Pediatric CAH - Dr. Agarwal

Congenital Adrenal Hyperplasia

• Autosomal recessive, no cortisol due to 21-hydroxlase deficiency most commonly
• No cortisol, no aldosterone... Only testosterone
• Classic- salt wasting, simple virilizing
• Non classic- late virilization
  

• Genital ambiguity - female more obvious, males may have only large phallus
• Hyper pigmentation - mucosa, palmar creases
• Androgen excess - early puberty- males less than 9, girls less than 8
• Fertility issues, testes cancer, adrenal crisis
  

Consider CAH in sick neonate
Always consider adrenal insufficiency in hypotensive patients who remain hypotensive despite appropriate fluid resuscitation and initiation of pressors (whether adult or pediatric)... but particularly think of it in neonates!!

• Hyperkalemia,  hyponatremia
• Management: fluids, glucose, treat hyperK, manage airway
• Stress dose steroids
  

1) BSA dosing - 100mg/m2 IV hydrocortisone
2) Age-based - 0-3 yo: 25mg IV; 3-12 yo: 50mg IV; >12 yo: 100mg IV
3) Randy's Rules (from the brilliant mind of Dr. Cordle) - Give at LEAST 25mg; 3x their current dose; or 2mg/kg

Steroid Production