Adamson D, Heinrichs W L, Raybin D M, Raffin T A
Am Rev Respir Dis. 1985 Oct;132(4):916-21. doi: 10.1164/arrd.1985.132.4.916.
A 35-yr-old woman, with profound dyspnea at rest and failure to thrive, asked to be evaluated at Stanford Medical Center. She requested a second opinion after entering death counseling in another institution because of an unrelenting, deteriorating course caused by pulmonary lymphangiomyomatosis. Her recurring right chylous pleural effusion was drained by chest tube, a LaVeen shunt was placed to decompress her severe chylous ascites, an oophorectomy was performed, large dose progesterone (medroxyprogesterone acetate) was initiated intramuscularly, and during her hospitalization she was fed by total parenteral nutrition. She was discharged mildly improved after 2 months. Now, 3 yr later, her pulmonary function tests and chest radiographs are improved, and she walks 2 miles per day and teaches school full time. Extensive hormonal analysis did not reveal an endocrinologic abnormality that could explain the pathophysiology of her disease. However, the patient had multiple human chorionic gonadotropin injections before becoming ill, as have a number of other patients who have developed this disease. This report is an example of successful treatment of severe pulmonary lymphangiomyomatosis.
一名35岁女性，静息时即有严重呼吸困难且发育不良，遂要求在斯坦福医学中心接受评估。她因肺淋巴管平滑肌瘤病导致病情持续恶化，在另一机构接受临终咨询后寻求第二种意见。她反复出现的右侧乳糜胸通过胸腔引流管引流，放置了Laven分流管以减轻严重的乳糜性腹水，进行了卵巢切除术，开始肌肉注射大剂量孕激素（醋酸甲羟孕酮），住院期间通过全胃肠外营养进行喂养。2个月后出院时症状稍有改善。现在，3年后，她的肺功能测试和胸部X光片有所改善，每天能步行2英里，并且全职任教。广泛的激素分析未发现可解释其疾病病理生理的内分泌异常。然而，该患者在患病前接受过多次人绒毛膜促性腺激素注射，其他一些患此病的患者也是如此。本报告是严重肺淋巴管平滑肌瘤病成功治疗的一个实例。
