Treatment effect of mTOR-inhibition on tissue composition of renal angiomyolipomas in tuberous sclerosis complex (TSC)

Tuberous sclerosis complex (TSC)-associated renal angiomyolipoma (AML) have a high lifetime risk of acute bleeding. MTOR-inhibitors are a promising novel treatment for TSC-AML, however adequate response to therapy can be difficult to assess. Early changes in MRI signal may serve as a novel early ind...

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Veröffentlicht in:PloS one 2017-12, Vol.12 (12), p.e0189132-e0189132
Hauptverfasser: Brakemeier, Susanne, Vogt, Lars, Adams, Lisa, Zukunft, Bianca, Diederichs, Gerd, Hamm, Bernd, Budde, Klemens, Makowski, Marcus R
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Sprache:eng
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Zusammenfassung:Tuberous sclerosis complex (TSC)-associated renal angiomyolipoma (AML) have a high lifetime risk of acute bleeding. MTOR-inhibitors are a promising novel treatment for TSC-AML, however adequate response to therapy can be difficult to assess. Early changes in MRI signal may serve as a novel early indicator for a satisfactory response to mTOR-inhibitor therapy of AML. Thirty-eight patients with the definite diagnosis of tuberous sclerosis receiving everolimus therapy and n = 19 patients without specific therapy were included. 1.5 Tesla MRI was performed including sequences with a selective fat suppression. Patients were investigated prior to the initiation of therapy (baseline) and after
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0189132