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经支气管肺泡灌洗诊断的家族性肺泡微石症。病例报告。

Familial pulmonary alveolar microlithiasis diagnosed by bronchoalveolar lavage. A case report.

作者信息

Monabati Ahmad, Ghayumi Mohammad Ali, Kumar Perikala V

机构信息

Department of Pathology, Hematology Research Center, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Acta Cytol. 2007 Jan-Feb;51(1):80-2. doi: 10.1159/000325688.

DOI:10.1159/000325688
PMID:17328501
Abstract

BACKGROUND

Familial alveolar microlithiasis is a rare lung disease. In this study we describe the cytologic features of this disease in bronchoalveolar lavage.

CASE

A 10-year-old girl and her uncle, a 50-year-old man, had dyspnea and diffuse interstitial pattern on chest radiograph with no defined cause at a period of 10 years apart. Open lung biopsy in the girl and transbronchial lung biopsy plus bronchoalveolar lavage (BAL) in the man were per-formed to determine the diagnosis. In cyopen lung biopsy the diagnosis was alveolar microlithiasis. BAL revealed rehtypical microliths (calcospherites), and th transbronchial lung biopsy performed in the same patient failed to disclose superficially reset any significant pathology. In cytologic a smears, extracellular and intracellular concentrically layered purple-brown, round-to-oval microliths were clearly seen. Cyanophilic periodic acid-Schiff positive intracytoplasmic amorphous material was also frequently seen in alveolar macrophages.

CONCLUSION

Familial alveolar microlithiasis is a rare interstitial lung disease that can be easily diagnosed by BAL. This procedure is a very useful tool in diagnosing and classifying some interstitial lung diseases.

摘要

背景

家族性肺泡微石症是一种罕见的肺部疾病。在本研究中,我们描述了该疾病在支气管肺泡灌洗中的细胞学特征。

病例

一名10岁女孩及其50岁的叔叔,在相隔10年的时间里均出现呼吸困难,胸部X线片显示弥漫性间质改变,但病因不明。对女孩进行了开胸肺活检,对该男子进行了经支气管肺活检及支气管肺泡灌洗(BAL)以明确诊断。开胸肺活检诊断为肺泡微石症。BAL显示典型的微石(钙球),而对同一患者进行的经支气管肺活检未发现明显的病理改变。在细胞学涂片上,可清晰看到细胞外和细胞内呈同心层状的紫褐色、圆形至椭圆形微石。在肺泡巨噬细胞中也经常可见嗜酸性高碘酸希夫阳性的胞质内无定形物质。

结论

家族性肺泡微石症是一种罕见的间质性肺疾病,可通过BAL轻松诊断。该检查方法是诊断和分类某些间质性肺疾病的非常有用的工具。

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Familial pulmonary alveolar microlithiasis diagnosed by bronchoalveolar lavage. A case report.经支气管肺泡灌洗诊断的家族性肺泡微石症。病例报告。
Acta Cytol. 2007 Jan-Feb;51(1):80-2. doi: 10.1159/000325688.
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Pulmonary alveolar microlithiasis in childhood: diagnosis by transbronchial biopsy.儿童肺泡微结石症:经支气管活检诊断
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