NDLI: Lung transplantation for lymphangioleiomyomatosis: the French experience.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Reynaud-Gaubert, Martine Mornex, Jean-François Mal, Hervé Treilhaud, Michèle Dromer, Claire Quétant, Sébastien Leroy-Ladurie, François Guillemain, Romain Philit, François Dauriat, Gaëlle Grenet, Dominique Stern, Marc
Spatial Coverage	France
Description	Country affiliation: France Author Affiliation: Reynaud-Gaubert M ( The Divisions of Pulmonary Medicine and Thoracic Surgery, Hôpital Ste Marguerite, Marseille, France. martine.reynaud@ap-hm.fr)
Abstract	BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare disease, leading in some cases to end-stage respiratory failure. Lung transplantation (LT) represents a therapeutic option in advanced pulmonary LAM. METHODS: We conducted a retrospective multicenter study of 44 patients who underwent LT for LAM at 9 centers in France between 1988 and 2006. RESULTS: All patients were women with a mean age of 41+/-10 years at LT. There were 34 single-lung transplants and 11 bilateral transplants (one retransplantation). Prior clinical events related to LAM were present in 75% of the patients and previous thoracic surgical procedures were noted in 86.6% of cases. At the latest preoperative evaluation, 30 patients had an obstructive pattern (mean forced expiratory volume in 1 second: 26%+/-14% of predicted) and 15 had a combined restrictive and obstructive pattern, with a mean KCO=27%+/-8.8% of predicted, PaO2=52.8+/-10.4 and PaCO2=42.6+/-9.8 mm Hg. Intraoperative cardiopulmonary bypass was required in 13 cases. The length of mechanical ventilation was 7.5+/-12.8 days. The median duration of follow-up was 37 months. The 1, 2, 5, and 10 years survival rates were 79.6%, 74.4%, 64.7%, and 52.4%, respectively. Extensive pleural adhesions were found in 21 patients leading to severe intraoperative hemorrhage. Postoperative LAM-related complications were pneumothorax in the native lung in five patients, chylothorax in six, bronchial dehiscence or stenosis in seven. There were two cases of recurrence of LAM. CONCLUSION: Despite a high morbidity mainly caused by previous surgical interventions and disease-related complications, LT is a satisfactory therapeutic option for end-stage respiratory failure in LAM.
File Format	HTM / HTML
ISSN	00411337
Issue Number	4
Volume Number	86
e-ISSN	15346080
Journal	Transplantation Journal
Language	English
Publisher	Lippincott Williams & Wilkins
Publisher Date	2008-08-27
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Transplantation Lung Neoplasms Surgery Lung Transplantation Statistics & Numerical Data Lymphangioleiomyomatosis Adult Biopsy Female France Functional Laterality Histocompatibility Testing Humans Intraoperative Complications Epidemiology Physiopathology Middle Aged Oxygen Consumption Respiratory Function Tests Retrospective Studies Smoking Questionnaires Treatment Failure Treatment Outcome Journal Article
Content Type	Text
Resource Type	Article
Subject	Transplantation

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