卟啉症的急性神经学表现及其类型:系统综述。

Q2 Medicine
Enrico A Barletta, Telmo A B Belsuzarri, Augusto R B Urena, Eduardo A Iunes
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引用次数: 0

摘要

简介:急性卟啉症引起危及生命的神经内脏非特异性症状发作,因此这种情况类似于许多急性医学和精神疾病。该病很容易误诊,可能是由于其发病率低和非典型症状,这延迟了有效治疗的开始。早期诊断和治疗可大大改善预后,并可预防神经病变表现的发展。方法:我们按照PRISMA指南并使用Pubmed作为我们的数据库,进行了系统的综述。我们的目的是显示在急性卟啉症发作中出现神经系统表现的患者的一些特点。我们获得了16例患者的年龄、性别、临床表现、神经系统表现和卟啉症类型。我们还评估了症状出现和神经系统表现之间的时间。平均年龄28.4±11.1岁;50%的患者为男性。结果:AIP是最常见的卟啉类型。从症状出现到神经系统表现的平均时间为9.53±11.6 d。腹痛;恶心、呕吐和精神症状是神经性发作前最常见的症状。癫痫发作和意识障碍是最常见的症状。我们还提出了一个案例来说明这种诊断是多么困难。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acute Neurological Manifestations of Porphyrias and its Types: A Systematic- Review.

Introduction: Acute porphyrias cause life-threatening attacks of neurovisceral non-specific symptoms, so this condition mimics many acute medical and psychiatric diseases. The disease is very misdiagnosed, probably due to its low incidence and non-pathognomonic symptoms, this delays the effective treatment onset. Early diagnosis and treatment highly improve the prognosis and can prevent the development of neuropathic manifestations.

Methods: We assembled a systematic review, following the PRISMA guidelines and using Pubmed as our database. Our aim was to show some peculiarities among patients that present neurological manifestations in acute porphyria attack. We obtained the patients' age, sex, clinical presentation, eurological manifestations and porphyria type of 16 patients. We also evaluated the time between symptoms onset and neurological manifestations. The average age was 28,4 ± 11,1; 50% of patients were male.

Results: AIP was the most prevalent porphyria type. The average time between symptoms onset and neurological manifestations was of 9,53 ± 11,6 days. Abdominal pain; nausea and vomiting and psychiatric manifestations were the most common symptoms preceding neurological attacks. Seizures and consciousness disturbance were the most prevalent findings within an attack. We also presenting a case to illustrate how difficult this diagnosis can be.

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来源期刊
Cardiovascular and Hematological Agents in Medicinal Chemistry
Cardiovascular and Hematological Agents in Medicinal Chemistry Medicine-Cardiology and Cardiovascular Medicine
CiteScore
2.70
自引率
0.00%
发文量
34
期刊介绍: Cardiovascular & Hematological Agents in Medicinal Chemistry aims to cover all the latest and outstanding developments in medicinal chemistry and rational drug design for the discovery of new Cardiovascular & Hematological Agents. Each issue contains a series of timely in-depth reviews written by leaders in the field covering a range of current topics in Cardiovascular & Hematological medicinal chemistry. Cardiovascular & Hematological Agents in Medicinal Chemistry is an essential journal for every medicinal chemist who wishes to be kept informed and up-to-date with the latest and most important developments in cardiovascular & hematological drug discovery.
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