罕见的肺甲状腺转蛋白淀粉样变

IF 0.7 Q4 RESPIRATORY SYSTEM
Marc Assaad, Roshan Acharya, Elspeth Springsted, Frank Biscardi
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引用次数: 0

摘要

Virchow在1853年描述了淀粉样变性。淀粉样变性是一种不溶性纤维蛋白错误折叠聚合四聚体的细胞外沉积,在组织内积累导致器官功能障碍。淀粉样变性每年影响100万人中的10人。淀粉样变性的特定种类是由不同的蛋白质引起的。组织受累可导致多器官系统功能障碍。淀粉样变累及肺部是非常罕见的,组织活检是诊断的关键。可根据怀疑的类型要求进行基因检测。肺受累是非常罕见的,主要是无症状的,一些病例只有在死后才被诊断出来。当有症状时,临床表现是非特异性的,包括咳嗽、呼吸困难和呼吸道感染。浸润过程可能影响肺力学,导致肺功能检测异常。肺淀粉样变的影像学表现包括胸膜、气管支气管树、肺泡和纵隔淋巴结。肺淀粉样变的治疗是基于治疗潜在的疾病过程。我们在此报告一例78岁男性老年性心脏淀粉样变性患者,他在我们的诊所表现为多发结节性肺混浊。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A rare presentation of pulmonary transthyretin amyloidosis
Virchow described amyloidosis in 1853. Amyloidosis is the extracellular deposition of an insoluble misfolded polymerized tetramer of fibrillary protein that accumulates within tissues leading to organ dysfunction. Amyloidosis affects 10 people per one million every year. Specific varieties of amyloidosis result from diversely involved proteins. Tissue involvement can lead to multiorgan system dysfunction. Lung involvement from amyloidosis is very rare, and tissue biopsy is crucial for diagnosis. Genetic testing might be requested based on the suspected type. Lung involvement is very rare and mainly asymptomatic, and some cases are only diagnosed postmortem. When symptomatic, the clinical manifestations are nonspecific and include cough, dyspnea, and respiratory infections. The infiltrative process might affect pulmonary mechanics producing abnormal pulmonary function testing. Radiological manifestations of pulmonary amyloidosis involve the pleura, the tracheobronchial tree, the alveoli, and the mediastinal lymph nodes. Treatment of pulmonary amyloidosis is based on treating the underlying disease process. We present herein a case of 78-year-old male with senile cardiac amyloidosis, who presents to our clinic with multiple nodular pulmonary opacities.
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来源期刊
Respiratory Medicine Case Reports
Respiratory Medicine Case Reports RESPIRATORY SYSTEM-
CiteScore
2.10
自引率
0.00%
发文量
213
审稿时长
87 days
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