Literature DB >> 16647410

Therapeutic efficacy and adverse effects of adrenocorticotropic hormone therapy in west syndrome: differences in dosage of adrenocorticotropic hormone, onset of age, and cause.

Shin-ichiro Hamano1, Shintaro Yamashita, Manabu Tanaka, Satoshi Yoshinari, Motoyuki Minamitani, Yoshikatsu Eto.   

Abstract

OBJECTIVES: To determine the dosage and factors influencing efficacy of adrenocorticotropic hormone (ACTH) for West syndrome. STUDY
DESIGN: A retrospective study of 135 patients receiving ACTH therapy with a synthetic analogue for initial effect, seizure outcome 1 year after therapy, and adverse effects. Efficacy and adverse effects were compared among the groups divided by clinical factors: dosage, treatment lag, onset age, and cause.
RESULTS: One hundred thirteen patients had seizure control with ACTH. For more than 1 year after ACTH, 59 remained seizure free. Adverse effects were observed in 57, and ACTH therapy was discontinued in 23. The lowest dosage group (0.0125 mg/kg/d) had fewer episodes of discontinuation (P<.05), whereas differences in efficacy between different dosages were insignificant. None of the clinical factors correlated with initial effect. The earlier-onset group (<4 months) showed unfavorable seizure outcome 1 year after ACTH (P<.01). The cryptogenic patients showed better seizure outcome (P<.05) compared with the symptomatic.
CONCLUSION: Synthetic ACTH therapy at a lower dosage is as effective as natural ACTH therapy at a higher dosage. Considering the adverse effects and the benefits for seizure control, the ACTH dosage of 0.0125 mg/kg/d (synthetic analogue) is more favorable than larger dosage.

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Year:  2006        PMID: 16647410     DOI: 10.1016/j.jpeds.2005.11.041

Source DB:  PubMed          Journal:  J Pediatr        ISSN: 0022-3476            Impact factor:   4.406


  5 in total

Review 1.  West Syndrome: A Review and Guide for Paediatricians.

Authors:  Renato D'Alonzo; Donato Rigante; Elisabetta Mencaroni; Susanna Esposito
Journal:  Clin Drug Investig       Date:  2018-02       Impact factor: 2.859

Review 2.  Interneuron, interrupted: molecular pathogenesis of ARX mutations and X-linked infantile spasms.

Authors:  Pedro R Olivetti; Jeffrey L Noebels
Journal:  Curr Opin Neurobiol       Date:  2012-05-05       Impact factor: 6.627

3.  ACTH therapy for West syndrome with severe hemophilia A.

Authors:  Kazuo Kubota; Yoriko Kinomura; Takahiro Yamamoto; Michio Ozeki; Minako Kawamoto; Norio Kawamoto; Toshiyuki Fukao
Journal:  Epilepsy Behav Case Rep       Date:  2016-05-10

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

Authors:  Yuki Ueda; Shuta Fujishige; Takeru Goto; Shuhei Kimura; Noriko Namatame; Masashi Narugami; Sachiko Nakakubo; Midori Nakajima; Kiyoshi Egawa; Naoya Kaneko; Kanako Nakayama; Nozomi Hishimura; Takeshi Yamaguchi; Akie Nakamura; Hideaki Shiraishi
Journal:  Epilepsia Open       Date:  2021-12-14

5.  Efficacy of long-term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series.

Authors:  Shimpei Baba; Tohru Okanishi; Yoichiro Homma; Takeshi Yoshida; Tomohide Goto; Tatsuya Fukasawa; Satoru Kobayashi; Atsushi Kamei; Yuji Fujii; Naomi Hino-Fukuyo; Keitaro Yamada; Atsuro Daida; Hisashi Kawawaki; Hideki Hoshino; Hitoshi Sejima; Yusuke Ishida; Tetsuya Okazaki; Takehiko Inui; Sotaro Kanai; Hirotaka Motoi; Shinji Itamura; Mitsuyo Nishimura; Hideo Enoki; Ayataka Fujimoto
Journal:  Epilepsia Open       Date:  2021-05-28
  5 in total

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