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EPIDEMIOLOGY

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The exact incidence of acute adrenal insufficiency in the neonate is unknown but likely mirrors the incidence of the underlying causes of the adrenal failure. Newborn screening and heightened awareness have resulted in earlier diagnosis of congenital adrenal hyperplasia (CAH) before an acute crisis develops. The incidence of adrenal suppression from pharmacological doses of steroids is likely underestimated, and the exact frequency of adrenal crises in these cases is not known.

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PATHOPHYSIOLOGY AND PRESENTATION

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An essential component of the normal stress response is the adrenal gland. Cortisol production is increased during stress to maintain hemodynamic stability and glucose homeostasis. In these instances, cortisol prevents hypotension by promoting water and sodium retention, increasing angiotensinogen synthesis in the liver, increasing the vascular reactivity to vasoconstrictors, promoting the enzymatic conversion of norepinephrine to epinephrine, decreasing capillary permeability, and decreasing nitric oxide and other vasodilatory mediators.1 Cortisol also prevents hypoglycemia by decreasing glucose uptake in the muscle, promoting catabolism of protein and muscle to produce substrates for gluconeogenesis, and increasing expression of gluconeogenic enzymes.1 Aldosterone is the primary mineralocorticoid produced in the adrenal cortex and exerts effects on the kidney by stimulating reabsorption of sodium and secretion of potassium and hydrogen ions. If an inadequate cortisol response occurs in the face of physiologic stress, the infant will develop an acute adrenal crisis.

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Central Adrenal Insufficiency

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In infants with central adrenal failure, ACTH (corticotropin) is deficient. ACTH primarily regulates glucocorticoid production and secretion. The renin-aldosterone system is controlled by systemic volume and blood pressure and is unaffected in cases of central adrenal failure; thus, the neonate will not have the severe hyperkalemia or hyponatremia that accompanies mineralocorticoid deficiency. The infant will still be at risk for hypotension, milder hyponatremia, and hypoglycemia.

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Primary Adrenal Insufficiency

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The adrenal cortex is affected in primary adrenal insufficiency; thus, the neonate may have both cortisol and mineralocorticoid deficiency. These infants are at risk for a salt-wasting adrenal crisis consisting of marked hyperkalemia, hyponatremia, dehydration, and hypotension.

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Acute Adrenal Failure or Crisis

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In the neonate, adrenal failure can result in complete circulatory collapse and metabolic dysregulation termed an adrenal crisis. Other clinical features of acute adrenal failure include severe hyperkalemia and hyponatremia and hypoglycemia, including seizure, hypotension, dehydration, prolonged jaundice, fever, vomiting, and acidosis (Table 104-1). Left untreated, the crisis will progress to shock and death.

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Table Graphic Jump Location
Table 104-1Clinical Features of Acute Adrenal Failure
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DIFFERENTIAL DIAGNOSIS

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Any form of adrenal insufficiency, whether from primary or central causes (Table 104-2), can present as acute adrenal failure or crisis. Transient ...

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