探索右肺发育不良伴支气管扩张的复杂病例:十年历程。

Journal of medical cases Pub Date : 2024-10-01 Epub Date: 2024-09-20 DOI:10.14740/jmc4283
Muhammad Umer Riaz Gondal, Grant Gillespie, Fawwad Ansari, Swarup Sharma Rijal, Zainab Kiyani, Ayushi Lalwani, Toqeer Khan, Syed Ayan Zulfiqar Bokhari, Ayushma Acharya, Ryan Zimmerman
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引用次数: 0

摘要

单侧肺发育不全(UPH)是一种罕见的先天性疾病,成年后很少发病。大多数患者在年幼时就会因并发症而死亡,存活下来的患者也很少,而且终生容易受到频繁的肺部感染。这种疾病的婴儿死亡率很高。我们现在介绍的病例是一名患有类风湿性关节炎和严重持续性哮喘的 66 岁男性患者,他于 2013 年因气短加重首次到我院急诊科就诊。通过计算机断层扫描(CT)进行胸部成像发现,他的右侧胸腔容积减小,并伴有肺发育不全、蜂窝肺形成和右侧纵隔移位。他因哮喘性支气管炎接受了强的松、吸入剂和抗生素治疗。在接下来的十年中,他因肺炎和哮喘加重而频繁入院(仅本院就收治了 56 例)。他的右肺发育不全被认为是导致反复感染/发炎的原因之一,目前他正在接受右肺切除术的重新评估,因为手术切除是治疗伴有反复呼吸道感染的局部支气管扩张症的一种选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Navigating a Complex Case of Hypoplastic Right Lung With Bronchiectasis: A Ten-Year Journey.

Unilateral pulmonary hypoplasia (UPH) is a rare congenital disorder that presents rarely in adulthood. Most patients succumb to complications at a young age, and those who survive are rare and susceptible to frequent lifelong pulmonary infections. It has a high infant mortality rate. We present the case of a 66-year-old male with rheumatoid arthritis and severe persistent asthma who first presented to our emergency department in 2013 with worsening shortness of breath. Chest imaging with a computed tomography (CT) scan revealed right hemithorax volume loss with hypoplasia, honeycomb lung formation, and right mediastinal shift. He was treated with prednisone, inhalers, and antibiotics for asthmatic bronchitis. He continued to suffer frequent hospital admissions (56 to our hospital alone) over the next decade for pneumonia and asthma exacerbations. The hypoplastic right lung was deemed to be contributing to recurrent infections/inflammation, and he is currently being re-evaluated for a right pneumonectomy, as surgical resection is an option for localized bronchiectasis associated with recurrent respiratory infections.

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