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肺结缔组织疾病是指肺的弹性纤维损坏、消失而导致肺结构破坏的疾病。它大致可分为肺气肿和胶原纤维增多的肺纤维症。正常肺组织富有弹性,能承受0~30cm 水柱的肺内外压力差,其容积变化可达10倍。Mead 认为弹性纤维与肺的弹性有密切关系,而胶原纤维则起“机械终止器”的作用。肺纤维症患者随着胶原纤维增加,肺的弹性下降,其结果肺功能受损、通气障碍,连残存的正常肺组织功能也受损,产生其他脏器纤维症时没有的严重症状。一、肺组织的细胞和结缔组织成分肺实质是由细胞(占80%)和结缔组织(占20%)组成。组成细胞有内皮细胞(38%)、间叶细胞(50%)、Ⅰ型肺泡上皮细胞(5%)及Ⅱ型肺泡上皮细胞(7%)。结缔组织是由
Pulmonary connective tissue disease refers to the destruction of the elastic fibers of the lungs, the disappearance of the lung structure damage. It can be roughly divided into emphysema and collagen fibers increased pulmonary fibrosis. Normal lung tissue is flexible, can withstand 0 ~ 30cm water column pressure difference between the inside and outside the lungs, the volume change up to 10 times. Mead thinks elastic fibers are closely related to the elasticity of the lungs, while collagen fibers act as “mechanical terminators.” Patients with pulmonary fibrosis with collagen fibers increased, decreased lung elasticity, resulting in impaired lung function, ventilation disorders, and even the function of residual normal lung tissue is also impaired, resulting in other organ failure without symptoms of fibrosis. First, the lung tissue cells and connective tissue components lung parenchyma by cells (80%) and connective tissue (20%). The constituent cells were endothelial cells (38%), mesenchyme (50%), type I alveolar epithelial cells (5%) and type II alveolar epithelial cells (7%). Connective tissue is made of