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Primary Adrenal Insufficiency, Pediatric

Jennifer M. Barker, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Deficiency in the secretion of cortisol and/or aldosterone by the adrenal glands 

EPIDEMIOLOGY

  • Age

    • Adrenal insufficiency associated with congenital adrenal hyperplasia (CAH) presents in...

DIAGNOSIS

HISTORY

Symptoms of primary adrenal insufficiency are nonspecific and similar to those found in many other disease processes. Symptoms may be present for years prior to diagnosis and can incl...

TREATMENT

MEDICATION

  • Acute adrenal crisis

    • Hydrocortisone: stress dosage of hydrocortisone: 100 mg/m2 IV/IM followed by 100 mg/m2/24 h of hydrocortisone divided q4–6h. Decrease to a physiologic replaceme...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Acute adrenal crisis usually improves rapidly with the administration of fluids and glucocorticoids. Once the acute phase of illness has resolved, steroids can be...

ADDITIONAL READING

  • Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and treatment of primary adrenal insufficiency: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab.  20...

CODES

ICD9

  • 255.41 Glucocorticoid deficiency

  • 759.1 Anomalies of adrenal gland

  • 277.86 Peroxisomal disorders

ICD10

  • E27.1 Primary adrenocortical insufficiency

  • Q89.1 Congenital malformations of adrenal gland

  • E71....

FAQ

  • Q: What are the indications for stress dosing and how rapidly can the stress hydrocortisone dose be tapered?

  • A: Patients will require stress dosing of hydrocortisone for surgical procedures, fever ...

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