摘要
先天性肺囊性疾病是较少见的先天性肺部疾病,是肺组织胚胎发育异常所形成的畸形。囊性病变包括囊性腺瘤样畸形、支气管源性肺囊肿、先天性大叶性肺气肿、肺隔离症。此类疾病的临床表现相似,但发生机制及病理改变有各自的特点。囊性腺瘤样畸形是由于细支气管发育停滞,以肺泡不发育为代价引起的肺间质大量增生,目前根据病理类型分成5型。支气管源性肺囊肿是胚胎发育时期支气管树某段的异常出芽形成的。先天性大叶性肺气肿是支气管内阻外压以及支气管壁的病变造成的气体陷闭,肺泡腔扩张。肺隔离症是部分肺组织与正常的支气管肺组织无交通,供血来自体循环,病变肺无正常功能,分叶内型和叶外型。
Congenital cystic lung lesions are a group of congenital lung diseases with low incidence.These include congenital cystic adenomatoid malformation,bronchogenic cyst,congenital lobar emphysema and pulmonary sequestration.These malformations occur during the period of lung development stimulated by various factors.Manifestations of the diseases are very similar,but the pathogenesis and pathology are very different.Congenital cystic adenomatoid malformations are thought to be the results of the cessation of bronchiolar maturation with overgrowth of mesenchymal elements and without development of alveoli.There are 5 pathological types of congenital cystic adenomatoid malformations.Bronchogenic cysts are the results of abnormal budding from a segment of the tracheobronchial tree during embryo development,and the buds with no communication with normal tracheobronchial tree.Congenital lobar emphysema is a term reserved for hyperinflation of alveoli from idiopathic reasons or extrinsic compression,as well as pathological changes of the bronchial wall.Pulmonary sequestrations account for parts of nonfunctioning lung tissue that mostly do not communicate with normal bronchoalveolar tree and vascularized by a systemic artery,two types （intralobar and extralobar sequestration） are described.
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2010年第3期292-294,共3页
Journal of Clinical Pediatrics
关键词
先天性肺囊性疾病
先天性囊性腺瘤样畸形
支气管源性肺囊肿
先天性大叶性肺气肿
肺隔离症
肺发育
congenital cystic lung lesions
congenital cystic adenomatoid malformations
bronchogenic cysts
congenital lobar emphysema
pulmonary sequestrations
lung development
