Jounieaux V, Druelle S, Mayeux I, Grimault E, Charet J C
Service de Pneumologie et Unité de Réanimation Respiratoire du CentreHospitalier Universitaire d'Amiens, France.
Eur Respir J. 1996 Nov;9(11):2423-5. doi: 10.1183/09031936.96.09112423.
We report the case of a young woman with tuberous sclerosis who developed a chylous pleural effusion after several invasive procedures for treatment of recurring pneumothoraces. Oophorectomy was rejected by the patient and progesterone therapy initiated. Medroxyprogesterone acetate administration led to a complete disappearance of the chylothorax in 8 months. The patient was kept on therapy, and no recurrence of pleural effusion has been observed up to the present (22 months follow-up). However, a progressive deterioration in pulmonary function was observed, with a significant decrease in pulmonary transfer factor and increase in airway obstruction. In conclusion, this report demonstrates an objective benefit of progesterone therapy on chylous effusion associated with pulmonary tuberous sclerosis.
我们报告了一例患有结节性硬化症的年轻女性病例，该患者在多次接受治疗复发性气胸的侵入性手术后出现了乳糜性胸腔积液。患者拒绝了卵巢切除术，并开始了孕激素治疗。醋酸甲羟孕酮的使用使乳糜胸在8个月内完全消失。患者持续接受治疗，截至目前（随访22个月）未观察到胸腔积液复发。然而，观察到肺功能逐渐恶化，肺转移因子显著降低，气道阻塞增加。总之，本报告证明了孕激素治疗对与肺部结节性硬化症相关的乳糜性胸腔积液具有客观益处。
