. It is remarkable that the patient showed a favourable outcome despite the severity of her initial symptoms. This case shows the importance of early and systematic symptomatic treatment in patients with severe neurologic manifestation of AIP.
我们报告一例18岁患有乳糜泻的白种人病例，该患者首次发作严重的急性间歇性卟啉病（AIP），伴有神经精神症状、严重的四肢轻瘫和呼吸功能不全。使用精氨酸血红素、高剂量静脉注射葡萄糖治疗以及严格的康复治疗，使其运动症状缓慢但几乎完全恢复。据我们所知，这是第一例在乳糜泻背景下由营养不良引发的急性间歇性卟啉病。值得注意的是，尽管患者初始症状严重，但仍取得了良好的预后。该病例显示了对有严重AIP神经表现的患者进行早期和系统对症治疗的重要性。
Zotero 插件