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Primary endobronchial amyloidosis: A rare case of endobronchial tumor

机译:原发性支气管内淀粉样变性:罕见的支气管内肿瘤病例

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Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8–37 months. The average life expectancy for primary tracheobronchial amyloidosis is approximately 9 years, so the early diagnosis of this disease is very critical to improve the prognosis of patients. We are presenting a case of 65 year old male which was initially diagnosed and treated as asthma exacerbation with minimal improvement. Further workup was done with CT chest, bronchoscopy and biopsy because of persistent shortness of breath, which revealed primary localized solitary endobronchial amyloidosis.
机译:原发性局限性支气管内淀粉样变性很罕见,因为肺淀粉样变性最常见于全身性AL淀粉样变性的一部分。它可以是无症状的,也可以表现为非特异性症状,例如进行性呼吸困难,咳嗽,喘息和很少出现呼吸衰竭。它经常被误诊为哮喘,COPD或肺炎。具有结节状外观的孤立性支气管内淀粉样变性可以模仿支气管内肿瘤。诊断通常会延迟8–37个月。原发性气管支气管淀粉样变性病的平均预期寿命约为9年,因此对该病的早期诊断对于改善患者的预后至关重要。我们介绍的是一例65岁的男性,该患者最初被诊断为哮喘加重,但改善程度很小。由于持续的呼吸短促,需要用CT胸部,支气管镜和活检进一步检查,这表明原发性局部性孤立性支气管内淀粉样变性。

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