Lange F, Bignon J
Rev Fr Mal Respir. 1980;8(2):99-108.
The definition and the classification of bullae are recalled and documented by some examples. Two types of bullae are opposed : bulla found in normal parenchyma and bulla associated with widespread emphysematous lesions. The biochemical and biological mechanisms of the emphysematous changes are briefly described, with a distinction of two different pathogenesis : on one hand, an inherited defect in connective tissue synthesis as in Marfan syndrome, and on the other hand, an unbalance between proteases and antiproteases. The pathologist's contribution to the classification of the surgical forms of emphysema is documented by an electron microscopic study of lung biopsy in some cases : two cases with bullae (a Marfan syndrome and an alpha 1 AT deficiency of the Pi Z phenotype) with diffuse panlobular emphysema; and 4 cases of bullae without radiological evidence of emphysema. The elastic fibres were modified only in the 2 first cases but were normal in the others. A follow up of such cases is needed.
大疱的定义和分类通过一些实例进行回顾和记录。大疱有两种类型:正常实质内的大疱和与广泛肺气肿病变相关的大疱。简要描述了肺气肿改变的生化和生物学机制,区分了两种不同的发病机制:一方面,如马凡综合征那样结缔组织合成存在遗传性缺陷;另一方面,蛋白酶和抗蛋白酶之间失衡。病理学家对肺气肿手术形式分类的贡献通过对某些病例肺活检的电子显微镜研究得以记录:两例伴有弥漫性全小叶肺气肿的大疱(一例马凡综合征和一例Pi Z表型的α1抗胰蛋白酶缺乏症);以及4例无肺气肿影像学证据的大疱。仅前两例的弹性纤维发生了改变,其他病例的弹性纤维正常。需要对这些病例进行随访。