Graham M L, Spelsberg T C, Dines D E, Payne W S, Bjornsson J, Lie J T
Mayo Clin Proc. 1984 Jan;59(1):3-11. doi: 10.1016/s0025-6196(12)60333-4.
The symptoms of progressive dyspnea, hemoptysis, spontaneous pneumothorax, chylous effusions, and cough in conjunction with ventilatory obstruction and abnormal gas exchange in a young woman should prompt the diagnosis of pulmonary lymphangiomyomatosis. Cytosol steroid-receptor assays and postmortem studies were conducted in an extensive investigation of a case of this disease. A biopsy specimen of the lung disclosed evidence of nuclear translocation of [3H]progesterone and the presence of a cytosolic receptor for progesterone, an indication that this disease could be treated effectively with progestin.
一名年轻女性出现进行性呼吸困难、咯血、自发性气胸、乳糜性胸腔积液和咳嗽等症状，同时伴有通气障碍和气体交换异常，应考虑诊断为肺淋巴管平滑肌瘤病。针对该疾病的一例病例进行了广泛调查，包括细胞溶质类固醇受体测定和尸检研究。一份肺活检标本显示存在[3H]孕酮的核转位以及孕酮的细胞溶质受体，这表明该疾病可用孕激素有效治疗。
