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非典型原发性肺淀粉样变性:一例罕见病例报告。

Atypical primary pulmonary amyloidosis: A rare case report.

作者信息

Peng Xiong, Wang Xiaolei, Luo Daya, Zuo Wei, Yao Huiming, Zhang Wei

机构信息

Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Nanchang University.

Second Department of Cardiovascular Medicine, Jiangxi Provincial People's Hospital Affiliated of Nanchang University.

出版信息

Medicine (Baltimore). 2020 Jun 26;99(26):e20828. doi: 10.1097/MD.0000000000020828.

DOI:10.1097/MD.0000000000020828
PMID:32590770
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7328997/
Abstract

RATIONALE

Pulmonary amyloidosis is a rare respiratory disease characterized by amyloid deposition in the lungs. The clinical manifestations of pulmonary amyloidosis are variable and without specific symptoms.

PATIENT CONCERNS

We report a rare case of tracheobronchial amyloidosis to improve our understanding of the disease.

DIAGNOSES

The diagnosis of tracheobronchial amyloidosis was finally established by transbronchoscopic lung biopsy and histological examination.

INTERVENTIONS

The patient significantly improved with methylprednisolone sodium succinate for injection (40 mg/day) for 5 days and low-dose oral prednisone for 10 days.

OUTCOMES

After treatment, discomfort, such as cough, stridor, dyspnea, and chest tightness, disappeared, and he was discharged. The patient was in good clinical condition after 8 months of follow-up.

CONCLUSION

This case clearly shows that it is difficult to distinguish tracheobronchial amyloidosis from other diseases with manifestations of cough, dyspnea and chest tightness because of their similar symptoms and imaging findings. Thus, the role of transbronchoscopic lung biopsy and histological examination in the diagnosis of tracheobronchial amyloidosis is very important.

摘要

原理

肺淀粉样变性是一种罕见的呼吸系统疾病,其特征是淀粉样物质在肺内沉积。肺淀粉样变性的临床表现多样,且无特异性症状。

患者情况

我们报告一例罕见的气管支气管淀粉样变性病例,以增进我们对该疾病的了解。

诊断

最终通过经支气管肺活检和组织学检查确诊为气管支气管淀粉样变性。

干预措施

患者接受注射用甲泼尼龙琥珀酸钠(40毫克/天)治疗5天及小剂量口服泼尼松治疗10天后病情明显改善。

结果

治疗后,咳嗽、喘鸣、呼吸困难和胸闷等不适症状消失,患者出院。随访8个月后患者临床状况良好。

结论

该病例清楚表明,由于气管支气管淀粉样变性与其他有咳嗽、呼吸困难和胸闷表现的疾病症状及影像学表现相似,难以将二者区分开来。因此,经支气管肺活检和组织学检查在气管支气管淀粉样变性诊断中的作用非常重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/2539c8b7da2f/medi-99-e20828-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/6342b829abac/medi-99-e20828-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/906a7ef182b9/medi-99-e20828-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/2539c8b7da2f/medi-99-e20828-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/6342b829abac/medi-99-e20828-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/906a7ef182b9/medi-99-e20828-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e8c/7328997/2539c8b7da2f/medi-99-e20828-g003.jpg

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本文引用的文献

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Lung India. 2019 May-Jun;36(3):229-232. doi: 10.4103/lungindia.lungindia_205_18.
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[Clinical features of primary tracheobronchial pulmonary amyloidosis].[原发性气管支气管肺淀粉样变性的临床特征]
Zhonghua Yi Xue Za Zhi. 2019 Mar 26;99(12):918-922. doi: 10.3760/cma.j.issn.0376-2491.2019.12.008.
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Amyloidosis of the Lung.肺淀粉样变性
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Colchicine Treatment for Tracheobronchial Amyloidosis.秋水仙碱治疗气管支气管淀粉样变性
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