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先天性肺泡毛细血管发育不良——新生儿呼吸窘迫的一种罕见病因。

Congenital alveolar capillary dysplasia--an unusual cause of respiratory distress in the newborn.

作者信息

Janney C G, Askin F B, Kuhn C

出版信息

Am J Clin Pathol. 1981 Nov;76(5):722-7. doi: 10.1093/ajcp/76.5.722.

Abstract

The clinical and anatomical features of a patient with an unusual pulmonary malformation, is reported. The clinical course was consistent with the syndrome of persistent fetal circulation; morphologically, however, the patient was found to have a unique form of pulmonary dysplasia. Failure of formation and ingrowth of alveolar capillaries led to absence of normal air-blood barriers in this term infant. In addition anomalous veins were present in the bronchovascular bundles. Morphometric study indicated that the lungs were otherwise mature. This selective deficiency and dysplasia suggests that distal pulmonary epithelial and vascular development operate under separate control mechanisms.

摘要

本文报告了一名患有罕见肺部畸形患者的临床及解剖学特征。临床病程符合持续性胎儿循环综合征;然而,在形态学上,该患者被发现患有独特形式的肺发育不良。肺泡毛细血管形成及向内生长失败导致该足月儿缺乏正常的气血屏障。此外,支气管血管束中存在异常静脉。形态计量学研究表明,肺部在其他方面是成熟的。这种选择性缺陷和发育不良提示远端肺上皮和血管发育受不同的控制机制调节。

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