PULMONARY ALVEOLAR PROTEINOSIS
PULMONARY ALVEOLAR PROTEINOSIS
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DOI：
10.1056/nejm195806052582301
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发表时间：
1958-01-01
影响因子：
158.5
通讯作者：
HUNT, RTN
中科院分区：
文献类型：
--
作者：
ROSEN, SH;CASTLEMAN, B;HUNT, RTN
本文根据27例患者的研究，描述了一种具有特征性组织学模式，但临床病程多变的肺部慢性疾病，这些患者的肺组织主要是活检材料。病变包括PAS（周期性酸-希夫）阳性颗粒状和絮状蛋白物质的沉积，富含脂质，在远端空气空间的管腔内，通常很少发生反应。这种物质来源于增殖的“间隔细胞”的颗粒转化及其随后的脱落和坏死。针状晶体和不同的，强烈染色的层状体，被认为是细胞碎片，在大多数情况下，在某些领域大量存在。影像学表现与肺水肿相似，但变化可能持续数月或数年。在临床上，这种疾病可能有发热性疾病，也可能没有。通常表现为呼吸困难，常伴有咳嗽，有时痰黄，易疲劳和体重减轻。关于这种情况的病因尚不清楚。
A chronic disease of the lungs with a characteristic histologic pattern, but variable clinical course, is described on the basis of the study of 27 patients from whom lung tissue, principally biopsy material, has been taken. The lesion consists of the deposition of a PAS (periodic acid-Schiff)-positive granular and floccular proteinaceous material, rich in lipids, within the lumens of the distal air spaces, where little, if any, reaction is usually produced. This material is derived from granular transformation of proliferated "septal cells," and their subsequent sloughing and necrosis. Acicular crystals, and variable, intensely staining laminated bodies, believed to be cellular fragments, were abundant in some fields in most cases. Radiographically, the appearance simulates that of pulmonary edema, but the changes may persist for months or years. Clinically, the disease may or may not be heralded by a febrile illness. It is usually manifested by dyspnea, often associated with cough, sometimes yielding a yellow sputum, and by increased fatigability and loss of weight. Nothing is known regarding the etiology of the condition.