LUNG IN CYSTIC-FIBROSIS - QUANTITATIVE STUDY INCLUDING PREVALENCE OF PATHOLOGIC FINDINGS AMONG DIFFERENT AGE-GROUPS
LUNG IN CYSTIC-FIBROSIS - QUANTITATIVE STUDY INCLUDING PREVALENCE OF PATHOLOGIC FINDINGS AMONG DIFFERENT AGE-GROUPS
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DOI：
10.1016/s0046-8177(76)80023-8
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发表时间：
1976-01-01
期刊：
影响因子：
3.3
通讯作者：
ROSENBERG, HS
中科院分区：
文献类型：
--
作者：
BEDROSSIAN, CWM;GREENBERG, SD;ROSENBERG, HS
本文对82例囊性纤维化尸检资料进行了回顾性分析。尽管支气管炎、粘液脓性堵塞和支气管扩张几乎普遍存在于所有年龄段的儿童中，但上皮化生和支气管扩张在婴儿中较为罕见，在老年组中逐渐更为普遍。2岁以下的患者没有肺气肿，2-6岁的患者中有11%和6岁以上的患者中有40%受到肺气肿的影响，但通过点计数法，肺气肿从未达到严重程度。肺出血虽然不常见，但通常与支气管扩张气道壁的突出动脉血管有关。支气管腺体定量评估显示，囊性纤维化患者的Reid指数显著高于非囊性纤维化患者，但这些指数并不随患者年龄的增长而增加。腺体肥大，腺体内粘液腺泡占优势，支气管粘膜杯状细胞增生，这些都可以解释囊性纤维化患者大量粘液分泌的原因。无法确定这些发现是否反映了此类患者的一般外分泌缺陷，或者它们是否仅仅是对慢性气道感染的反应，尽管后者是一个更合理的假设。
The autopsies of 82 patients with cystic fibrosis were reviewed with respect to pathologic changes in the lungs and their respective prevalence among different age groups. Although ronchitis, mucopurulent plugging and bronchopneumonia were almost universally present among children of all ages, epithelial metaplasia and bronchiectasis were rarer among infants and progressively more prevalent in older age groups. Emphysema was absent in patients under 2 yr of age and affected 11% of the patients 2-6 yr of age and 40% of the patients older than 6 yr but was never of a severe degree by the point count method. Pulmonary hemorrhage, although uncommon, was usually associated with prominent arterial vessels in walls of bronchiectatic airways. Quantitative assessment of bronchial glands revealed Reid indices significantly higher in patients with cystic fibrosis when compared to noncystic fibrosis patients, but there was no increase in these indices with the age of the patients. Glandular hypertrophy, predominance of mucous acini within glands and goblet cell hyperplasia of the bronchial mucosa all suggest an explanation for the copious mucous secretion of patients with cystic fibrosis. It was not possible to ascertain whether these findings reflect a general exocrine defect of such patients or whether they were merely a response to chronic airway infection, even though the latter is a more plausible assumption.