Low-dose ACTH therapy for West syndrome

Abstract

Low-dose synthetic ACTH therapy is as effective for the treatment of WS as the higher doses used in previous studies. The dosage of synthetic ACTH used in the treatment of WS can be decreased as much as possible to avoid serious adverse effects.

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

“…This agrees with the results of a Japanese multi-institutional study of 138 patients with West syndrome. 2 This also showed that the severity of adverse effects correlated with the ACTH dosage, similar to our finding. Some studies, including this, showed death associated with higher dosages of ACTH.…”

“…*There were significant differences statistically between the cryptogenic group and the symptomatic group (P ϭ .0131), by2 test. 488 Hamano et al The Journal of Pediatrics • April 2006…”

“…The classic method is to use 0.25 mg (10 IU) for patients younger than 1 year of age, given daily for 2 weeks, and then tapered off over the next 6 weeks. 2 Recently, Japanese pediatric neurologists have tended to use a lower dose of synthetic ACTH than that of the traditional method, since Awaya et al 3 advocated doing so initially in 1984. Ito et al 2 documented that lower-dose ACTH therapy was as effective to control spasms as the traditional method was.…”

Hypothalamic-pituitary-adrenal axis hypofunction after adrenocorticotropic hormone therapy

“…The CRH test was started between 08:00 and 09:00 a.m. on the third day or later after the last ACTH-Z injection (at the same time as "post-ACTH"). After CRH injection, blood samples were collected serially at 0, (15), 30, 60, 90, and 120 minutes for measurements of serum cortisol and plasma ACTH levels . In the CRH test, a serum cortisol increase to 20 µg/dL or higher is the criterion of normal HPA axis function [21][22][23].…”

“…Nonetheless, contrary to its proposed benefits, some adverse effects occurred during or after ACTH therapy. Hence, the dose of ACTH-Z was gradually reduced from 0.025 to 0.005-0.0125 mg/kg/dose once daily for 2 weeks and tapered in a phased manner [5][6][7][8][9][10][11][12][13][14][15]. Although the dose reduction minimized the adverse effects considerably, precaution is still needed for those adverse effects.…”