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[特发性弥漫性肺骨化:一例报告及文献复习]

[Idiopathic diffuse pulmonary ossification: a case report and review of the literature].

作者信息

Bai Peng, Sun Yong-chang, Chen Dong-ning, Jin Jian-min, Zhuo Jie, Liu Hong-gang

机构信息

Department of Respiratory Medicine, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

出版信息

Zhonghua Jie He He Hu Xi Za Zhi. 2009 Aug;32(8):588-92.

Abstract

OBJECTIVE

To describe the clinical features and diagnosis of idiopathic diffuse pulmonary ossification (DPO).

METHODS

A case of DPO confirmed by video-assisted thoracoscopic (VATS) lung biopsy was reported, and the literature was reviewed.

RESULTS

A 32 year-old male was admitted to this hospital because of increased lung markings on chest X-ray for 7 years, and diffuse micro-nodular and reticular lesions on chest CT for 2 years. There were no significant symptoms, such as cough, sputum production and shortness of breath. Routine examinations and transbronchial lung biopsy failed to give a definite diagnosis, and therefore VATS lung biopsy was performed. The pathological study confirmed the presence of bone tissue in the lung, and the diagnosis of idiopathic DPD was made after careful exclusion of underlying diseases. Eleven cases of DPO diagnosed by lung biopsy in living patients were collected by review of the literature. The patients were all males, with a mean age of (48 +/- 17) years. No clinical symptoms were present in 4 cases, while spontaneous pneumothorax was the initial presentation in 3 cases. Other complaints included cough and shortness of breath. No case was reported in the Chinese literature.

CONCLUSION

DPO is a rare disease, often without significant symptoms despite radiologically diffuse pulmonary lesions, which are easily misdiagnosed as other interstitial lung diseases.

摘要

目的

描述特发性弥漫性肺骨化(DPO)的临床特征及诊断方法。

方法

报告1例经电视辅助胸腔镜(VATS)肺活检确诊的DPO病例,并复习相关文献。

结果

1例32岁男性因胸部X线显示肺纹理增多7年、胸部CT显示弥漫性微结节及网状病变2年入院。患者无咳嗽、咳痰及气短等明显症状。常规检查及经支气管肺活检未能明确诊断,遂行VATS肺活检。病理研究证实肺内存在骨组织,在仔细排除基础疾病后诊断为特发性DPO。通过文献复习收集到11例经肺活检确诊的现患DPO病例。患者均为男性,平均年龄(48±17)岁。4例无临床症状,3例以自发性气胸为首发表现。其他症状包括咳嗽和气短。国内文献尚无相关报道。

结论

DPO是一种罕见疾病,尽管影像学表现为弥漫性肺部病变,但常无明显症状,易被误诊为其他间质性肺疾病。

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