1/8. Pulmonary lymphangiomyomatosis treated by single lung transplantation.Pulmonary lymphangiomyomatosis is a rare disease resistant to almost all medical treatments to date. We describe the case of a 44-yr-old woman with end-stage pulmonary lymphangiomyomatosis who was treated by single-lung transplantation. The patient is doing well in her sixteenth post-transplantation month and has a marked improvement in her pulmonary function tests and walking distance as compared with preoperative values, and she is enjoying an unrestricted life-style.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Pulmonary lymphangioleiomyomatosis followed by a localized retroperitoneal lymphangioleiomyoma.lymphangioleiomyomatosis is a rare disease that affects females of reproductive age. Microscopically, it is characterized by abnormal proliferation of immature smooth muscle-like cells that grow diffusely in the lung. Extrapulmonary manifestations in the mediastinum, peritoneum and pelvic lymph nodes are uncommon. We here describe a patient who initially presented with pulmonary lymphangioleiomyomatosis and subsequently developed a localized retroperitoneal mass. Pathologic examination showed that the mass was a lymphangioleiomyoma. The result of the immunohistochemical study was the same as that for the pulmonary lesion. It is therefore suggested that metastatic lymphangioleiomyoma should be included in the differential diagnosis in the patient with pulmonary lymphangioleiomyomatosis presenting with an extrapulmonary lesion.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Treatment of lymphangioleiomyomatosis. A meta-analysis.lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. It is sufficiently uncommon that randomization or any other systematic evaluation of regimens of treatment has been difficult. review of scattered case reports implies that a number of hormonal manipulations may be equally effective. A comprehensive review of the literature revealed 30 cases of LAM treated with eight regimens of treatment. Evaluation with predetermined criteria (meta-analysis) shows that administration of progesterone or oophorectomy or both are the most effective treatments, resulting in improvement or stabilization of the disease in the majority of cases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Pulmonary lymphangiomyomatosis. Report of a case with immunohistochemical and ultrastructural findings.Pulmonary lymphangiomyomatosis is a rare disease characterized by wide-spread proliferation of smooth muscle cells around lymphatic and blood vessels, smaller airways and in the interstitium. Young women in the fertile status are almost exclusively affected. Spontaneous recurrent pneumothorax is the most important symptom. Chylous effusions are reported in 37.5% of cases. Our observation deals with a woman aged 43. light- and electron-microscopic findings are described. Estrogen receptors have been detected on smooth muscle cells. Bilateral oophorectomy and treatment with progesterone were successful.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Pulmonary lymphangiomyomatosis.Pulmonary lymphangiomyomatosis is a rare disease of smooth muscle proliferation in the walls of lymphatic vessels and in the interstitial areas of the lungs. It only affects women of reproductive age. The majority of patients die from respiratory failure within 10 years. A new observation of pulmonary lymphangiomyomatosis is reported in a woman of 36 years. The disease was discovered at an advanced stage of respiratory failure. Left-sided pleurectomy and treatment with medroxy-progesterone and tamoxifen did not improve the patient's condition.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. Pulmonary lymphangiomyomatosis: treatment with castration.Pulmonary lymphangiomyomatosis (PLM) is a rare disease of smooth muscle proliferation that generally leads to an early death due to respiratory insufficiency. The disease only affects women. Exacerbations with pregnancy and after hormonal manipulation have been documented. There is presently no successful treatment of the disease. castration as a means of early therapy has been associated with a stable respiratory picture in our patient. Peritoneal-jugular shunt can benefit women with significant ascities.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. lymphangioleiomyomatosis.Lymphangiomyomatosis (LAM) is a progressive disease afflicting women of childbearing age. LAM remains a rare disease, unfamiliar to many clinicians. It usually poses a diagnostic dilemma for the primary physician, possibly resulting in a delayed or missed diagnosis, unnecessary operative intervention, and inappropriate therapy. LAM is characterized microscopically by abnormal smooth muscle proliferation causing gradual obstruction of small airways, lymphatics, and vasculature. The proliferation eventually results in a characteristic clinical syndrome of progressive dyspnea, recurrent pneumothorax, chylous effusion, and hemoptysis. Despite a variety of treatment regimens developed since the first description of LAM, patient survival has not improved appreciably. Most patients die within 10 years of the time of diagnosis. This report presents a patient with LAM and a review of the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Pulmonary lymphangioleiomyomatosis--a case report.lymphangioleiomyomatosis is a rare disease entity. A case of a 51-year-old man treated for respiratory insufficiency has been reported. Due to disseminated lesions in the pulmonary tissue the patient was assessed for the presence of sarcoidosis. Histopathological examination of the lung specimen obtained during videothoracoscopy revealed lesions of the lymphangioleiomyomatosis type.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |