Addison Disease

(aka primary adrenal insufficency*)

CHRISSY RAMAN
MAY 20, 2011
 *

Primary Adrenal Insufficiency can be caused by

CAH, sepsis, AIDS, a drenoleukodystrophy,
TB...autoimmune adrenalitis is most comon cause
 Incidence in adults 1 in 75,000, lower in kids. In adults, 80%
are women; in kids, 75% are boys
 Caused by circulating anti-adrenal antibodies; autopsy shows
lymphocytic infiltrate of adrenal cortex
 Signs and Symptoms

Glucocorticoid deficiency signs are earlier: weakness,

weight loss, fatigue, hypoglycemia, anorexia
Mineralocorticoid deficiency signs are later:
hypotension, hyponatremia/hypokalemia,
tachycardia, acidosis
Signs of chronic adrenal insufficiency:
hyperpigmentation, diarrhea, sparse axillary or
pubic hair, weight loss
 Hyperpigmentation most notable on sun-exposed skin and on
flexor surfaces
 Adrenal Crisis

Commonly seen in kids with undiagnosed chronic

adrenal insufficiency when exposed to
stress/trauma/illness/surgery
Abdominal pain, fever, hypoglycemia (poss with
seizures), n/v/d, electrolyte disturbances (hypo-Na,
hypo-K, hypo-Cl), hypotension, cardiovascular
collapse, death!
Treatment is fluids, electrolytes and STRESS DOSE
STEROIDS (and controlling underlying issue)
 Diagnosis

Low first morning cortisol level with high ACTH

May do ACTH stimulation test (measure cortisol
after 1 hr IV ACTH)
 Cortisol level still low in Addison’s
Supporting findings: hypoglycemia, lymphocytosis,
azotemia, anemia, eosinophilia
Don't forget to look for other endocrine probs: 50%
of adults have antibodies to other endocrine organs.
 Treatment

Adrenal crisis/stress-dosing: Hydrocortisone 50

mg/m2 divided Q6 hrs in addition to…
Maintenance:
 Hydrocortisone 15-25 mg/m2 divided Q8  
 Prednisolone 3-5 mg/m2 divided Q12 
 Allow free access to salty foods
 Sources

Rudolph, Colin D, et al. Rudolph's Pediatrics, 21st

ed, McGraw-Hill 1996
Uptodate.com. “Primary Adrenal Insufficiency”
accessed 5/14/11
Uptodate.com “Treatment of Primary Adrenal
Insufficiency in Children” accessed 5/14/11
Thanks!