Lung Transplantation in the Management of Patients With Lymphangioleiomyomatosis: Baseline Data From the NHLBI LAM Registry
| Autor: | Eric Olson, Joel Moss, Jeffrey T. Chapman, Susan Sherer, Gerald J. Beck, Jay H. Ryu, Kevin K. Brown, June L. McMahan, Stephen J. Ruoss, Geraldine A. Finlay, Jar Chi Lee, Janet R. Maurer |
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| Rok vydání: | 2007 |
| Předmět: |
Adult
Pulmonary and Respiratory Medicine medicine.medical_specialty Lung Neoplasms Biopsy medicine.medical_treatment Article Pulmonary function testing Quality of life Internal medicine medicine Humans Lung transplantation Medical history Longitudinal Studies Lymphangioleiomyomatosis Registries Lung Transplantation medicine.diagnostic_test business.industry Middle Aged medicine.disease United States Respiratory Function Tests Surgery Treatment Outcome medicine.anatomical_structure Quality of Life Female National Heart Lung and Blood Institute (U.S.) Cardiology and Cardiovascular Medicine business Lung Transplantation |
| Zdroj: | The Journal of Heart and Lung Transplantation. 26:1293-1299 |
| ISSN: | 1053-2498 |
| DOI: | 10.1016/j.healun.2007.09.013 |
| Popis: | Background In 1997, the National Heart, Lung, and Blood Institute of the National Institutes of Health established a Registry to better characterize the demographic, clinical, physiologic and radiographic features of patients with lymphangioleiomyomatosis (LAM). Herein we report data collected at enrollment from patients who had either undergone transplant prior to enrollment, underwent transplant during the 5-year study, or were evaluated/wait-listed for lung transplant during the 5-year study. Methods The LAM Registry enrolled patients from six clinical centers between August 1998 and October 2001. On entry, patients filled-out questionnaires covering their medical history, symptoms, treatment and quality of life (SF-36 and St. George’s Respiratory Questionnaire). Enrollees underwent blood laboratory work and testing for arterial blood gases and pulmonary function. Follow-up was done at 6-month and/or yearly intervals. Diagnoses were confirmed by biopsy or typical clinical presentation plus computerized tomography (CT) findings confirmed by independent expert radiologists. A total of 243 women were enrolled. Of these, 13 (5.3%) had been transplanted at time of entry (Group A), 21 (8.6%) were transplanted during the study (Group B), and 48 (19.8%) were either wait-listed for transplant or underwent evaluation after enrollment during the study period (Group C). The remaining 161 (66.3%) registrants were neither considered for nor listed for transplant during the Registry period (Group D). Results One-third of patients in a large sample of LAM patients had either been transplanted or were being considered for transplant. At enrollment, patients who had already been transplanted and those not in need of transplant (Groups A and D) had better pulmonary function and quality-of-life scores compared with patients who subsequently underwent lung transplant during the Registry period (Group B). Conclusions In this large Registry of LAM patients, lung transplantation appears to be associated both with significantly improved lung function and quality of life compared with patients with advanced disease. |
| Databáze: | OpenAIRE |
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