addisons disease

Addisons disease, also called adrenal hypofunction or adrenal

insufficiency, occurs in two forms: primary and secondary. This
relatively uncommon disorder occurs in people of all ages and
both sexes. Either primary or secondary Addisons disease can
progress to adrenal crisis.
Acute adrenal crisis, also called addisonian crisis, is a deficiency
of mineralocorticoids and glucocorticoids that requires
immediate treatment. (See Understanding adrenal crisis.)
Understanding adrenal crisis
Adrenal crisis (acute adrenal insufficiency) is the most
serious complication of Addisons disease. It may occur
gradually or suddenly.
Whos at risk
This potentially lethal condition usually develops in
patients who:
dont respond to hormone replacement therapy
undergo extreme stress without adequate glucocorticoid
replacement
abruptly stop hormone therapy
undergo trauma
undergo bilateral adrenalectomy
develop adrenal gland thrombosis after a severe infection
(Waterhouse-Friderichsen syndrome).
What happens
In adrenal crisis, destruction of the adrenal cortex leads
to a rapid decline in the steroid hormones cortisol and
aldosterone. This directly affects the liver, stomach, and
kidneys. The flowchart below depicts what happens in
adrenal crisis.
What causes it
Causes of Addisons disease are classified according to whether
they result in primary or secondary hypofunction.
Whos on first?
In primary hypofunction, the cause lies with the adrenal glands
when approximately 90% of the gland is destroyed, apparently
through an autoimmune process.
Other causes of primary hypofunction include:
tuberculosis
bilateral adrenalectomy
hemorrhage into the adrenal glands
neoplasms
infections.
Whats on second?
In secondary hypofunction, the cause lies outside the adrenal
gland and includes:
pituitary gland (decreased secretion of ACTH)
abrupt steroid withdrawal due to adrenal atrophy from longterm
therapy (which suppresses corticotropin secretion by the
pituitary)
removal of a corticotropin-secreting tumor.
how it happens
Addisons disease is characterized by decreased secretion of the
adrenal hormones glucocorticoids (cortisol and aldosterone), mineralocorticoids,
and androgens. Addisonian crisis can occur with
severe stress, such as illness, surgery, sepsis, trauma, and abrupt
discontinuation of steroid therapy.
Release the aldosterone!
Aldosterone release occurs in response to hypovolemia as the
body attempts to maintain vascular volume. Aldosterone causes
the renal tubules to resorb sodium. As sodium is resorbed, water
naturally follows and is resorbed. Vascular volume and pressure
increase. Cortisol release causes an increase in glucose production
as the body attempts to supply the body with this fuel.
Emergency!
In acute addisonian crisis, both hormones are suddenly depleted.
Heres what happens:
First, blood pressure drops, due to hypovolemia and hyponatremia.
Standard pressor therapy is difficult to regulate because
the response to catecholamines by the patient is unpredictable.
Second, blood glucose plummets and coma and death ensue if
immediate treatment isnt available.
What to look for
Typical clinical features of Addisons disease include:
profound hypotension
dehydration
profound weakness and fatigue
nausea and vomiting
hypoglycemia
neurologic changes
hyperkalemia
hyponatremia
hypercalcemia
tachycardia
hyperpigmentation.
What tests tell you
In a patient with typical addisonian symptoms, these findings
strongly suggest acute adrenal insufficiency:
Plasma cortisol is decreased.
Serum sodium and fasting blood glucose levels are decreased.
Corticotropin is increased.
Serum potassium level is increased.
Serum BUN level is increased.
Various radiologic tests identify adrenal gland size such as
X-rays that show adrenal calcification.
how its treated
Treatment for the patient with adrenal crisis is prompt I.V. bolus
administration of 100 mg of hydrocortisone, followed by hydrocortisone
diluted with normal saline with added electrolytes given
as an I.V. infusion until the patients condition stabilizes.
Furthermore...
Further treatment includes:
aggressive fluid replacement with up to 5 L of I.V. saline with
electrolyte replacement, such as sodium and potassium
vasopressors (if the patient doesnt respond to the initial treatment),
such as epinephrine or norepinephrine, titrated to the
patients blood pressure
hormone replacement, such as hydrocortisone or fludrocortisone
blood glucose management with I.V. dextrose solution
maintenance corticosteroid (hydrocortisone) replacement when
the patients condition is stable.
What to do
Explain all procedures and tests to the patient and his family.
Monitor the patients vital signs and oxygen saturation closely.
Assist with insertion of a pulmonary artery catheter as indicated
for fluid status evaluation. Assess hemodynamic parameters and
monitor for shock.
Assess the patients respiratory status and auscultate breath
sounds for crackles, which may indicate fluid overload.
Monitor cardiac rhythm and assess for possible arrhythmias
secondary to electrolyte imbalance (evidenced by tall, tented T
waves and widening QRS complex associated with hyperkalemia).
Monitor the patients intake and output, fluid replacement therapy,
and daily weight.
Monitor the patients blood glucose levels and administer I.V.
glucose if necessary.
Monitor electrolytes for sodium and potassium imbalances,
including hyponatremia and hyperkalemia during crisis.
Monitor renal function studies and laboratory test results,
including hemoglobin (Hb) and hematocrit, serum electrolyte, and
blood glucose levels.
Monitor the patients NG tube in case of vomiting.
Institute emergency measures if needed, such as mechanical
ventilation in case of cardiopulmonary arrest.
Maintain a quiet environment.
Maintain standard infection control measures.