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多佐胺治疗淀粉样变轻链淀粉样变性致难治性神经源性直立性低血压和反射性心动过缓:1 例报告。

Droxidopa as an effective treatment for refractory neurogenic orthostatic hypotension and reflex bradycardia in amyloid light-chain amyloidosis: a case report.

机构信息

Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA.

出版信息

J Med Case Rep. 2020 Jun 20;14(1):73. doi: 10.1186/s13256-020-02405-w.

DOI:10.1186/s13256-020-02405-w
PMID:32560740
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7305628/
Abstract

BACKGROUND

Droxidopa is an oral treatment for the stepwise treatment of neurogenic orthostatic hypotension from autonomic dysfunction. It has been shown to be useful predominantly with neurogenic orthostatic hypotension secondary to Parkinson's disease, but only a few cases have documented its usefulness in patients with neurogenic orthostatic hypotension due to amyloidosis, which is often severe and refractory. In addition, only one source in the literature reports the concomitant use of midodrine and droxidopa for such patients. Finally, we argue that droxidopa seems to have a protective effect against episodes of reflex bradycardia, which is not previously reported.

CASE PRESENTATION

A 64-year-old white man was admitted for 1 year of worsening syncopal episodes, diarrhea, failure to thrive, heart failure, and neuropathy. Medical emergencies were called five times on the overhead hospital intercom over a 4-day period in the beginning of his admission due to severe hypotension and bradycardia. He was eventually diagnosed as having amyloid light-chain amyloidosis and myeloma. After starting droxidopa, both his systolic blood pressure and reflex bradycardia improved, and no more medical emergency events were called during the remaining 30 days of admission. He felt much better subjectively and was able to sit upright and engage in physical therapy.

CONCLUSIONS

We show that droxidopa is effective when used with midodrine to treat refractory neurogenic orthostatic hypotension in patients with amyloidosis. There are very few cases reporting the use of droxidopa in amyloidosis, with only one study that uses droxidopa and midodrine concomitantly. In addition, our patient's reflex bradycardia improved drastically after starting droxidopa, which we believe is mediated by increased systemic norepinephrine. There were no side effects to droxidopa, and the benefits lasted well beyond the reported duration of 1-2 weeks that was noted to be a limitation in some studies.

摘要

背景

Droxidopa 是一种口服药物,可用于治疗自主功能障碍引起的神经源性直立性低血压的逐步治疗。它已被证明对帕金森病引起的神经源性直立性低血压有用,但只有少数病例记录了其在淀粉样变性引起的神经源性直立性低血压患者中的有用性,这种情况通常很严重且难以治疗。此外,文献中只有一个来源报告了同时使用米多君和 droxidopa 治疗此类患者。最后,我们认为 droxidopa 似乎对反射性心动过缓发作有保护作用,这一点以前没有报道过。

病例介绍

一名 64 岁白人男性因 1 年的恶化性晕厥发作、腹泻、体重减轻、心力衰竭和神经病而入院。他入院的前 4 天内,由于严重的低血压和心动过缓,医院的头顶对讲机呼叫了 5 次医疗急救。他最终被诊断为淀粉样轻链淀粉样变性和骨髓瘤。开始使用 droxidopa 后,他的收缩压和反射性心动过缓都得到了改善,在入院的剩余 30 天内,没有再呼叫医疗急救事件。他主观上感觉好多了,能够坐直并进行物理治疗。

结论

我们表明,当与米多君联合使用时,droxidopa 可有效治疗淀粉样变性患者的难治性神经源性直立性低血压。报告使用 droxidopa 治疗淀粉样变性的病例非常少,只有一项研究同时使用 droxidopa 和米多君。此外,我们的患者在开始使用 droxidopa 后,反射性心动过缓明显改善,我们认为这是通过增加系统性去甲肾上腺素介导的。droxidopa 没有副作用,其益处持续时间远远超过一些研究中指出的 1-2 周的报告持续时间。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/f65434c5ad3f/13256_2020_2405_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/4069bbead29e/13256_2020_2405_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/d6c7cd320660/13256_2020_2405_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/6ea1298bbb6d/13256_2020_2405_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/5b02a0f6f02a/13256_2020_2405_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/f65434c5ad3f/13256_2020_2405_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/4069bbead29e/13256_2020_2405_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/d6c7cd320660/13256_2020_2405_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/6ea1298bbb6d/13256_2020_2405_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/5b02a0f6f02a/13256_2020_2405_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab8c/7305628/f65434c5ad3f/13256_2020_2405_Fig5_HTML.jpg

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