ABSTRACT
Lymphangioleiomyomatosis is a rare disease which mostly occurs during the reproductive ages of premenopausal women. The presenting symptoms of lymphangioleiomyomatosis include cystic lung lesions, progressive dyspnea on exertion, and recurrent pneumothorax. HRCT shows bilateral diffusely scattered tiny cysts and PFT shows obstructive airflow limitation and decreased diffusion capacity. We report a case of lymphangioleiomyomatosis which presented with recurrent pneumothorax and characteristic cysts in HRCT. The diagnosis was confirmed by pathology. This is the first case of lymphangioleiomyomatosis diagnosed for a postmenopausal woman in Korea. Lymphangioleiomyomatosis must be considered in the diagnosis for a postmenopausal woman with characteristic symptoms and radiologic findings for Korean patients.
Subject(s)
Female , Humans , Diagnosis , Diffusion , Dyspnea , Korea , Lung , Lymphangioleiomyomatosis , Menopause , Pathology , Pneumothorax , Rare DiseasesABSTRACT
We report a case of heart-lung transplantation in a 32 year-old female with Eisenmenger syndrome secondary to patent ductus arteriosus. She has been suffered from congestive heart failure since June 1996 and repeatedly treated at Intensive Care Unit with intravenous inotropic support since July 1997. Preoperative echocardiography showed a patent ductus arteriosus with right to left shunt, severe regurgitation of tricuspid valve and estimated right ventricular systolic pressure of 100mmHg. The brain-dead donor was an 18 year-old male with head trauma from traffic accident 3 days ago. Heart-lung block procurement was performed at another general hospital and was transported to the Seoul National University Hospital by ambulance. Total ischemic time of the transplanted heart and lung were 249 minutes and 270 minutes, respectively. The immunosuppressive therapy was commenced preoperatively with cyclosporine and azathioprine. Corticosteroid was not used until postoperative 3 weeks in order to avoid infection and delayed healing at the tracheal anastomotic site. The patient was discharged at 31st postoperative day, and has been regularly followed up at outpatient clinic without specific complication. The follow-up bronchoscopy, performed 2 weeks and 4 months after surgery, revealed no evidence of cellular rejection.
Subject(s)
Adolescent , Adult , Female , Humans , Male , Accidents, Traffic , Ambulances , Ambulatory Care Facilities , Azathioprine , Blood Pressure , Bronchoscopy , Craniocerebral Trauma , Cyclosporine , Ductus Arteriosus, Patent , Echocardiography , Eisenmenger Complex , Follow-Up Studies , Heart , Heart Failure , Heart-Lung Transplantation , Hospitals, General , Intensive Care Units , Lung , Seoul , Tissue Donors , Tricuspid ValveABSTRACT
BACKGROUND: Since tumor necrosis factor was discovered in 1975, TNF has been well known about its cytotoxic effect on tumor cells in vivo and in vitro. According to the recent improvement of molecular biological techinques, it is possible that exogenous TNF gene is transferred to tumor cells and is expressed in theirs. By virtue of TNF gene transfer, we have expected that TNF expressed in TNF-gene-transferred tumor cells would kill tumor cells in vivo without systemic side effect. The expected mechanisms in which antitumor effects of TNF expressed in TNF-gene-transferred tumor cells are working would be as followings. In the first mechanism, TNF expressed in TNF-gene-transferred tumor cells would kill tumor cells around (like homicide). In the second mechanism, TNF expressed in TNF-gene-transferred tumor cells would kill themselves (like suicide). In the third mechanism, TNF expressed in TNF-gene-transferred tumor cells would recruit immune effector cells and kill tumor cells indirectly. In the last mechanism, TNF expressed in TNF-gene-transferred tumor cells would augment cytokine such as interferon-γ to kill tumor cells. Among these four mechanisms of antitumor effect, only the second mechanism has not been established yet. Therefore, to elucidate the second mechanism, We performed this study. METHOD: We transferred TNF-α gene to NCI-H2058, a human mesothelioma cell line and WEHI164, a murine fibrosarcoma cell line by using retroviral vector(pLT12SNTNF). And, We determined by using MTT assay whether TNF expressed in TNF-gene-transferred tumor cell lines would kill themselves like suicide or not. Then, if TNF-gene-transferred tumor cell lines would not suicide themselves, 1 would know more about the TNF sensitivity of TNF-gene-transferred tumor cell lines to exogenous TNF also by MTT assay. RESULT: NCI-H2058 and WEHI164 which were sensitive to TNF, became far less sensitive to endogenous and exogenous TNF after being transferred TNF-α gene to. CONCLUSION: TNF-gene-transfer to NCI-H2058 and WEHI164 gaffe them resistance to TNF.