| Title: | Resolution of multifocal micronodular pneumocyte hyperplasia with everolimus in a patient with tuberous sclerosis complex |
| Authors: | Shoji, Tetsuaki Browse this author |
| Niida, Yo Browse this author |
| Osawa, Takahiro Browse this author |
| Matsumoto, Ryuji Browse this author |
| Sakurai, Kotaro Browse this author |
| Suzuki, Masaru Browse this author |
| Matsuno, Yoshihiro Browse this author |
| Konno, Satoshi Browse this author →KAKEN DB |
| Keywords: | Tuberous sclerosis complex |
| Multifocal micronodular pneumocyte |
| hyperplasia |
| Everolimus |
| Surfactant Protein-A (SP-A) |
| Surfactant protein-B (SP-B) |
| Issue Date: | 6-Oct-2021 |
| Publisher: | Elsevier |
| Journal Title: | Respiratory Medicine Case Reports |
| Volume: | 34 |
| Start Page: | 101526 |
| Publisher DOI: | 10.1016/j.rmcr.2021.101526 |
| Abstract: | A woman with a diagnosis of tuberous sclerosis complex (TSC) presented with TSC2 gene mutation and various manifestations, including epilepsy, renal angiomyolipomas (AML), and pathologically confirmed multifocal micronodular pneumocyte hyperplasia (MMPH). With oral administration of evemlimus, a mammalian target of rapamycin (mTOR) inhibitor, MMPH and AML were markedly reduced. Further, after starting treatment with everolimus, serum levels of surfactant protein (SP)-A and SP-D, which reflect type II pneumocyte hyperplasia, decreased to the normal range. At the time of writing of this manuscript, 6 years after starting evemlimus, MMPH lesions did not relapse and SP-A/D remained the low levels. This is the first case of evemlimus efficacy shown for histologically confirmed MMPH in genetically determined TSC patient, with time course of serum SP-A and SP-D. |
| Type: | article |
| URI: | http://hdl.handle.net/2115/83359 |
| Appears in Collections: | 医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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