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一名既往健康且免疫功能正常的年轻成年人复发性肺炎:病例报告——穆尼耶-库恩综合征

Recurrent Pneumonias in a Previously Healthy and Immunocompetent Young Adult: A Case Report Mounier-Kuhn Syndrome.

作者信息

Estrada David Esteban, Uribe-Buritica Francisco L, Vargas Carlos Andrés, García Carlos, Martínez William

机构信息

Faculty of Health Sciences, Icesi University, Cali, Colombia.

Center of Clinical Investigations (CIC), Lili Valley Foundation, Cali, Colombia.

出版信息

Am J Case Rep. 2020 Feb 9;21:e918535. doi: 10.12659/AJCR.918535.

Abstract

BACKGROUND Mounier-Kuhn syndrome is a rare clinical condition characterized by marked tracheal and bronchial dilatation. It is commonly associated with multiple respiratory infections due to the inability to mobilize secretions, chronic cough, and decline in lung capacity. Although it is a rare disease, the number of cases reported in the literature has increased, and physicians should be aware of its existence to be able to recognize and diagnose it. Suspicion should arise regarding young adults who develop recurrent airway infections when immunosuppression has been ruled out. CASE REPORT We present the case of a young adult, previously healthy and immunocompetent, who was diagnosed with Mounier-Kuhn syndrome after 5 years of multiple respiratory infections and multiple respiratory failures, who received intensive care management with multiple complications and difficulties in achieving adequate ventilation. Due to the risk of respiratory failure and major complications, its management was challenging. We were able to treat this patient and prevent complications with a multidisciplinary approach with a strong emphasis on constant and intense pulmonary therapy. CONCLUSIONS Mounier-Kuhn syndrome is rare but does occur, and physicians need to have a high index of suspicion to diagnose it. Its management must be done by a multidisciplinary team, and respiratory therapy should be constantly available at bedside.

摘要

背景

穆尼尔-库恩综合征是一种罕见的临床病症,其特征为气管和支气管显著扩张。由于无法排出分泌物、慢性咳嗽以及肺功能下降,该病症常伴有多种呼吸道感染。尽管它是一种罕见疾病,但文献报道的病例数量有所增加,医生应了解其存在以便能够识别和诊断。对于排除免疫抑制后出现反复气道感染的年轻人,应引起怀疑。

病例报告

我们报告一例年轻成年人的病例,该患者此前健康且免疫功能正常,在经历5年的多次呼吸道感染和多次呼吸衰竭后被诊断为穆尼尔-库恩综合征,接受重症监护管理时出现多种并发症且难以实现充分通气。由于呼吸衰竭和主要并发症的风险,其管理具有挑战性。我们通过多学科方法成功治疗了该患者并预防了并发症,该方法高度重视持续且强化的肺部治疗。

结论

穆尼尔-库恩综合征罕见但确实存在,医生需要高度怀疑以进行诊断。其管理必须由多学科团队完成,呼吸治疗应随时在床边进行。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ecf8/7032529/aa53b5ea8059/amjcaserep-21-e918535-g001.jpg

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