Kawanami O, Ferrans V J, Fulmer J D, Crystal R G
Am J Pathol. 1979 Feb;94(2):301-22.
Ultrastructural study of pulmonary biopsy specimens from patients with fibrotic lung disease disclosed the presence of nuclear inclusions in 1% or less of cuboidal alveolar epithelial cells in 9 of 19 patients, including 6 of 12 patients with idiopathic pulmonary fibrosis, 2 of 3 patients with collagen-vascular disease, and 1 of 3 patients with sarcoidosis. Nuclear inclusions were not observed by ultrastructural study in 5 control patients. The inclusions consisted of masses or aggregates of tubules which probably were derived from the inner nuclear membranes. These tubules were smooth-walled, showed branchings and bifurcations, were composed of single trilaminar membranes, usually had a clear content, and ranged from 500 to 1000 A in diameter. They resembled nuclear tubules which occur in other cell types under conditions of rapid growth or specific hormonal stimulation. Statistically significant differences between the groups of patients with and without nuclear inclusions in cuboidal alveolar epithelial cells were not found with respect to smoking history, degree of fibrosis in the lung biopsy specimen, or the degree of pulmonary physiologic impairment. However, the average age of the patients having nuclear inclusions was significantly greater than that of patients not having nuclear inclusions. In addition, the frequency of indentations in the nuclei of cuboidal alveolar epithelial cells was greater in patients with nuclear inclusions than in patients without nuclear inclusions. Highly significant correlations were observed between the presence of nuclear inclusions and the presence of a) anchoring fibrils and hemidesmosomes along the basal surfaces of alveolar epithelial cells and b) multilayering of the alveolar epithelium.
对肺纤维化疾病患者的肺活检标本进行超微结构研究发现，19例患者中有9例（包括12例特发性肺纤维化患者中的6例、3例胶原血管病患者中的2例以及3例结节病患者中的1例），1%或更少的立方肺泡上皮细胞中存在核内包涵体。5例对照患者经超微结构研究未观察到核内包涵体。这些包涵体由可能源自内核膜的小管团块或聚集体组成。这些小管壁光滑，有分支和分叉，由单层三层膜组成，通常内容物清晰，直径在500至1000埃之间。它们类似于在其他细胞类型快速生长或特定激素刺激条件下出现的核小管。在有和没有立方肺泡上皮细胞核内包涵体的患者组之间，在吸烟史、肺活检标本中的纤维化程度或肺生理损害程度方面未发现统计学上的显著差异。然而，有核内包涵体的患者的平均年龄显著高于没有核内包涵体的患者。此外，有核内包涵体的患者立方肺泡上皮细胞核内凹陷的频率高于没有核内包涵体的患者。在核内包涵体的存在与以下情况之间观察到高度显著的相关性：a）沿肺泡上皮细胞基底表面的锚定原纤维和半桥粒；b）肺泡上皮的多层化。
