Pulmonary lymphangiomyomatosis: with particular reference to steroid-receptor assay studies and pathologic correlation.
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.
D000328:Adult; D003600:Cytosol / Q000032:analysis; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000032:analysis* / Q000473:pathology; D008203:Lymphangiomyoma / Q000032:analysis* / Q000473:pathology; D008232:Lymphoproliferative Disorders; D011960:Receptors, Estrogen / Q000032:analysis; D011980:Receptors, Progesterone / Q000032:analysis; D011987:Receptors, Steroid / Q000032:analysis*
D011960:Receptors, Estrogen; D011980:Receptors, Progesterone; D011987:Receptors, Steroid