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肺泡蛋白沉积症的临床线索:11例报告及文献复习

The Clinical Clues of Pulmonary Alveolar Proteinosis: A Report of 11 Cases and Literature Review.

作者信息

Mo Qiongya, Wang Bingbin, Dong Nian, Bao Lianmin, Su Xiaoqiong, Li Yuping, Chen Chengshui

机构信息

Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, China.

出版信息

Can Respir J. 2016;2016:4021928. doi: 10.1155/2016/4021928. Epub 2016 Apr 26.

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterized by the abnormal alveolar accumulation of surfactant components. The diagnosis of PAP can be easily missed since it is rare and lacks specific clinical symptoms. It is of great importance to have a better understanding of the crucial clue to clinically diagnose PAP and take PAP into consideration in the differential diagnosis of interstitial pulmonary diseases or other diseases with similar manifestations. Here, we analyze the clinical characteristics of 11 cases of PAP patients in local hospital and review the relevant literature in order to provide more information in diagnosis and management of PAP. In our observation, cyfra21-1 and neuron-specific enolase (NSE) known as tumor markers probably can be useful serum markers for diagnosis of PAP. As for the method of pathologic diagnosis, open-lung biopsy was the gold standard but now it is less required because findings on examination of bronchoalveolar lavage fluid (BALF) can help to make the diagnosis. We also have deep experience about when and how to carry out lung lavage.

摘要

肺泡蛋白沉积症(PAP)是一种罕见的间质性肺疾病,其特征为表面活性物质成分在肺泡内异常蓄积。由于PAP罕见且缺乏特异性临床症状,其诊断很容易被漏诊。更好地了解临床诊断PAP的关键线索,并在间质性肺疾病或其他有相似表现的疾病的鉴别诊断中考虑到PAP,具有重要意义。在此,我们分析了当地医院11例PAP患者的临床特征,并复习相关文献,以便为PAP的诊断和治疗提供更多信息。在我们的观察中,作为肿瘤标志物的细胞角蛋白19片段(cyfra21-1)和神经元特异性烯醇化酶(NSE)可能是诊断PAP有用的血清标志物。至于病理诊断方法,开胸肺活检曾是金标准,但现在由于支气管肺泡灌洗术(BALF)检查结果有助于诊断,其需求已减少。我们在何时以及如何进行肺灌洗方面也有丰富经验。

相似文献

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[Pulmonary alveolar proteinosis: a report of 2 cases, with literature review].
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Pulmonary alveolar proteinosis in pediatric leukemia.小儿白血病中的肺泡蛋白沉积症
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本文引用的文献

6
Pulmonary alveolar proteinosis.肺泡蛋白沉积症
N Engl J Med. 2003 Dec 25;349(26):2527-39. doi: 10.1056/NEJMra023226.
7
Pulmonary alveolar proteinosis.肺泡蛋白沉积症
N Engl J Med. 1958 Jun 5;258(23):1123-42. doi: 10.1056/NEJM195806052582301.
8
Pulmonary alveolar proteinosis: progress in the first 44 years.肺泡蛋白沉积症:前44年的进展
Am J Respir Crit Care Med. 2002 Jul 15;166(2):215-35. doi: 10.1164/rccm.2109105.
9
CT features of pulmonary alveolar proteinosis.肺泡蛋白沉积症的CT特征。
AJR Am J Roentgenol. 2001 May;176(5):1287-94. doi: 10.2214/ajr.176.5.1761287.

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