title: Progesterone treatment in chylothorax associated with pulmonary tuberous sclerosis.
abstract: 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.
authors.affiliation: Service de Pneumologie et Unité de Réanimation Respiratoire du CentreHospitalier Universitaire d'Amiens, France.
authors.forename: V
authors.initials: V
authors.lastname: Jounieaux
authors.forename: S
authors.initials: S
authors.lastname: Druelle
authors.forename: I
authors.initials: I
authors.lastname: Mayeux
authors.forename: E
authors.initials: E
authors.lastname: Grimault
authors.forename: J C
authors.initials: JC
authors.lastname: Charet
mesh_terms: D000328:Adult; D002916:Chylothorax / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000150:complications*; D017258:Medroxyprogesterone Acetate / Q000627:therapeutic use*; D011030:Pneumothorax / Q000150:complications; D045167:Progesterone Congeners / Q000627:therapeutic use*; D014402:Tuberous Sclerosis / Q000150:complications*
chemical_list: D045167:Progesterone Congeners; D017258:Medroxyprogesterone Acetate