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[一例合并先天性膈疝及右位心的右肺发育不全病例]

[A case of right pulmonary hypoplasia with congenital diaphragmatic hernia and dextrocardia].

作者信息

Andou A, Shimizu N, Okabe K, Date H, Teramoto S

机构信息

Department of Surgery, Okayama University School of Medicine, Japan.

出版信息

Nihon Kyobu Shikkan Gakkai Zasshi. 1992 Oct;30(10):1889-94.

PMID:1464995
Abstract

Chest X-ray of a 28-year-old woman revealed an abnormal shadow in the right lower lung field and dextrocardia, for which detailed investigation was performed. Since the CT number of the tumor shadow corresponded to that of the liver on chest CT, diaphragmatic hernia of the liver was suspected, and was confirmed by MRI and angiography of the abdomen. In addition, the pulmonary artery and vein were hypoplastic, and angiography of the pulmonary artery demonstrated pulmonary hypoplasia. This case was considered to have primary pulmonary hypoplasia, because the dextrocardia was considered to have occurred secondary to pulmonary hypoplasia and the diaphragmatic hernia of the liver was not sufficiently large to cause pulmonary hypoplasia. Pulmonary hypoplasia first diagnosed in adulthood is rare, with a clinical course and roentgenographic appearance differing from those of pulmonary hypoplasia in children.

摘要

一名28岁女性的胸部X光显示右下肺野有异常阴影及右位心,为此进行了详细检查。由于胸部CT上肿瘤阴影的CT值与肝脏的CT值相对应,怀疑为肝膈疝,并经腹部MRI和血管造影证实。此外,肺动脉和肺静脉发育不全,肺动脉造影显示肺发育不全。该病例被认为是原发性肺发育不全,因为右位心被认为是继发于肺发育不全,而肝膈疝不够大,不足以导致肺发育不全。成年期首次诊断出的肺发育不全很少见,其临床病程和影像学表现与儿童期肺发育不全不同。

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