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一名患有与胎儿肺发育相关病症的新生儿出现喉闭锁、胸膜固定术和膈肌肥大。

Laryngeal atresia, pleurodesis, and diaphragmatic hypertrophy in a newborn infant with findings relevant to fetal lung development.

作者信息

Walton J C, Silver M M, Chance G W, Vilos G A

出版信息

Pediatr Pathol. 1985;4(1-2):1-11. doi: 10.3109/15513818509025898.

Abstract

A rare congenital malformation, cartilaginous subglottic laryngeal atresia, was found associated with developmental growth disturbances in the lungs (hypoplasia), diaphragm (hypertrophy), and pleural cavities (pleurodesis) in a newborn premature male infant who died immediately after birth. Because of coexistent esophageal atresia, tracheoesophageal fistula, and anal atresia, the lower respiratory tract and gastrointestinal canal together formed a closed system throughout fetal life. A mechanism whereby diaphragmatic hypertrophy and mesenchymal obliteration of the pleural cavities may have evolved during uterine development is suggested. Since pulmonary hypoplasia was also present, an explanation of this unique constellation of development abnormalities has a bearing on normal lung development.

摘要

在一名出生后即死亡的早产男婴中,发现一种罕见的先天性畸形——软骨性声门下喉闭锁,同时伴有肺部发育生长障碍(肺发育不全)、膈肌(肥大)和胸腔(胸膜粘连)异常。由于并存食管闭锁、气管食管瘘和肛门闭锁,下呼吸道和胃肠道在整个胎儿期共同形成了一个封闭系统。本文提出了一种在子宫发育过程中膈肌肥大和胸腔间充质闭塞可能的演变机制。鉴于同时存在肺发育不全,对这一独特的发育异常组合的解释与正常肺发育相关。

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