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新生儿中一种伴有机械性呼吸障碍的特殊形式颅面畸形(作者译)

[An unusual form of craniofacial dysmorphism with mechanical respiratory disturbances in newborns (author's transl)].

作者信息

Heinicke S, Roth B, Krüger G, Günther H

出版信息

Monatsschr Kinderheilkd. 1982 Feb;130(2):110-3.

PMID:7062919
Abstract

Gross and histologic findings at autopsy of a one week old newborn baby with respiratory distress and mechanical obstacles to adequate intubation revealed a variation of the craniofacial dysmorphy syndrome: hypoplasia of the mandible combined with dysplastic and hypoplastic tongue and complete situs inversus. The tongue consisted of an hypoplastic smooth anterior part and a hypertrophic posterior part with the usual papillae. Both parts were separated by a deep sulcus across the middle of the tongue with a predominantly fibrous amuscular bottom. Because of this anomaly the posterior part of the tongue was retracted covering the orifice of the larynx and thus causing the respiratory distress. The lesion is discussed in context with data of similar syndromes.

摘要

一名患有呼吸窘迫且存在充分插管机械障碍的一周大新生儿尸检时的大体及组织学发现显示出颅面发育异常综合征的一种变异

下颌骨发育不全合并发育异常及发育不全的舌头以及完全性内脏转位。舌头由发育不全的光滑前部和具有正常乳头的肥厚后部组成。两部分由一条横跨舌中部的深沟分隔,沟底主要为纤维组织且无肌肉。由于这种异常,舌头后部回缩覆盖了喉口,从而导致呼吸窘迫。结合类似综合征的数据对该病变进行了讨论。

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