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一名患有潜在淋巴管平滑肌瘤病的患者因新冠病毒病导致急性低氧血症性呼吸衰竭

Acute Hypoxemic Respiratory Failure Due to COVID-19 in a Patient With Underlying Lymphangioleiomyomatosis.

作者信息

Glowacki Joseph, Holland Gregory, Graham Colleen, Bakhsh Khazenay

机构信息

Internal Medicine, Rowan University School of Osteopathic Medicine, Stratford, USA.

Pulmonology, Jefferson Health New Jersey, Turnersville, USA.

出版信息

Cureus. 2022 Jun 12;14(6):e25871. doi: 10.7759/cureus.25871. eCollection 2022 Jun.

DOI:10.7759/cureus.25871
PMID:35836441
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9275532/
Abstract

Lymphangioleiomyomatosis (LAM) is a rare disorder that can cause lesions that develop into cysts, most commonly in the lung parenchyma and renal angiomyolipomas. We report a case of a young female with LAM who was admitted to the hospital for a COVID-19 infection, with the objective of discussing the management of LAM with concurrent COVID-19 infection. She ultimately showed overall clinical improvement after receiving dexamethasone and remdesivir, while holding her outpatient mammalian target of rapamycin (mTOR) inhibitor. When patients with rare diseases acquire COVID-19, an individualized approach to treatment is often most effective as information and studies may be limited.

摘要

淋巴管平滑肌瘤病(LAM)是一种罕见疾病,可导致形成囊肿的病变,最常见于肺实质和肾血管平滑肌脂肪瘤。我们报告一例患有LAM的年轻女性因COVID-19感染入院的病例,目的是讨论LAM合并COVID-19感染的治疗方法。她在接受地塞米松和瑞德西韦治疗后,同时停用门诊使用的哺乳动物雷帕霉素靶蛋白(mTOR)抑制剂,最终总体临床症状得到改善。当罕见病患者感染COVID-19时,由于相关信息和研究可能有限,个体化治疗方法往往最为有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/924d/9275532/97c6abb596b3/cureus-0014-00000025871-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/924d/9275532/97c6abb596b3/cureus-0014-00000025871-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/924d/9275532/97c6abb596b3/cureus-0014-00000025871-i01.jpg

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本文引用的文献

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COVID-19 and lymphangioleiomyomatosis: Experience at a reference center and the potential impact of the use of mTOR inhibitors.新型冠状病毒肺炎与淋巴管平滑肌瘤病:一家参考中心的经验及使用雷帕霉素靶蛋白抑制剂的潜在影响
Am J Med Genet A. 2020 Dec;182(12):3068-3070. doi: 10.1002/ajmg.a.61877. Epub 2020 Sep 18.
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Effect of Dexamethasone on Days Alive and Ventilator-Free in Patients With Moderate or Severe Acute Respiratory Distress Syndrome and COVID-19: The CoDEX Randomized Clinical Trial.地塞米松对中重度 COVID-19 相关急性呼吸窘迫综合征患者存活天数和无呼吸机天数的影响:CoDEX 随机临床试验。
JAMA. 2020 Oct 6;324(13):1307-1316. doi: 10.1001/jama.2020.17021.
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Tuberous sclerosis complex (TSC), lymphangioleiomyomatosis, and COVID-19: The experience of a TSC clinic in Italy.
结节性硬化症复合征(TSC)、淋巴管平滑肌瘤病和 COVID-19:意大利 TSC 诊所的经验。
Am J Med Genet A. 2020 Nov;182(11):2479-2485. doi: 10.1002/ajmg.a.61810. Epub 2020 Aug 17.
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EPIDEMIOLOGY, PATHOGENESIS and DIAGNOSIS of LYMPHANGIOLEIOMYOMATOSIS.淋巴管平滑肌瘤病的流行病学、发病机制与诊断
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Minireview: Lymphangioleiomyomatosis (LAM): The "Other" Steroid-Sensitive Cancer.综述:淋巴管平滑肌瘤病(LAM):另一种对类固醇敏感的癌症
Endocrinology. 2016 Sep;157(9):3374-83. doi: 10.1210/en.2016-1395. Epub 2016 Jul 13.
6
Efficacy and safety of sirolimus in lymphangioleiomyomatosis.西罗莫司治疗淋巴管平滑肌瘤病的疗效和安全性。
N Engl J Med. 2011 Apr 28;364(17):1595-606. doi: 10.1056/NEJMoa1100391. Epub 2011 Mar 16.
7
Lymphangioleiomyomatosis: a clinical update.淋巴管平滑肌瘤病:临床最新进展
Chest. 2008 Feb;133(2):507-16. doi: 10.1378/chest.07-0898.