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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-11518

Benden, C; Rea, F; Behr, J; Corris, P A; Reynaud-Gaubert, M; Stern, M; Speich, R; Boehler, A (2009). Lung transplantation for lymphangioleiomyomatosis: the European experience. Journal of Heart and Lung Transplantation, 28(1):1-7.

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Lung transplantation has been accepted widely as therapy for end-stage pulmonary lymphangioleiomyomatosis (LAM); however, single-center and national experience is limited due to the rarity of LAM.

We report the recent European experience of lung transplantation for LAM. A self-administered questionnaire was distributed to 30 European lung transplant centers to evaluate patients who underwent primary lung transplantation for LAM (1997 to 2007).

Seventy percent of centers responded to the questionnaire. A total of 61 lung transplants were undertaken in women only, with mean age at transplant 41.3 years (SD 5.1). Centers performed a median of 2 (0 to 9) transplant operations. Severe pleural adhesions were the most common intra-operative complication. Early deaths (N = 6) were due to primary graft or multiple-organ failure or sepsis. Twelve recipients were diagnosed with bronchiolitis obliterans syndrome at a median of 20 months (range 10 to 86 months) post-transplant. LAM-related complications included renal angiomyolipoma and pneumothorax in the native lung. Recurrence of LAM occurred in 4 recipients. As of December 2007, actuarial Kaplan–Meier survival was 79% at 1 year and 73% at 3 years post-transplant.

Post-transplant outcome for pulmonary LAM in the recent era appears to have improved compared with the previous era. LAM-related complications remain common, but recurrence of LAM in the allograft is rare.


25 citations in Web of Science®
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Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Pneumology
04 Faculty of Medicine > University Hospital Zurich > Clinic and Policlinic for Internal Medicine
Dewey Decimal Classification:610 Medicine & health
Deposited On:30 Jan 2009 11:52
Last Modified:27 Nov 2013 17:34
Publisher DOI:10.1016/j.healun.2008.09.014
PubMed ID:19134523

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