Iatrogenic tertiary AI caused by suppression of the hypothalamic-pituitary adrenal (HPA) axis secondary to glucocorticoid administration is the most common cause of central AI, with an estimated prevalence of 150- 280 per 1,000,000. Secondary AI secondary to intracranial pathology is also rare and may be isolated deficiency of ACTH or CRH, or it may be part of other pituitary hormonal deficiencies, called hypopituitarism.
The most common cause of CAH is 21-hydroxylase deficiency, accounting for ~ 90% of all CAH cases, with an incidence of 1 in 14,000 live births. The most common cause of primary AI in children is congenital adrenal hyperplasia (CAH) which accounts for 70% of pediatric patients with primary AI, whereas autoimmune adrenalitis (Addison’s disease) accounts for up to 15% of cases. Primary AI is rare with a prevalence of approximately 93-140 per 1,000,000. The secondary and tertiary AI can also be called central AI. IntroductionĪdrenal Insufficiency (AI) may be caused by destruction or dysfunction of the adrenal gland (primary AI, Addison’s disease), deficient pituitary adrenocorticotrophic hormone (ACTH) secretion (secondary AI), or deficient hypothalamic secretion of corticotropic releasing hormone (CRH) (tertiary AI). This review summarizes the etiologies, presentation, and diagnosis of adrenal insufficiency utilizing different dynamic hormone testing and describes current treatment recommendations and new therapies. Timely diagnosis and clinical management of adrenal insufficiency are critical to prevent morbidity and mortality. 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. Adrenal insufficiency may result from a wide variety of congenital or acquired disorders of hypothalamus, pituitary, or adrenal cortex.
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