Difference between revisions of "Congenital adrenal hyperplasia"

(Source)
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==Disposition==
 
==Disposition==
 
Admission
 
Admission
 +
 +
==See Also==
 +
*[[Adrenal Crisis]]
  
 
==Source==
 
==Source==
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[[Category:Peds]]
 
[[Category:Peds]]
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[[Category:Endo]]

Revision as of 06:16, 31 October 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

  1. Adrenal salt-wasting crisis
  2. Sepsis
  3. Congenital heart disease
  4. Inborn errors of metabolism

Treatment

  1. NS 10-20cc/kg bolus
  2. Steroid replacement
    1. Neonates: Hydrocortisone 25mg IV/IO
  3. Hyperkalemia
    1. Do NOT give insulin/glucose (may lead to profound hypoglycemia)
    2. NS and hydrocortisone are usually sufficient
    3. May add calcium gluconate if symptomatic

Disposition

Admission

See Also

Source

Tintinalli