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Key Features

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Essentials of Diagnosis
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  • Primary adrenal insufficiency

    • Hereditary enzyme defects (congenital adrenal hyperplasia)

    • Autoimmune destruction of the glands (Addison disease)

      • May be familial

      • Can be isolated or associated with hypoparathyroidism, mucocutaneous candidiasis, hypothyroidism, pernicious anemia, hypogonadism, and diabetes mellitus

    • Destruction of the gland by tumor, calcification, or hemorrhage (Waterhouse-Friderichsen syndrome)

    • Adrenal disease secondary to opportunistic infections (fungal or tuberculous) is reported in immunocompromised patients

  • Central adrenal insufficiency

    • Intracranial neoplasm or its treatment

    • Congenital midline defects associated with optic nerve hypoplasia (septo-optic dysplasia)

    • Chronic iatrogenic exposure to exogenous glucocorticoids

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General Considerations
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  • Acute illness, surgery, trauma, or hyperthermia may precipitate an adrenal crisis in patients with adrenal insufficiency

  • Patients with primary adrenal insufficiency are at greater risk for life-threatening crisis than patients with central ACTH deficiency because mineralocorticoid secretion and low-level autonomous cortisol secretion remain intact in central ACTH deficiency

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Clinical Findings

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Symptoms and Signs
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  • Acute form (adrenal crisis)

    • Nausea, vomiting, diarrhea, abdominal pain

    • Dehydration

    • Fever (sometimes followed by hypothermia)

    • Weakness

    • Hypotension and circulatory collapse

    • Confusion and coma

  • Chronic form

    • Fatigue

    • Hypotension

    • Weakness

    • Weight loss or failure to gain weight

    • Salt craving (primary insufficiency)

    • Vomiting and dehydration

    • Recurrent hypoglycemia

    • Diffuse tanning occurs with increased pigmentation over pressure points, scars, and mucous membranes

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Differential Diagnosis
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  • Sepsis diabetic coma

  • CNS disturbances

  • Acute poisoning

  • In the neonatal period, may be clinically indistinguishable from respiratory distress, intracranial hemorrhage, or sepsis

  • Anorexia nervosa

  • Certain muscular disorders (myasthenia gravis)

  • Salt-losing nephritis

  • Chronic debilitating infections

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Diagnosis

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Laboratory Findings
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  • Primary adrenal insufficiency

    • Decreased serum sodium, serum bicarbonate, arterial partial pressure of carbon dioxide, blood pH, and blood volume

    • Increased serum potassium, urea nitrogen levels

    • Urinary sodium and the ratio of urinary sodium to potassium are inappropriate for the degree of hyponatremia

    • Eosinophilia and moderate lymphopenia

    • Plasma cortisol < 18 mcg/dL 30 and 60 minutes after 250 μg of cosyntropin given intravenously and aldosterone fails to rise above baseline

    • Baseline serum ACTH concentration

      • Elevated in primary adrenal failure

      • Low in central adrenal insufficiency

    • Decreased urinary free cortisol

  • Central adrenal insufficiency

    • Serum sodium levels may be mildly decreased as a result of impaired water excretion

    • Eosinophilia and moderate lymphopenia

    • Plasma cortisol < 18 mcg/dL 30 and 60 minutes after 1 μg of cosyntropin given intravenously

    • Baseline serum ACTH concentration

      • Elevated in primary adrenal failure

      • Low in central adrenal insufficiency

    • Decreased urinary free cortisol

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Treatment

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  • Acute insufficiency (adrenal crisis)

    • Hydrocortisone sodium succinate

      • 50 mg/m2 intravenously over 2–5 minutes or intramuscularly) is given initially followed by 12.5 mg/m2 every 4–6 hours until stabilization is achieved and oral therapy can be tolerated

      • Cortisol replacement is critical because vasopressor agents may be ineffective in adrenal insufficiency

    • Fluids and electrolytes

      • In primary adrenal insufficiency, 5–10% glucose in normal saline, 10–20 mL/kg intravenously, is given over the first hour and repeated if necessary to reestablish vascular volume

      • Normal saline is continued thereafter at 1.5–2 times the maintenance fluid requirements

      • In central adrenal insufficiency, routine fluid management is generally ...

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