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

Ueda, Yuki; Fujishige, Shuta; Goto, Takeru; Kimura, Shuhei; Namatame, Noriko; Narugami, Masashi; Nakakubo, Sachiko; Nakajima, Midori; Egawa, Kiyoshi; Kaneko, Naoya; Nakayama, Kanako; Hishimura, Nozomi; Yamaguchi, Takeshi; Nakamura, Akie; Shiraishi, Hideaki

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

Ueda, Yuki; Fujishige, Shuta; Goto, Takeru et al.

Epilepsia Open, 2022, 7, 194-200

Permalink : https://hdl.handle.net/2115/84502

このアイテムのアクセス数:399件（2026-02-09 11:49 集計）

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https://doi.org/10.1002/epi4.12566			1

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アクセス権	metadata only access
URI	https://hdl.handle.net/2115/84502
タイトル	Adrenal function during long-term ACTH therapy for patients with developmental and epileptic encephalopathy
著者	Ueda, Yuki ; Fujishige, Shuta ; Goto, Takeru ; Kimura, Shuhei ; Namatame, Noriko ; Narugami, Masashi ; Nakakubo, Sachiko ; Nakajima, Midori ; Egawa, Kiyoshi ; Kaneko, Naoya ; Nakayama, Kanako ; Hishimura, Nozomi ; Yamaguchi, Takeshi ; Nakamura, Akie ; Shiraishi, Hideaki
著者名 Ueda, Yuki
著者名 Fujishige, Shuta
著者名 Goto, Takeru
著者名 Kimura, Shuhei
著者名 Namatame, Noriko
著者名 Narugami, Masashi
著者名 Nakakubo, Sachiko
著者名 Nakajima, Midori
著者名 Egawa, Kiyoshi
著者名 Kaneko, Naoya
著者名 Nakayama, Kanako
著者名 Hishimura, Nozomi
e-Rad_Researcher 80894972 著者名 Yamaguchi, Takeshi
著者名 Nakamura, Akie
e-Rad_Researcher 80374411 著者名 Shiraishi, Hideaki
言語	英語
キーワード	ACTH therapy
	adrenal insufficiency
	developmental and epileptic encephalopathy
	long-term treatment
発行日	2022-03
出版者	John Wiley & Sons
収録物名	Epilepsia Open
巻(号)	7
ページ	194-200
抄録	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.
資源タイプ	学術雑誌論文
出版タイプ	Version of Record
関連情報 (isIdenticalTo)	出版社版DOI https://doi.org/10.1002/epi4.12566