Sirolimus for Angiomyolipoma in Tuberous Sclerosis Complex or Lymphangioleiomyomatosis | Zendy

John J. Bissler | Zendy; Francis X. McCormack | Zendy; Lisa R. Young | Zendy; Jean Elwing | Zendy; Gail Chuck | Zendy; Jennifer Leonard | Zendy; Vincent J. Schmithorst | Zendy; Tal Laor | Zendy; Alan S. Brody | Zendy; Judy A. Bean | Zendy; Shelia Salisbury | Zendy; David Neal Franz | Zendy

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Sirolimus for Angiomyolipoma in Tuberous Sclerosis Complex or Lymphangioleiomyomatosis

Author(s) -

John J. Bissler,

Francis X. McCormack,

Lisa R. Young,

Jean Elwing,

Gail Chuck,

Jennifer Leonard,

Vincent J. Schmithorst,

Tal Laor,

Alan S. Brody,

Judy A. Bean,

Shelia Salisbury,

David Neal Franz

Publication year - 2008

Publication title -

new england journal of medicine

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 19.889

H-Index - 1030

eISSN - 1533-4406

pISSN - 0028-4793

DOI - 10.1056/nejmoa063564

Subject(s) - lymphangioleiomyomatosis , tuberous sclerosis , medicine , sirolimus , angiomyolipoma , magnetic resonance imaging , gastroenterology , vital capacity , urology , pathology , lung , radiology , lung function , kidney , diffusing capacity

Angiomyolipomas in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis are associated with mutations in tuberous sclerosis genes resulting in constitutive activation of the mammalian target of rapamycin (mTOR). The drug sirolimus suppresses mTOR signaling.

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