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纯粹自主神经衰竭:反复直立性低血压病例报告。

Pure Autonomic Failure: A Case Report of Recurrent Orthostatic Hypotension.

机构信息

Department of Internal Medicine, Universal College of Medical Sciences, Bhairahawa, Nepal.

Universal College of Medical Sciences, Bhairahawa, Nepal.

出版信息

JNMA J Nepal Med Assoc. 2021 Jul 1;59(238):589-592. doi: 10.31729/jnma.6480.

DOI:10.31729/jnma.6480
PMID:34508404
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8369549/
Abstract

Pure autonomic failure is a neurodegenerative disorder affecting the autonomic nervous system which clinically presents with orthostatic hypotension. It is a diagnosis of exclusion after detailed clinical examinations and relevant investigations. Here, we discuss a case of 68 years old male who had complaints of multiple episodes of loss of consciousness on standing from a sitting position for the last 3 years. The diagnosis was considered by clinical examinations revealing autonomic dysfunctions with normal appropriate investigations. The patient was treated successfully with midodrine, fludrocortisone, and other non-pharmacological interventions. We focused on doing various autonomic dysfunction tests in the evaluation of a patient with recurrent orthostatic hypotension. We suspect that pure autonomic failure might not have been considered in the differential diagnosis of recurrent orthostatic hypotension and suggest that it is to be kept as a differential in such a scenario. Midodrine has an effective role in syncope due to sympathetic vasoconstrictor failure.

摘要

纯粹自主神经衰竭是一种影响自主神经系统的神经退行性疾病,其临床表现为直立性低血压。在经过详细的临床检查和相关调查后,排除其他可能性才能做出这一诊断。在这里,我们讨论了一个 68 岁男性的病例,他在过去 3 年中多次出现从坐姿站立时失去意识的情况。临床检查显示自主神经功能障碍,同时适当的检查结果正常,因此考虑到这一诊断。患者成功接受了米多君、氟氢可的松和其他非药物干预治疗。我们专注于通过各种自主神经功能障碍测试来评估反复发生直立性低血压的患者。我们怀疑在反复发生直立性低血压的鉴别诊断中可能没有考虑到纯粹自主神经衰竭,并建议在这种情况下将其作为鉴别诊断之一。米多君在因交感血管收缩失败引起的晕厥中具有有效作用。

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Efficacy of midodrine vs placebo in neurogenic orthostatic hypotension. A randomized, double-blind multicenter study. Midodrine Study Group.米多君与安慰剂治疗神经源性直立性低血压的疗效比较:一项随机、双盲、多中心研究。米多君研究组
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