儿童期肺淋巴管平滑肌瘤病？一名青春期前女孩肺部平滑肌细胞显著增生，伴有反复气胸和进行性呼吸困难。
Pulmonary lymphangiomyomatosis in childhood? Marked smooth muscle cell proliferation of the lung in a preadolescent girl with repeated pneumothorax and progressive dyspnea.
作者信息
Sakano T, Hamasaki T, Kawaguchi Y, Tanaka Y, Ueda K, Hiramoto T
出版信息
Hiroshima J Med Sci. 1989 Sep;38(3):147-9.
PMID:2584060
Abstract
A 13-year-old girl with repeated spontaneous pneumothorax and progressive dyspnea is described. The biopsy specimen of the lung showed marked proliferation of smooth muscle cells in the thickened bullous wall and alveolar septa, which was similar to the findings of the pulmonary lymphangiomyomatosis. Pulmonary lymphangiomyomatosis is one of the diseases which should be considered when children have progressive dyspnea, chylous effusions and repeated spontaneous pneumothorax.
摘要
本文描述了一名13岁反复自发性气胸且进行性呼吸困难的女孩。肺部活检标本显示，增厚的肺大疱壁和肺泡间隔中有平滑肌细胞显著增殖，这与肺淋巴管平滑肌瘤病的表现相似。当儿童出现进行性呼吸困难、乳糜性胸腔积液和反复自发性气胸时，肺淋巴管平滑肌瘤病是应考虑的疾病之一。
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