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[儿童先天性大叶性肺气肿]

[Congenital lobar emphysema in childhood].

作者信息

Mikhaĭlova V

出版信息

Khirurgiia (Sofiia). 1996;49(3):8-12.

PMID:9011675
Abstract

Over a 20-year period, thirty-two children with infantile (congenital) lobar emphysema (ILE) are subjected to treatment in the department of pediatric chest surgery--Emergency Medicine Institute "N. I. Pirogov". The age distribution of the patients is as follows: neonates--7 (21.8 per cent), sucklings--17 (53.8 per cent), 1 y.--5 (15.6 per cent), 2 y.--2 (6.2 per cent), 12 y.--1 (3.1 per cent). In seventeen children (53.3 per cent) the lobar emphysema is located in the left upper lobe. The onset of the clinical picture is in the neonatal period (17 cases, 53.3 per cent) and in the suckling age (13 cases, 40.6 per cent), and becomes manifest with: dyspnea--16 children (50.0 per cent), tachypnea--26 (81.2 per cent) and cyanosis--18 (56.2 per cent). Twenty-seven children are admitted with varying degree respiratory insufficiency (84.4 per cent). Diagnosis is made on the ground of conventional roentgen examinations mainly. Stenosis of the respective lobar bronchus is discovered in 8 children by CAT and bronchoscopy. Scarce vascular pattern is established angiopulmographically in 87.5 per cent of children, and fan-like displacement of the vessels-in 75.0 per cent. All children undergo operation--removal of the emphysematous lobe. Morphological study of the resected lung shows cartilage deficit in the bronchial wall in 14 children (34.5 per cent), bronchial stenosis in 26.2 per cent whereas in 43.8 per cent the underlying cause of ILE is unclear. In 4 children (12.5 per cent) the outcome is lethal because of inflammatory pulmonary process in the postoperative period.

摘要

在20年期间,32例患有婴儿(先天性)大叶性肺气肿(ILE)的儿童在“N. I. 皮罗戈夫”急诊医学研究所小儿胸外科接受治疗。患者的年龄分布如下:新生儿——7例(21.8%),乳儿——17例(53.8%),1岁——5例(15.6%),2岁——2例(6.2%),12岁——1例(3.1%)。17例儿童(53.3%)的大叶性肺气肿位于左上叶。临床表现的发作在新生儿期(17例,53.3%)和乳儿期(13例,40.6%),表现为:呼吸困难——16例儿童(50.0%),呼吸急促——26例(81.2%),发绀——18例(56.2%)。27例儿童因不同程度的呼吸功能不全入院(84.4%)。诊断主要基于传统的X线检查。通过CT和支气管镜检查在8例儿童中发现了相应叶支气管狭窄。87.5%的儿童通过肺血管造影显示血管纹理稀少,75.0%的儿童显示血管呈扇形移位。所有儿童均接受手术——切除肺气肿叶。对切除肺的形态学研究显示,14例儿童(34.5%)的支气管壁软骨缺乏,26.2%的儿童有支气管狭窄,而43.8%的ILE潜在病因不明。4例儿童(12.5%)因术后肺部炎症过程导致死亡。

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[Congenital lobar emphysema in childhood].[儿童先天性大叶性肺气肿]
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Congenital lobar emphysema in neonates: Anaesthetic challenges.新生儿先天性大叶性肺气肿:麻醉挑战
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Congenital lobar emphysema: a diagnostic challenge and cause of progressive respiratory distress in a 2 month-old infant.先天性大叶性肺气肿:一名2个月大婴儿的诊断难题及进行性呼吸窘迫的病因
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