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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
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Signs and symptoms of Adrenal Crisis:
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weakness
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fever
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abdominal pain
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hypotension
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dehydration
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hypoglycemia
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seizure
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shock
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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®.
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Undiagnosed primary adrenal insufficiency causes
chronic fatigue, weakness, abdominal pain, anorexia, weight loss,
vomiting, diarrhea, salt craving, skin pigmentation
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Other autoimmune conditions may be a clue to the
presence of Addison disease
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Undiagnosed ACTH deficiency may be associated with
headache, visual changes, seizures, focal neurological signs
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Laboratory Evaluation drawn BEFORE
administering exogenous steroids
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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 |
↓ |
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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 |
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Further specific diagnostic testing is required to
establish exact cause of adrenal insufficiency
TREATMENT OF ADRENAL CRISIS
- Assure airway and breathing are adequate
- 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
- 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 |
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| 3-12 years old– 50 mg |
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| >12 years old – 100 mg |
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| |
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| Dexamethasone q12h |
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| <3 years old – 0.5 mg |
No effect on blood tests |
No mineralocorticoid effect |
| 3-12 years old – 1 mg |
Long acting |
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| >12 years old – 2-4 mg |
|
|
| |
|
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| Prednisolone q12h |
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| <3 years old – 5 mg |
Active metabolite
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May interfere with tests |
| 3-12 years old – 10 mg |
Moderate mineralocorticoid |
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| >12 years old – 20 mg
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Ongoing Care
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Admit to PICU
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Close monitoring of hydration and blood glucose
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Check electrolytes q2-4 h depending on degree of
illness
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Replace 50% of fluid deficit in first 8 hours, using
0.9% saline with 5% glucose
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Replace remaining fluid deficit over 8-24 hours
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Add potassium to IV as allowed by serum K+ level
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Diagnose cause of adrenal insufficiency (See Table 4)
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Taper glucocorticoid to physiological dose over 1-3
days as patient condition allows
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Begin oral fludrocortisone (0.1 mg once daily) if
mineralocorticoid deficiency present
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Prescribe injectable (IM) steroid for home emergency
use (e.g. Hydrocortisone)
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Advise patient to wear medical ID indicating “adrenal
insufficiency.”
Table 4. Features of Adrenocortical Failure Caused
by Adrenal or CNS Disease
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Adrenalcortical Disease |
Hypothalamic-Pituitary Disease |
Biosynthetic Defect (CAH – 21-OHase) |
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Deficient Hormone(s) |
Cortisol
Aldosterone |
ACTH/CRF
Cortisol
(Aldosterone pathway intact) |
Cortisol
±Aldosterone (salt wasting variant) |
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Hyperpigmentation |
Yes |
No |
Yes |
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Serum Na+ |
Low (salt wasting) |
Mildly low when patient well (unable to excrete
free water load) |
Low if salt waster |
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Serum K+ |
High |
Normal |
High if salt waster |
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Serum ACTH |
High |
Low |
High |
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Cortisol Response to IV ACTH |
None |
Increase |
Increase |
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17-OH Progesterone Response to IV ACTH |
None |
Increase |
Exaggerated increase |
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