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一例罕见的孤立性胸段AL淀粉样瘤导致右肺完全肺不张。

A rare case of isolated thoracic AL-amyloidoma causing complete atelectasis of the right lung.

作者信息

Rath Smruti, Dcunha Nicholas Joseph, Bhan Neel, Cox Bettye, Adrish Muhammad

机构信息

Department of Medicine, Icahn School of Medicine at Mount Sinai Hospital, New York, NY, USA.

Department of Pathology, Baylor College of Medicine, Houston, TX, USA.

出版信息

Respir Med Case Rep. 2023 Mar 13;43:101837. doi: 10.1016/j.rmcr.2023.101837. eCollection 2023.

Abstract

Tumoral amyloidosis, or amyloidoma, is a benign, but rare form of amyloidosis that has been reported with a favorable prognosis following surgical resection in some case reports. We present a case of acute on chronic respiratory failure secondary to extensive growth of a thoracic amyloidoma causing atelectasis of the right lung. Our case patient had greater morbidity due to late presentation and extensive disease at diagnosis, precluding any surgical intervention. Radiation therapy and medical management were unsuccessful in reducing disease burden. Early diagnosis and detection are pivotal to improving survival in patients with isolated thoracic amyloidoma.

摘要

肿瘤性淀粉样变性,即淀粉样瘤,是一种良性但罕见的淀粉样变性,在一些病例报告中显示手术切除后预后良好。我们报告一例因胸段淀粉样瘤广泛生长导致右肺肺不张继发的急性慢性呼吸衰竭病例。我们的病例患者因诊断时出现较晚且疾病范围广泛,发病率更高,无法进行任何手术干预。放射治疗和药物治疗未能减轻疾病负担。早期诊断和检测对于提高孤立性胸段淀粉样瘤患者的生存率至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0672/10031536/86c6692d89c2/gr1.jpg

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