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Adrenal function during long-term ACTH therapy for patients with developmental and epileptic encephalopathy

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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
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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