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马方综合征所致肺尖部病变误诊为肺结核。

Apical pulmonary lesions due to Marfan syndrome misdiagnosed as pulmonary tuberculosis.

作者信息

Gupta Prem P, Gupta Krishan B, Gulia Joginder S, Yadav Rohtas, Kumar Sanjeev, Agarwal Dipti

机构信息

Respiratory Medicine, Postgraduate Institute of Medical Sciences, University of Health Sciences, Rohtak, India.

出版信息

N Z Med J. 2010 Jul 16;123(1318):67-72.

Abstract

A 55-year-male with chest symptoms and apical pulmonary lesions was diagnosed as a case of sputum smear-negative pulmonary tuberculosis at a peripheral health centre in India on the basis of Revised National Tuberculosis Control Programme Guidelines--he was put on antitubercular chemotherapy. He had no radiological or clinical improvement with antitubercular treatment, so the patient was referred to our institute. On evaluation, we found that the patient had multisystem involvement with typical features of Marfan syndrome and a suggestive history in other blood-relatives. Upper lobe fibrosis, bronchiectasis, emphysematous changes, multiple blebs, small pneumothorax, pleural fibrosis and pleural thickening were seen which were due to Marfan syndrome rather than tuberculosis. The present case seems to signify the search for alternative aetiologies in similar clinico-radiological presentations if, after 3 months, cultures for Mycobacterium are still negative (despite sputum induction and/or bronchoscopy with biopsies) and the patient is having no radiological improvement.

摘要

一名55岁男性,有胸部症状及肺尖部病变,在印度一家基层健康中心根据修订的国家结核病控制规划指南被诊断为痰涂片阴性肺结核病例,随后接受抗结核化疗。接受抗结核治疗后,他的影像学及临床症状均无改善,因此该患者被转诊至我院。经评估,我们发现该患者有多系统受累,具有马方综合征的典型特征,且家族中其他血亲也有类似病史。可见上叶纤维化、支气管扩张、肺气肿改变、多发肺大疱、小气胸、胸膜纤维化及胸膜增厚,这些均由马方综合征而非结核病所致。如果3个月后结核分枝杆菌培养仍为阴性(尽管进行了痰液诱导和/或支气管镜检查及活检)且患者影像学无改善,本病例似乎表明在类似临床-影像学表现中应寻找其他病因。

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