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SYMPTOMS AND CAUSES

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Most patients with chronic adrenal insufficiency have weakness, fatigue, anorexia, weight loss, hypotension, and hyperpigmentation (Table 527-1). Patients with acute adrenal insufficiency may have hypotension, shock, weakness, apathy, confusion, anorexia, nausea, vomiting, dehydration, abdominal or flank pain, hyperthermia, and hypoglycemia. Deficient adrenal androgen secretion will compromise the acquisition of virilizing secondary sexual characteristics (pubic and axillary hair, acne, axillary odor) in female adolescents.

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TABLE 527-1SIGNS AND SYMPTOMS OF ADRENAL INSUFFICIENCY
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Many conditions will cause adrenal insufficiency, including congenital adrenal hyperplasia, hypopituitarism, and primary adrenal disorders (Table 527-2). Primary adrenal insufficiency is commonly termed Addison disease; in adults, over 80% of Addison disease is due to autoimmune adrenalitis. The adrenal disorders of infants, children, and adolescents differ from those of adults and are shown in Table 527-2. Adrenal disorders are typically divided into chronic and acute causes, but many acute presentations reflect emergence of an undiagnosed underlying chronic or developmental process. Acute presentations may be triggered by intercurrent illness, trauma, or surgery, with poor fluid and sodium intake. A correct diagnosis facilitates long-term management and genetic counseling and assessing associated features.

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TABLE 527-2CAUSES OF ADRENAL INSUFFICIENCY
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PRIMARY ADRENAL INSUFFICIENCY

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Acute Primary Adrenal Disease

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Acute adrenal crisis commonly occurs in the child with undiagnosed chronic adrenal insufficiency at the time of additional stress (major illness, trauma, or surgery). The presenting symptoms and signs include abdominal pain, fever, hypoglycemia with seizures, weakness, apathy, nausea, vomiting, anorexia, hyponatremia, hypochloremia, acidemia, hyperkalemia, hypotension, shock, cardiovascular collapse, and death. Treatment requires fluid and electrolyte resuscitation, ample doses of glucocorticoids, chronic glucocorticoid and mineralocorticoid replacement, and treatment of the precipitating illness.

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