The US Experience with Lung Transplantation for Pulmonary Lymphangioleiomyomatosis

Kpodonu, Jacques; Massad, Malek G.; Chaer, Rabih A.; Caines, Amitra E.; Evans, Alexander; Snow, Norman; Geha, Alexander S.

doi:10.1016/j.healun.2004.09.013

Cited by 99 publications

(63 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…LAM compares favourably to patients transplanted for other indications [38,88,103,104]. In a recent survey the actuarial survival of lung transplantation for LAM was 86% at 1 yr, 76% at 3 yrs, and 65% at 5 yrs [104].…”

Section: Treatment and Follow-up Of Angiomyolipomamentioning

confidence: 99%

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

Johnson¹,

Cordier²,

Lazor³

et al. 2009

European Respiratory Journal

475

561

View full text Add to dashboard Cite

Section: Treatment and Follow-up Of Angiomyolipomamentioning

confidence: 99%

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

Johnson¹,

Cordier²,

Lazor³

et al. 2009

European Respiratory Journal

475

561

View full text Add to dashboard Cite

“…It is evident from the literature that LAM could recur as early as within two years after the lung transplantation. Although the recurrence of LAM is rare, the posttransplant survival of these patients when compared to all other indications of transplant is better [11] . But the number of patients that have undergone transplantation for LAM as the primary indication is very small and predications regarding this disease and survival posttransplant should be tempered.…”

Section: Discussionmentioning

confidence: 97%

“…The estimated five year post lung transplant survival among LAM patients is between 60%-70%. The recurrence is rare, and the rate between 3.7%-7% has been reported in the largest European and United States studies [10,11] . It is likely that recurrence rate could be higher in long term survivors as early recurrence may be asymptomatic.…”

Section: Discussionmentioning

confidence: 98%

“…The recurrence of LAM following lung transplantation is rare and only nine cases have been reported in the literature [1,[5][6][7][8][9][10] . The largest LAM database from Europe demonstrated only single digit recurrence rate of LAM after transplantation (6%-7%) [10,11] . Due to the rarity of LAM and low rate of recurrence following lung transplantation, there is a paucity in our current knowledge regarding the treatment and rate of its progression.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation

Zaki

Aryan

Mehta

et al. 2016

WJT

View full text Add to dashboard Cite

Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive lethal lung disease primary afflicting young women. LAM is characterized by proliferation of abnormal smooth muscle cells that target the lungs, causing cystic destruction and eventual respiratory failure leading to death. Recent ten year mortality due to end stage LAM has been reported to be approximately 10%-20%, but may vary. The decline in lung function in LAM is gradual, occurring at a rate of about 3% to 15% per year but can vary from patient to patient. But recently therapy with mammalian target of rapamycin (mTOR) inhibitors such as sirolimus has shown promising results in the stabilization of lung function and reduction of chylous effusions in LAM. Lung transplantation is a viable option for patients who continue to have decline in lung function despite mTOR therapy. Unique issues that may occur post-transplant in a recipient with LAM include development of chylous effusion and a risk of recurrence. We describe a case of LAM recurrence in a bilateral lung transplant recipient who developed histological findings of LAM nine years after transplantation.

show abstract

“…(4) Lung transplantation and combined heart-lung transplantation remain as options for advanced cases. (5) More than 25 years ago, it was demonstrated that hormones are involved in the pathogenesis of LAM. Brentani et al reported the presence of estrogen, progesterone and glucocorticoids receptors in the lung tissue of patients with LAM.…”

mentioning

confidence: 99%

Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

et al. 2011

View full text Add to dashboard Cite

In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV 1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin. ResumoNas células musculares lisas atípicas características da linfangioleiomiomatose (LAM) encontram-se receptores de estrogênio e progesterona, de modo que o tratamento anti-hormonal pode ser considerado uma opção, mas ainda com resultados controversos. O objetivo deste trabalho foi avaliar retrospectivamente parâmetros hormonais e espirométricos em nove mulheres com LAM após o tratamento com goserelina por um ano. Houve um aumento médio de 80 mL e 130 mL, respectivamente, em VEF 1 e CVF, assim como bloqueio hormonal efetivo. Ainda não se pode excluir um potencial efeito favorável da utilização de análogos de hormônio liberador de gonadotrofina em pacientes com LAM, reforçando a necessidade de ensaios randomizados.

show abstract

The US Experience with Lung Transplantation for Pulmonary Lymphangioleiomyomatosis

Cited by 99 publications

References 11 publications

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation

Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

Contact Info

Product

Resources

About