| Title: | Adrenal function during long-term ACTH therapy for patients with developmental and epileptic encephalopathy |
| Authors: | Ueda, Yuki Browse this author |
| Fujishige, Shuta Browse this author |
| Goto, Takeru Browse this author |
| Kimura, Shuhei Browse this author |
| Namatame, Noriko Browse this author |
| Narugami, Masashi Browse this author |
| Nakakubo, Sachiko Browse this author |
| Nakajima, Midori Browse this author |
| Egawa, Kiyoshi Browse this author |
| Kaneko, Naoya Browse this author |
| Nakayama, Kanako Browse this author |
| Hishimura, Nozomi Browse this author |
| Yamaguchi, Takeshi Browse this author →KAKEN DB |
| Nakamura, Akie Browse this author |
| Shiraishi, Hideaki Browse this author →KAKEN DB |
| Keywords: | ACTH therapy |
| adrenal insufficiency |
| developmental and epileptic encephalopathy |
| long-term treatment |
| Issue Date: | Mar-2022 |
| Publisher: | John Wiley & Sons |
| Journal Title: | Epilepsia Open |
| Volume: | 7 |
| Start Page: | 194 |
| End Page: | 200 |
| Publisher DOI: | 10.1002/epi4.12566 |
| Abstract: | Some patients with developmental and epileptic encephalopathy (DEE) respond to adrenocorticotropic hormone (ACTH) therapy but relapse soon after. While long-term ACTH therapy (LT-ACTH) has been attempted for these patients, no previous studies have carefully assessed adrenal function during LT-ACTH. We evaluated the effectiveness of LT-ACTH, as well as adverse effects (AE), including their adrenal function in three DEE patients. Patients underwent a corticotropin-releasing hormone (CRH) stimulation test during LT-ACTH, and those with peak serum cortisol below 15 mu g/dL were considered to be at high risk of adrenal insufficiency (AI). Two of three responded, and their life-threatening seizures with postgeneralized electroencephalogram (EEG) suppression decreased. Although no individuals had serious AE, CRH stimulation test revealed relatively weak responses, without reaching normal cortisol peak level (18 mu g/dL). Hydrocortisone replacement during stress was prepared in a case with lower cortisol peak than our cutoff level. LT-ACTH could be a promising treatment option for cases of DEE that relapse soon after effective ACTH treatment. The longer duration and larger cumulative dosage in LT-ACTH than in conventional ACTH could increase the relative risk of AI. Careful evaluation with pediatric endocrinologists, including hormonal stimulation tests, might be useful for continuing this treatment safely. |
| Type: | article |
| URI: | http://hdl.handle.net/2115/84502 |
| Appears in Collections: | 北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)
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