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Management of Children with an Adrenal Crisis

The following guidelines are generally applicable. Individual situations may require deviation from these guidelines.

DIAGNOSIS

  1. Signs and symptoms of Adrenal Crisis:

    • weakness

    • fever

    • abdominal pain

    • hypotension

    • dehydration

    • hypoglycemia

    • seizure

    • shock
       

  2. Patient may have a history of adrenal insufficiency or exogenous glucocorticoid use

    Note: MEGACE® suppresses hypothalamic-pituitary-adrenal axis and adrenal crisis can occur shortly after discontinuation of MEGACE®.
     

  3. Undiagnosed primary adrenal insufficiency causes chronic fatigue, weakness, abdominal pain, anorexia, weight loss, vomiting, diarrhea, salt craving, skin pigmentation
     

  4. Other autoimmune conditions may be a clue to the presence of Addison disease
     

  5. Undiagnosed ACTH deficiency may be associated with headache, visual changes, seizures, focal neurological signs
     

  6. Laboratory Evaluation drawn BEFORE administering exogenous steroids
     

  7. DO NOT wait for results to come back to start treatment if you suspect Adrenal Crisis

  • STAT labs for diagnosis-serum electrolytes and glucose, urine
    electrolytes
  • Check creatinine, BUN, Urinalysis, CBC with diff to assist with management
  • Draw 5-10 mL extra clotted blood in case you want to measure steroid concentrations
  • ACTH levels require pre-chilled purple top tube sent immediately to lab

Table 1. STAT Lab Results Supporting Diagnosis of Adrenal Insufficiency

  Serum  Urine
Na+  ↑
K+
Total CO2  
Glucose  ↓  

Consult attending physician/endocrinologist for appropriate diagnostic tests if patient not known to have a condition that causes adrenal insufficiency (See Table 2 below)

Table 2 – Endocrine Lab Assessment of Adrenal Insufficiency

Condition Under Consideration Cortisol ACTH 17-OH-Progesterone Aldo Renin
Primary Adrenal Failure (e.g. Hemorrhage, Waterhouse-Friderichsen, Autoimmune, Infection, Neoplasia, Adrenoleukodystrophy, Hypoplasia)

 

X

 

X

 

-

 

X

 

X

ACTH Deficiency (Pituitary/Hypothalamus)

X

X

-

-

-

21-Hydroxylase Deficiency

-

-

X

-

X

  1. Further specific diagnostic testing is required to establish exact cause of adrenal insufficiency

TREATMENT OF ADRENAL CRISIS

  1. Assure airway and breathing are adequate
     
  2. Patient will virtually always have significant volume contraction
  • Cardiac monitor
  • Replace fluid loss
    • Isotonic crystalloid with added dextrose
    • 20 mL/kg rapid infusion
  • May need to repeat bolus
  1. Give IV glucocorticoid – if possible, AFTER diagnostic blood tests drawn (Table 1 above)

Table 3.  IV Steroid Treatment for Adrenal Crisis

Choice Benefit Drawback
Hydrocortisone q8h Mineralocorticoid effect Interferes with blood tests
<3 years old – 25 mg    
3-12 years old– 50 mg    
>12 years old – 100 mg    
     
Dexamethasone q12h    
<3 years old – 0.5 mg No effect on blood tests No mineralocorticoid effect
3-12 years old – 1 mg Long acting  
>12 years old – 2-4 mg    
     
Prednisolone q12h    
<3 years old – 5 mg Active metabolite          May interfere with tests
3-12 years old – 10 mg Moderate mineralocorticoid  
>12 years old – 20 mg           
  1. Ongoing Care

    • Admit to PICU

    • Close monitoring of hydration and blood glucose

    • Check electrolytes q2-4 h depending on degree of illness

    • Replace 50% of fluid deficit in first 8 hours, using 0.9% saline with 5% glucose

    • Replace remaining fluid deficit over 8-24 hours

    • Add potassium to IV as allowed by serum K+ level

    • Diagnose cause of adrenal insufficiency (See Table 4)

    • Taper glucocorticoid to physiological dose over 1-3 days as patient condition allows

    • Begin oral fludrocortisone (0.1 mg once daily) if mineralocorticoid deficiency present

    • Prescribe injectable (IM) steroid for home emergency use (e.g. Hydrocortisone)

    • Advise patient to wear medical ID indicating “adrenal insufficiency.”

Table 4. Features of Adrenocortical Failure Caused by Adrenal or CNS Disease

 

Adrenalcortical Disease

Hypothalamic-Pituitary Disease

Biosynthetic Defect (CAH – 21-OHase)

Deficient Hormone(s)

Cortisol
Aldosterone

ACTH/CRF
Cortisol
(Aldosterone pathway intact)

Cortisol
±Aldosterone (salt wasting variant)

Hyperpigmentation

Yes

No

Yes

Serum Na+

Low (salt wasting)

Mildly low when patient well (unable to excrete free water load)

Low if salt waster

Serum K+

High

Normal

High if salt waster

Serum ACTH

High

Low

High

Cortisol Response to IV ACTH

None

Increase

Increase

17-OH Progesterone Response to IV ACTH

None

Increase

Exaggerated increase


Copyright 2002
Medical College of Georgia
All rights reserved.

Pediatric Endocrinology
Department of Pediatrics | Medical College of Georgia
Please email comments, suggestions or questions to:
Andrew Muir, M.D., amuir@mcg.edu

February 27, 2004