Abstract
Prednisolone and adrenocorticotropic hormone (ACTH) are “hormone” therapies for infantile spasms. There is limited data on the occurrence of decreased adrenal reserve or signs of clinical adrenal insufficiency after hormone therapy. This is a retrospective medical record review of patients referred to our Infantile Spasms Program. Our standardized infantile spasms management guideline began in September 2012 and initially included a post-hormone laboratory assessment of adrenal function. Medical records were assessed for hormone treatments, adrenal function testing, and signs of adrenal insufficiency. Forty-two patients who received one or both hormone therapies met inclusion criteria. A post-hormone laboratory assessment of adrenal function was done in 14 patients. Of these 14 patients, 2 had an abnormal laboratory assessment of adrenal function, both by adrenal stimulation testing – one after ACTH and one after prednisolone. One patient received hydrocortisone replacement and the other received stress dose hydrocortisone as needed; neither patient developed signs of adrenal insufficiency. Another patient treated with both types of hormone therapy in tandem, who did not have a post-hormone laboratory assessment, developed signs of mild adrenal insufficiency and required replacement hydrocortisone. Our study suggests that adrenal suppression can occur after modern hormone therapy regimens. We found two patients with abnormal adrenal function testing after hormone therapy and another patient with signs adrenal insufficiency. Given the seriousness of adrenal crisis, caregiver education on the signs of adrenal insufficiency is critical. Greater vigilance may be indicated in patients receiving both types of hormone therapy in tandem. Although a routine post-hormone laboratory assessment of adrenal function may not be feasible in all patients, replacement or stress dose hydrocortisone is necessary for all patients with suspected adrenal insufficiency.
Highlights
Infantile spasms are seizures that most often begin in the first year of life
Natural gel Adrenocorticotropic hormone (ACTH) was given at a dose of 75 IU/m2 twice daily for 2 weeks followed by a taper over 15 days [30 IU/m2/ day for 3 days, 15 IU/m2/day for 3 days, 10 IU/m2/day for 3 days, and 10 IU/m2/day every other day for three doses [5]] and prednisolone was given at a dose of 40–60 mg/day in three to four divided doses for 2 weeks followed by a 15-day taper [10 mg three times daily for 5 days, 10 mg twice daily for 5 days, and 10 mg daily for 5 days [8]]
Our study suggests that adrenal suppression can occur after treatment of modern hormone therapy regimens
Summary
Infantile spasms are seizures that most often begin in the first year of life. Adrenocorticotropic hormone (ACTH) and oral corticosteroids (most commonly prednisolone) are types of “hormone” therapies that are commonly used first-line treatments for patients with infantile spasms [3]. Some infantile spasms patients who do not respond to ACTH will respond to oral corticosteroids, and vice versa [4]. In the United States, the duration of hormone therapy with ACTH or prednisolone is about 1 month [3, 5,6,7]. But clinicians in the United States often use “high-dose” hormone therapy [3]. High-dose regimens typically begin with 150 IU/m2/day for natural gel ACTH [5] and 40–60 mg/day for oral prednisolone [8]
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