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

Hamano, Shin‐ichiro; Yamashita, Shintaro; Tanaka, Manabu; Yoshinari, Satoshi; Minamitani, Motoyuki; Eto, Yoshikatsu

doi:10.1016/j.jpeds.2005.11.041

Cited by 46 publications

(38 citation statements)

References 5 publications

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“…He was admitted to the hospital for adrenocorticotropic hormone therapy to control his epileptic attacks at the age of 2 years and 11 months. 28 At this time, his height was 87.8 cm (10th percentile), his weight was 11 kg (3rd percentile) and his head circumference was 48.1 cm (o25th percentile). Dysmorphic features including, a broad nasal root, a high arched palate, low-set ears, rough hair, telecanthus, microretrognathia (Figure 3), and flat feet were noted.…”

Section: Patient Reportmentioning

confidence: 95%

A functional analysis of GABARAP on 17p13.1 by knockdown zebrafish

et al. 2010

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Array-based comparative genomic hybridization identified a 2.3-Mb microdeletion of 17p13.2p13.1 in a boy presenting with moderate mental retardation, intractable epilepsy and dysmorphic features. This deletion region was overlapped with the previously proposed shortest region overlapped for microdeletion of 17p13.1 in patients with mental retardation, microcephaly, microretrognathia and abnormal magnetic resonance imaging (MRI) findings of cerebral white matter, in which at least 17 known genes are included. Among them, DLG4/PSD95, GPS2, GABARAP and KCTD11 have a function in neuronal development. Because of the functional importance, we paid attention to DLG4/PSD95 and GABARAP, and analyzed zebrafish in which the zebrafish homolog of human DLG4/PSD95 and GABARAP was knocked down and found that gabarap knockdown resulted in small head and hypoplastic mandible. This finding would be similar to the common findings of the patients with 17p13.1 deletions. Although there were no pathogenic mutations in DLG4/PSD95 or GABARAP in a cohort study with 142 patients with idiopathic developmental delay with/without epilepsy, further studies would be required for genes included in this region.

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Section: Patient Reportmentioning

confidence: 95%

A functional analysis of GABARAP on 17p13.1 by knockdown zebrafish

et al. 2010

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“…Our facility has access only to tetracosactide -a long-acting corticotrophin preparation administered intramuscularly. [12] Patients received 20 units (0.25 mg), which was appropriate for the age and weight of patients <1 year of age, daily for the first 3 days, and then on alternate days for 5 doses. A total of 14 days of treatment accounts for a single course.…”

Section: Final Outcomesmentioning

confidence: 99%

The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

et al. 2017

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“…Some adverse effects after ACTH therapy have been reported previously [8][9][10][11][12], especially when using the synthetic ACTH; ACTH-Z, which is the only formulation available for ACTH therapy in Japan and has much higher potential of adverse effects than natural ACTH. Hence, there is a need to study these effects in detail using synthetic Five of six cases (cases 1, 2, 3, 4, and 6) had peak serum cortisol and plasma ACTH levels at 15 or 30 minutes after a CRH injection, whereas case 5 had unique increment patterns in both serum cortisol and plasma ACTH levels.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

“…The adverse effects of ACTH therapy include infection, hypertension, bradycardia, hypertrophic cardiomyopathy, brain shrinkage, subdural effusion, irritability, hypokalemia, adrenocortical dysfunction, and suppression of the hypothalamic-pituitary-adrenal (HPA) axis or HPA axis insufficiency (HPAI) [6,7,12,14]. Although HPAI is notably one of the most significant adverse effects, only four previous reports have demonstrated the adverse effects of ACTH therapy on the HPA axis [16][17][18][19].…”

Section: Introductionmentioning

confidence: 99%

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Hypothalamic-pituitary-adrenal axis hypofunction after adrenocorticotropic hormone therapy

Abe

Sassa

Kikuchi

et al. 2018

E&S

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Purpose:To investigate the adver se effects of adrenocor ticotropic hor mone (ACTH) ther apy on function of the hypothalamic-pituitary-adrenal (HPA) axis and the necessity of subsequent adrenocortical hormone replacement therapy (HRT). Methods: We conducted a retrospective review of patients with epilepsy who received ACTH therapy and underwent assessment of HPA axis function. Results: Six cases analyzed for hor mones, including a symptomatic patient with HPA axis insufficiency (HPAI), were studied. Serum cortisol concentrations in the morning were at HPAI levels (<5.0 µg/dL) in three cases, at suspected HPAI levels (>5.0 and <13 µg/dL) in two cases, and normal in one case. However, in the corticotrophin-releasing hormone (CRH) test, while both serum cortisol and plasma ACTH levels were reactive in five cases, only one case exhibited lazy patterns in the time courses of serum cortisol and plasma ACTH levels. Consequently, all six patients who received ACTH therapy had some degree of HPA axis hypofunction for approximately 2-3 months after ACTH therapy and were treated with HRT. Conclusion: Patients who r eceive ACTH ther apy may be at r isk of HPA axis hypofunction for a certain period and HRT may be recommended until their serum cortisol levels return to basal levels.

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

Cited by 46 publications

References 5 publications

A functional analysis of GABARAP on 17p13.1 by knockdown zebrafish

A functional analysis of GABARAP on 17p13.1 by knockdown zebrafish

The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

Hypothalamic-pituitary-adrenal axis hypofunction after adrenocorticotropic hormone therapy

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