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Signs and symptoms of adrenal insufficiency are listed in Table 534-1. Most patients with chronic adrenal
insufficiency have weakness, fatigue, anorexia, weight loss, hypotension,
and hyperpigmentation. 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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Many conditions will cause adrenal insufficiency, including congenital
adrenal hyperplasia (CAD), hypopituitarism with corticotropin (ACTH)
deficiency, and primary adrenal disorders. 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 534-2.
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.1-3
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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 ...