Pediatric Adrenal Insufficiency: Diagnosis, Management, and New Therapies

Abstract

Adrenal insufficiency may result from a wide variety of congenital or acquired disorders of hypothalamus, pituitary, or adrenal cortex. Destruction or dysfunction of the adrenal cortex is the cause of primary adrenal insufficiency, while secondary adrenal insufficiency is a result of pituitary or hypothalamic disease. Timely diagnosis and clinical management of adrenal insufficiency are critical to prevent morbidity and mortality. This review summarizes the etiologies, presentation, and diagnosis of adrenal insufficiency utilizing different dynamic hormone testing and describes current treatment recommendations and new therapies.

A Biopredictive In Vitro Approach for Assessing Compatibility of a Novel Pediatric Hydrocortisone Drug Product within Common Pediatric Dosing Vehicles

“…Occasionally, it can be caused by autoimmune disease (Addison's disease) or pituitary conditions. AI is a life-long and life-threatening condition and is associated with a multitude of symptoms that are often unspecific and thus make diagnosis difficult (1).…”

“…To avoid risks of hypoglycemia and adrenal crisis associated with undertreatment, and to avoid growth suppression and reduced final height potential associated with steroid overdosing, appropriate replacement doses of glucocorticoids are important to determine in both primary and secondary AI in children (3). Hydrocortisone (the synthetic version of cortisol) represents the glucocorticoid of choice for treating pediatric AI patients (1,4). It is administered orally in two to four divided doses per day, where the single doses should be adjusted to the individual need based on body surface area (1,4).…”

“…Hydrocortisone (the synthetic version of cortisol) represents the glucocorticoid of choice for treating pediatric AI patients (1,4). It is administered orally in two to four divided doses per day, where the single doses should be adjusted to the individual need based on body surface area (1,4). Since AI can present at birth and usually requires life-long and patient-tailored oral glucocorticoid medication, oral dosage forms that ensure acceptability in children of different age groups are an important prerequisite for safe, reliable and effective glucocorticoid substitution.…”

“…Primary AI is caused by destruction or dysfunction of the adrenal cortex and presents with impaired cortisol and aldosterone production. Secondary AI is caused by a lack of pituitary adrenocorticotropic hormone (ACTH) which prevents the body from producing enough cortisol ( 1 ).…”

Current Insights Into Adrenal Insufficiency in the Newborn and Young Infant

“…Adrenal insufficiency (AI) is a potentially life-threatening condition that needs urgent diagnosis and treatment (1)(2)(3)(4). AI is relatively rare in early life, affecting approximately 1:5,000-10,000 children, and its features can be non-specific.…”