Researchers presented the challenges of pediatric adrenal insufficiency and reviewed existing therapeutic solutions, as published in Therapeutic and Clinical Risk Management. The adrenal glands are physiologically important because they are responsible for producing aldosterone, cortisol, and androgen.
These hormones are the end result of pathways that start from the hypothalamic-pituitary axis. Primary adrenal insufficiency is caused by impaired steroid synthesis and adrenal destruction; acquired adrenal insufficiency is otherwise known as Addison’s disease.
A number of diseases, including genetic disorders, can result in adrenal pathology. Lysosomal acid lipase deficiency (LAL-D), for example, can cause esterified lipids to accumulate in the adrenal glands, leading to their calcification.
“In infantile form, that is Wolman disease; hepatosplenomegaly with hepatic fibrosis and malabsorption lead to death in the first year of life, if not treated with enzyme replacement therapy such as sebelipase alfa,” Nisticò and colleagues wrote.
Read more about LAL-D overview
Adrenal insufficiency often presents with nonspecific symptoms and can sometimes be mistaken for life-threatening conditions, such as sepsis or an acute abdomen. Glucocorticoid insufficiency can manifest as hyponatremia, hyperkalemia, tachycardia, acidosis, and hypotension.
The typical treatment for adrenal insufficiency is long-term glucocorticoid replacement therapy, the first-line drug being oral hydrocortisone, which has shown to have fewer adverse effects than prednisolone or dexamethasone. The best parameters for measuring the effectiveness of glucocorticoid therapy are growth, weight, and general well-being, the authors said.
In cases of an adrenal crisis, emergency treatment must be administered immediately, since it is a life-threatening condition. Standard treatment includes hydrocortisone delivered via intravenous bonus, followed by continuous infusion until the patient becomes stable.
“Education for caregivers and patients (if adolescent) is crucial to prevent adrenal crisis. They should recognize signs and symptoms of adrenal crisis and should receive a steroid emergency card with the sick day rules,” Nisticò et al concluded. In adrenal insufficiency, like so many other diseases, education is vital for all stakeholders involved.
Nisticò D, Bossini B, Benvenuto S, Pellegrin MC, Tornese G. Pediatric adrenal insufficiency: challenges and solutions. Ther Clin Risk Manag. Published online January 11, 2022. doi:10.2147/TCRM.S294065