PULMONARY ALVEOLAR PROTEINOSIS - REPORT OF 6 CASES REVIEW OF LITERATURE AND FORMULATION OF A NEW THEORY
PULMONARY ALVEOLAR PROTEINOSIS - REPORT OF 6 CASES REVIEW OF LITERATURE AND FORMULATION OF A NEW THEORY
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DOI：
10.7326/0003-4819-62-2-292
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发表时间：
1965-01-01
影响因子：
39.2
通讯作者：
GORDINIER, R
中科院分区：
文献类型：
--
作者：
LARSON, RK;GORDINIER, R
本文报告85例肺泡蛋白沉积症，其中6例为新病例。几个重要的临床特征得到确认，需要重新强调。合并肺部感染是常见且危险的。从这些病人身上分离出的各种各样的致病生物，特别是真菌，强调了文化研究在指导治疗中的重要性。感染的特异性治疗和促进肺泡物质消除的非特异性方法已经成功地保持了大多数患者的生命和良好的状态。肾上腺类固醇的使用似乎是禁忌症。提出了一种新的发病机制理论。指出肺泡蛋白沉积的肺泡填充物质与正常肺泡上皮细胞分泌的表面活性剂在化学上的相似性。某些疾病，如新生儿呼吸窘迫综合征，可归因于缺乏这种表面活性物质。通过类比其他条件，似乎也有可能发现一种与分泌过多相对应的疾病。我们认为肺泡蛋白沉积症可能就是这样一种疾病。虽然这一理论不能进一步解释疾病的病因，但它可能为今后的研究提供一个有用的概念。
Eighty-five cases of pulmonary alveolar proteinosis, including 6 new cases, are reviewed. Several important clinical features are confirmed and need re-emphasis. Superimposed pulmonary infection is frequent and hazardous. The wide variety of pathogenic organisms, especially fungi, that have been isolated from these patients emphasizes the importance of cultural studies in guiding treatment. Specific therapy of infections and nonspecific methods of promoting the elimination of the alveolar material have been successful in keeping the majority of patients alive and in good condition. The use of adrenal steroids would appear to be contraindicated. A new theory of pathogenesis has been proposed. The chemical similarity between the alveolar filling material in pulmonary alveolar proteinosis and the normal surface-active agent secreted by the alveolar epithelial cells has been pointed out. Certain diseases such as the respiratory distress syndrome of newborns have been attributed to a deficiency of this surface-active material. By anaology to other conditions, it would seem plausible that a disease corresponding to hypersecretion would also be found. It is proposed that pulmonary alveolar proteinosis might be such a disease. Although this theory does not go much further in explaining the etiology of the disease, it may serve as a useful concept for future investigation.