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婴儿肺泡毛细血管发育不良:一种罕见且致命的漏诊疾病。

Pulmonary alveolar capillary dysplasia in infants: A rare and deadly missed diagnosis.

作者信息

Saadi S, Masmoudi T, Ben Daly A, Jedidi M, Mokni M, Ben Dhiab M, Zemni M, Kamel Souguir M

机构信息

Department of legal medicine, Farhat Hached university hospital, 4000 Sousse, Tunisia; University of Sousse, 4000 Sousse, Tunisia.

Department of legal medicine, Farhat Hached university hospital, 4000 Sousse, Tunisia; University of Sousse, 4000 Sousse, Tunisia.

出版信息

Ann Cardiol Angeiol (Paris). 2022 Apr;71(2):112-114. doi: 10.1016/j.ancard.2020.07.017. Epub 2020 Aug 20.

Abstract

The pulmonary alveolocapillary dysplasia (ACD) with pulmonary vein misalignment (PVM) is a rare condition characterized by a congenital anomaly of the development of the pulmonary parenchyma. We present a case of an 8-month-old infant who died quickly from acute respiratory failure complicating an unknown ACD. We also describe its epidemiological characteristics in infants and we discuss the diagnosis's difficulties. In this case, a pulmonary arterial hypertension was decompensated by an infection. A medico-legal autopsy was performed. As for the Histological examination, it showed the features of ACD/PVM.

摘要

伴有肺静脉错位(PVM)的肺泡毛细血管发育不良(ACD)是一种罕见疾病,其特征为肺实质发育的先天性异常。我们报告一例8个月大的婴儿,因不明原因的ACD并发急性呼吸衰竭而迅速死亡。我们还描述了其在婴儿中的流行病学特征,并讨论了诊断的困难之处。在该病例中,肺动脉高压因感染而失代偿。进行了法医尸检。组织学检查显示为ACD/PVM的特征。

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