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PAEDIATRICS


CONGENITAL ADRENAL HYPERPLASIA

Vomiting + flat neonate 1 wk -2 months
You WILL get a gas. Vomiting + Acidotic Gas = CAH
(Other neonatal gas + vomiting - Hypochloraemic met Alkalosis = Pyloric stenosis)
PromptVBG findings
ResponseAcidosis - HAGMA +/- NAGMA from bicarb losses or renal- Acidosis indicates mineralocorticoid deficiency
Respiratory compensation - Reduced GCS/hyperventilation
Hyperkalaemia + Hyponatraemia - mineralocorticoid deficiency
Hypoglycaemia - Low in cortisol deficiency
Hypochloraemia - vomiting
Lactate
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PromptDifferentials for CAH
ResponseCAH
Congenital heart disease
Dehydration
Renal failure
Sepsis
GI disorder - Malritation, pyloric stenosis, Necrotising enterocolitis, intussusception
Inborn error of metabolism (lethargy, history of seizure)
Endocrine disorders
Heart failure
THE MISFITS
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PromptInvestigations in CAH
ResponseInvestigations ideally prior to steroid administration
Cortisol
17 hydroxyprogesterone
Plasma renin
ACTH level
Urinary steroid profile

EUC - hyponatraemia + hyperkalaemia
Blood gas
Blood glucose (formal and bedside) - low = cortisol insufficiency
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PromptManagement of CAH
ResponseSeverely unwell if unstable or unwell (Vomiting/diarrhoea/drowsy)
Steroid replacement
  • 25 mg IV/IM (up to age 3) then 5-10 mg Q6h
  • Fludrocortisone 0.05- 0.1mg (not really needed acutely though)
IV fluids
  • 10-20 mL/kg N/S + repeat until circulation restored - SBP>80, CRT <3s
  • Remaining deficit + maintenance over next 24h N/S+5%
  • Gas + EUC monitoring Q2h
Treat hypoglycaemia
  • Bolus 10% dextrose 2-5 mL/kg + recheck 30 mins. Target >4
  • Can increase to 10% dextrose + N/S if needing to
Hyperkalaemia
  • ECG if K>6
  • Calcium gluconate 0.5 mL/kg + insulin infusion 0.1 u/kg/h
Precipitating illness/injury
  • Seek and treat precipitating illness
Consultation with paediatrics for admission + PICU
Emperic antibiotics
RCH + some answers added in
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