Nakamura Y, Fukuda S, Hashimoto T
Department of Pathology, St. Mary's Hospital, Kurume-shi, Japan.
Pediatr Pathol. 1990;10(5):689-706. doi: 10.3109/15513819009064705.
Normal human pulmonary elastic fiber development and development in some pathological conditions were examined using elastic stains by light microscopy, electron microscopy, and immunohistochemistry. In normal development elastic fibers, composed mainly of microfibrils, first appeared around primitive bronchioles at 10 weeks of gestation. As they matured, their appearance became more amorphous, and they extended into the peripheral alveolar walls. Development of elastic fibers was retarded in the hypoplastic lungs of the oligohydramnios syndrome, diaphragmatic hernia, and hydrops fetalis. Elastic development was also retarded in congenital pulmonary lymphangiectasia and in focal areas of lungs with pulmonary dysplasia. Distribution of well-developed elastic fibers was found around the dilated bronchioles and alveoli in cases of congenital cystic adenomatoid malformation and extralobar pulmonary sequestration. Elastic fibers were distributed irregularly and unevenly in the lungs of bronchopulmonary dysplasia and ventilated cases of Wilson Mikity syndrome. In addition, four very immature infants who had progressively deteriorating respiratory function showed an almost total lack of elastic fibers in their alveolar walls.
利用弹性染色法，通过光学显微镜、电子显微镜和免疫组织化学技术，对正常人类肺弹性纤维的发育以及某些病理状况下的发育情况进行了研究。在正常发育过程中，主要由微原纤维组成的弹性纤维在妊娠10周时首次出现在原始细支气管周围。随着它们的成熟，其外观变得更加无定形，并延伸至外周肺泡壁。羊水过少综合征、膈疝和胎儿水肿等肺发育不全的肺部中，弹性纤维的发育受到阻碍。先天性肺淋巴管扩张症以及肺发育异常的肺部局部区域，弹性纤维的发育也受到阻碍。在先天性囊性腺瘤样畸形和肺叶外型肺隔离症病例中，在扩张的细支气管和肺泡周围发现了发育良好的弹性纤维分布。在支气管肺发育不良和Wilson Mikity综合征通气病例的肺部中，弹性纤维分布不规则且不均匀。此外，四名呼吸功能逐渐恶化的极不成熟婴儿，其肺泡壁几乎完全缺乏弹性纤维。
