Difference between revisions of "Congenital adrenal hyperplasia"

(Text replacement - "Pts " to "Patients ")
(Clinical features/evaluation)
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*Patients present during 2nd-5th week of life in crisis
 
*Patients present during 2nd-5th week of life in crisis
  
==Diagnosis==
+
==Clinical features/evaluation==
 
*Patient presents in 2nd week of life in crisis
 
*Patient presents in 2nd week of life in crisis
**Lethargy, irritability, poor feeding, vomiting, dehydration, shock
+
**[[Lethargy]], irritability, poor feeding, [[vomiting]], [[dehydration (peds)|dehydration]], [[pediatric shock|shock]]
 
*Salt-wasting
 
*Salt-wasting
**Hyponatremia, hyperkalemia
+
**[[Hyponatremia]], [[hyperkalemia]]
 
*Virilization
 
*Virilization
  
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*Bedside glucose
 
*Bedside glucose
 
*Chemistry
 
*Chemistry
**Hyponatremia
+
**[[Hyponatremia]]
**Hyperkalemia - rarely causes cardiac dysfunction
+
**[[Hyperkalemia]] - rarely causes cardiac dysfunction
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
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==Management==
 
==Management==
 
#[[NS]] 10-20cc/kg bolus
 
#[[NS]] 10-20cc/kg bolus
#Steroid replacement
+
#[[Steroid]] replacement
 
#*Neonates: [[Hydrocortisone]] 25mg IV/IO
 
#*Neonates: [[Hydrocortisone]] 25mg IV/IO
 
#[[Hyperkalemia]]
 
#[[Hyperkalemia]]
#*Do NOT give insulin/glucose (may lead to profound [[Neonatal hypoglycemia|hypoglycemia]])
+
#*Do NOT give [[insulin]]/glucose (may lead to profound [[Neonatal hypoglycemia|hypoglycemia]])
 
#*[[NS]] and [[hydrocortisone]] are usually sufficient
 
#*[[NS]] and [[hydrocortisone]] are usually sufficient
 
#*May add [[calcium gluconate]] if symptomatic
 
#*May add [[calcium gluconate]] if symptomatic

Revision as of 21:59, 25 August 2019

Background

  • 95% of cases due to deficiency of 21-hydroxylase
    • Leads to cortisol deficiency, aldosterone deficiency, virilization
  • Patients present during 2nd-5th week of life in crisis

Clinical features/evaluation

Work-Up

Differential Diagnosis

  • Adrenal salt-wasting crisis

Sick Neonate

THE MISFITS [1]

Management

  1. NS 10-20cc/kg bolus
  2. Steroid replacement
  3. Hyperkalemia

Disposition

Admission

See Also

References

  1. Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.