Dovey M, Wisseman C L, Roggli V L, Roomans G M, Shelburne J D, Spock A
Department of Pathology, Duke University, Medical Center, Durham, North Carolina.
J Submicrosc Cytol Pathol. 1989 Jul;21(3):521-34.
Cystic fibrosis (CF) is the most common lethal genetic disease among Caucasians, with much of the morbidity and most of the mortality related to pulmonary complications. The underlying defect in this disease has yet to be precisely defined, so it is somewhat surprising that a comprehensive study of the ultrastructural morphology of the lung in CF has not heretofore been reported. We used transmission electron microscopy to examine the small airways in 15 patients who had died of CF, and compared the findings with 15 disease controls with non-CF chronic airways disease and 15 patients with normal lung morphology. The lung parenchyma was also examined ultrastructurally in 7 patients with CF, 4 disease controls, and 4 normal lung cases. In addition, the literature regarding the ultrastructural morphology of the large airways in CF was reviewed. Patients with CF showed non-specific ciliary abnormalities, hyperplasia of mucous cells, increased numbers of pulmonary neuroendocrine and indeterminate cells, degeneration and sloughing of epithelial cells, and colonization of bacteria of the mucous layer of the small airways when compared with normal controls. Alveoli showed non-specific injury and regeneration of type II pneumocytes. However, these changes were all similar to those observed in the disease controls. Specifically, no cellular or subcellular ultrastructural abnormality unique to CF was observed. It is probable that the most useful ultrastructural approach to the lung in CF in future studies will involve X-ray microanalytical studies of ionic composition using cryotechniques.
囊性纤维化（CF）是白种人中最常见的致死性遗传病，其大部分发病率和多数死亡率与肺部并发症相关。该疾病的潜在缺陷尚未得到精确界定，因此，此前尚未有关于CF患者肺部超微结构形态的全面研究报道，这多少有些令人惊讶。我们使用透射电子显微镜检查了15例死于CF的患者的小气道，并将结果与15例患有非CF慢性气道疾病的疾病对照以及15例肺形态正常的患者进行了比较。还对7例CF患者、4例疾病对照和4例肺正常病例的肺实质进行了超微结构检查。此外，还回顾了关于CF患者大气道超微结构形态的文献。与正常对照相比，CF患者表现出非特异性纤毛异常、黏液细胞增生、肺神经内分泌细胞和不确定细胞数量增加、上皮细胞变性和脱落以及小气道黏液层细菌定植。肺泡显示出非特异性损伤和II型肺泡上皮细胞再生。然而，这些变化与疾病对照中观察到的变化均相似。具体而言，未观察到CF特有的细胞或亚细胞超微结构异常。在未来的研究中，对CF患者肺部最有用的超微结构研究方法可能是使用低温技术对离子组成进行X射线微分析研究。
