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Iatrogenic diabetes mellitus during ACTH therapy in an infant with West syndrome
Valeria Calcaterra1, Andrea Bottazzi2, Chrissoula Tzialla3
1Department of Pediatrics, University of Pavia and IRCCS Policlinico San Matteo Foundation, P.le Golgi n.2, 27100, Pavia, Italy. v.calcaterra@smatteo.pv.it.
Insights
Adrenocorticotropic hormone (ACTH) therapy for West syndrome can cause iatrogenic diabetes mellitus. Careful monitoring of blood glucose levels is essential during ACTH treatment for infants with this rare epileptic disorder.
Area of Science:
- Pediatric Neurology
- Endocrinology
- Epileptology
Background:
- West syndrome is a rare infantile epileptic disorder characterized by spasms, hypsarrhythmia, and psychomotor impairment.
- Adrenocorticotropic hormone (ACTH) is the standard first-line treatment, despite potential adverse effects.
Observation:
- A rare case of iatrogenic diabetes mellitus occurred during ACTH therapy in an infant with symptomatic West syndrome.
- The infant exhibited Cushingoid features, hirsutism, biochemical diabetes, adrenal hyperplasia, weight gain, electrolyte imbalances, and infections.
Findings:
- Excessive glucocorticoid production secondary to ACTH therapy was identified as the cause of diabetes.
- The patient showed signs of hypercortisolism without mineralocorticoid excess.
Implications:
- This case highlights a significant, albeit rare, adverse effect of ACTH therapy in West syndrome.
- Close monitoring of glycemic levels is crucial throughout ACTH treatment to detect and manage potential diabetes development.
Abstract:
West syndrome is a rare epileptic disease of infancy, typified by an association of characteristic spasms, hypsarrhythmia on electroencephalography and severe psychomotor retardation or deterioration. Adrenocorticotropic hormone (ACTH) is the current first-line therapy for West syndrome despite the fact that ACTH therapy is associated with various adverse effects. We describe a rare case of iatrogenic diabetes mellitus during ACTH therapy in a patient with symptomatic West syndrome. The infant had cushingoid facies, hirsutism and biochemical evidence of diabetes due to excessive glucocorticoid production with hyperplasia of both adrenal glands at ultrasound examination, without mineralocorticoid excess; in addition, he presented also short-term weight gain, marked electrolyte disturbances, hypokalemic alkalosis and infections. When ACTH is used to treat patients with West syndrome, it is necessary to follow glycemic levels until to the end of therapy.
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