Clinical lung and heart/lung transplantationLung Transplantation in the Management of Patients With Lymphangioleiomyomatosis: Baseline Data From the NHLBI LAM Registry
Section snippets
Methods
Patients with LAM entered the Registry between August 1998 and October 2001, through six participating centers. A description of the Registry is detailed in a previous publication6; aspects pertinent to the present report are summarized herein.
Comparison of baseline Group D (no transplant evaluation) and Group B (transplant during the Registry period) patients
Baseline data from the 161 Group D patients were compared with baseline data of Group B patients who had advanced disease and would subsequently undergo transplant during the course of the study. (The 48 patients who were wait-listed during the study or underwent transplant evaluation are not included in this initial analysis; see later section for analysis including this group of patients). When compared with Group D patients, the Group B patients tended to be younger (41.5 ± 7.9 vs 44.5 ±
Discussion
The LAM Registry brings together data on a large number of LAM patients with a broad spectrum of disease severity. We have done several analyses to compare pulmonary function and quality-of-life data of patients with early to moderate disease (Group D) with those of advanced or end-stage disease. In our analysis, patients entering the study who were subsequently transplanted were considered to have end-stage disease (Group B). In addition, we considered patients who were wait-listed or
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Cited by (40)
Lung Transplantation: Recipient Selection
2021, Encyclopedia of Respiratory Medicine, Second EditionLymphangioleiomyomatosis
2021, Encyclopedia of Respiratory Medicine, Second EditionPregnancy unmasking symptoms of undiagnosed lymphangioleiomyomatosis: Case report and review of literature
2019, Respiratory Medicine Case ReportsCitation Excerpt :There are no absolute contraindications for transplant and indications are yet to be established. It has been shown that lung transplantation improves lung function and quality of life [39]. Some anecdotal cases have been reported of recurrence of LAM after bilateral lung transplant although this recurrence did not seem to affect survival [40].
Lung Transplantation in Interstitial Lung Disease
2018, Interstitial Lung DiseaseMayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis
2015, Respiratory MedicineCitation Excerpt :Despite the limited numbers, the LT survival rate for LAM was comparable to that of LT for other end-stage lung diseases [9]. Importantly, it appears that LT is also associated with significantly improved quality of life in patients with advanced disease [10]. Nonetheless, outcomes of LT in the modern era may be influenced by the peri-transplant utilization of sirolimus.
Imaging of cystic lung disease
2015, Seminars in RoentgenologyCitation Excerpt :This has included hormonal manipulation and more recently immunomodulation with limited success.3 In patients with advanced disease, transplantation may be the only treatment option.12 However, there are multiple case reports of LAM recurrence in transplanted lungs.13
Supported by NHLBI grant U01 HL58440 and the NHLBI division of Intramural Research.