Congenital adrenal hyperplasia: Difference between revisions
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==DDx== | ==DDx== | ||
#Adrenal salt-wasting crisis | |||
#Sepsis | #Sepsis | ||
#Congenital heart disease | #Congenital heart disease |
Revision as of 19:23, 25 August 2011
Background
- 95% of cases due to deficiency of 21-hydroxylase
- Leads to cortisol deficiency, aldosterone deficiency, virilization
- Pts present during 2nd-5th week of life in crisis
Diagnosis
- Pt presents in 2nd week of life in crisis
- Lethargy, irritability, poor feeding, vomiting, dehydration, shock
- Salt-wasting
- Hyponatremia, hyperkalemia
- Virilization
Work-Up
- Bedside glucose
- Chemistry
- Hyponatremia
- Hyperkalemia - rarely causes cardiac dysfunction
DDx
- Adrenal salt-wasting crisis
- Sepsis
- Congenital heart disease
- Inborn errors of metabolism
Treatment
- NS 10-20cc/kg bolus
- Steroid replacement
- Neonates: Hydrocortisone 25mg IV/IO
- Hyperkalemia
- Do NOT give insulin/glucose (may lead to profound hypoglycemia)
- NS and hydrocortisone are usually sufficient
- May add calcium gluconate if symptomatic
Disposition
Admission
Source
Tintinalli