PAEDIATRICS
CONGENITAL ADRENAL HYPERPLASIA
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
Respiratory compensation - Reduced GCS/hyperventilation
Hyperkalaemia + Hyponatraemia - mineralocorticoid deficiency
Hypoglycaemia - Low in cortisol deficiency
Hypochloraemia - vomiting
Lactate
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
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
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
Cortisol
17 hydroxyprogesterone
Plasma renin
ACTH level
Urinary steroid profile
EUC - hyponatraemia + hyperkalaemia
Blood gas
Blood glucose (formal and bedside) - low = cortisol insufficiency
PromptManagement of CAH
ResponseSeverely unwell if unstable or unwell (Vomiting/diarrhoea/drowsy)
Steroid replacement
Emperic antibiotics
RCH + some answers added in
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)
- 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
- Bolus 10% dextrose 2-5 mL/kg + recheck 30 mins. Target >4
- Can increase to 10% dextrose + N/S if needing to
- ECG if K>6
- Calcium gluconate 0.5 mL/kg + insulin infusion 0.1 u/kg/h
- Seek and treat precipitating illness
Emperic antibiotics
RCH + some answers added in
You WILL get a gas. Vomiting + Acidotic Gas = CAH
(Other neonatal gas + vomiting - Hypochloraemic met Alkalosis = Pyloric stenosis)