CAH Adrenal Crisis Protocol
Source: Dabas A, et al. Management of Infants with Congenital Adrenal Hyperplasia. Indian Pediatrics 2020;57:159–164 — Box I (in-hospital protocol) and Web Box I (disease card / home rules).
Background: incidence of adrenal crisis in CAH ≈ 7.55 per 100 patient-years (El-Maouche 2018). GI and URI infections are the commonest triggers. Lower age, lower HC dose, and higher fludrocortisone dose are risk factors.
STEP 1 — Recognise: who and what
Suspect adrenal crisis in any known CAH patient with:
- Lethargy, poor feeding, or irritability
- Vomiting, diarrhoea, abdominal pain
- Dehydration, hypotension, shock
- Hypoglycaemia, seizures
- A preceding minor illness (URI, gastroenteritis, fever, trauma, surgery, missed doses)
Diagnosis is clinical — symptoms are non-specific. Do not wait for biochemistry.
Red-flag features for ICU triage: shock, altered sensorium, seizures, severe hyponatraemia / hyperkalaemia with ECG changes, hypoglycaemia.
STEP 2 — Home rules (what parents should have already done)
This is what the disease card instructs caregivers — confirm on arrival and continue:
| Scenario |
Action |
| Routine well days |
Continue regular oral hydrocortisone + fludrocortisone. Never stop steroids. |
| Minor illness — URI, acute diarrhoea, mild fever |
Double the usual oral hydrocortisone dose. Continue fludrocortisone. |
| Moderate–severe illness — vomiting, fever > 38.5 °C, lethargy, poor feeding, dehydration, surgery, trauma |
3–5× the usual oral hydrocortisone dose. Switch to IV if vomiting or hospitalised. Withhold fludrocortisone while on stress HC doses. |
| Pre-hospital, sick child |
Single IM/IV hydrocortisone bolus 50–75 mg/m² if available, then bring to hospital. |
| Hospitalised |
IV hydrocortisone 50–75 mg/m² stat, then resume protocol below. |
After the acute episode resolves, taper back to the maintenance HC dose and resume fludrocortisone.
STEP 3 — Resuscitate (A → B → C)
- Airway / Breathing — oxygen if hypoxic; intubate if obtunded.
- Circulation — wide-bore IV access. If shock signs:
- Isotonic saline 20 mL/kg over 10 min, repeat as needed up to 60 mL/kg.
- Further fluids guided by perfusion, urine output, electrolytes.
- Newborns: continue maintenance at 1.5–2× normal with half-normal saline in 5% dextrose (0.45% NaCl + D5W).
STEP 4 — Correct hypoglycaemia
- Check capillary glucose immediately.
- If low: 10% dextrose 5 mL/kg IV bolus. Recheck in 15 min.
STEP 5 — Give IV hydrocortisone (stress dose)
| Patient |
Bolus |
Maintenance (next 24 h) |
| Neonate |
~25 mg IV bolus |
5–6 mg IV every 6 h |
| Infant / child |
50–100 mg/m² IV bolus |
50–100 mg/m²/day divided q6h (4 doses) |
| Pragmatic age-based fallback |
Infants 25 mg, children 50 mg, adolescents 100 mg |
Same maintenance rule |
- Hydrocortisone provides both glucocorticoid AND sufficient mineralocorticoid activity at these stress doses — no separate fludrocortisone needed while IV HC is running.
- Continue IV until the child is tolerating oral intake; then step down to oral HC at 3–5× maintenance, then taper back to baseline over 24–48 h once illness resolves.
STEP 6 — Check and correct dyselectrolytaemia
- Serum Na, K, glucose, urea, creatinine, venous blood gas.
- Typical pattern in SW-CAH crisis: hyponatraemia, hyperkalaemia, metabolic acidosis, hypoglycaemia.
- Hyperkalaemia: ECG, calcium gluconate / insulin-dextrose / salbutamol as per standard paediatric protocols.
- Avoid sodium bicarbonate unless severe acidosis (pH < 7.1) — risk of worsening hypokalaemia later.
STEP 7 — Monitor
- Continuous vitals, hourly urine output, sensorium.
- Repeat electrolytes and glucose 2–4 hourly until stable.
- Watch for fluid overload, especially in neonates.
STEP 8 — Step-down and discharge
- Once vomiting stops and the child is tolerating fluids → switch to oral HC at 3–5× maintenance dose.
- Once afebrile and clinically well → taper to baseline maintenance over 24–48 h.
- Resume fludrocortisone when back on oral HC at maintenance.
- Before discharge: confirm parents understand sick-day rules, replenish any IM emergency hydrocortisone supply, re-issue the disease card if missing.
STEP 9 — Investigate the trigger
- Septic screen (urine, blood culture if febrile).
- Stool studies if diarrhoea.
- Review compliance — missed doses, expired stock, wrong preparation (pre-dissolved liquid).
- Review fludrocortisone dose — overdose can precipitate hypokalaemia and crises in some patients.
Quick reference card
- Suspect crisis → treat empirically, do not wait for labs.
- Fluids: NS 20 mL/kg bolus, up to 60 mL/kg if shocked.
- Hydrocortisone IV bolus:
- Neonate ≈ 25 mg
- Infant / child 50–100 mg/m²
- Maintenance: 50–100 mg/m²/day IV q6h; neonate ≈ 5–6 mg q6h.
- Glucose: 10% dextrose 5 mL/kg if hypoglycaemic.
- Withhold fludrocortisone while on stress HC.
- Home: double for minor illness, 3–5× for moderate–severe; never stop steroids.
Related skills
- [[cah-infant-hydrocortisone-dosing]] — chronic maintenance dosing.
- [[cah-fludrocortisone-salt-supplementation]] — mineralocorticoid management.
- [[cah-sick-day-rules]] — parent-facing version of Step 2.
1---2name: cah-adrenal-crisis-protocol3description: CAH Adrenal Crisis Protocol4---56# CAH Adrenal Crisis Protocol78**Source:** Dabas A, et al. Management of Infants with Congenital Adrenal Hyperplasia. *Indian Pediatrics* 2020;57:159–164 — Box I (in-hospital protocol) and Web Box I (disease card / home rules).910Background: incidence of adrenal crisis in CAH ≈ **7.55 per 100 patient-years** (El-Maouche 2018). GI and URI infections are the commonest triggers. Lower age, lower HC dose, and higher fludrocortisone dose are risk factors.1112---1314## STEP 1 — Recognise: who and what1516**Suspect adrenal crisis in any known CAH patient with:**1718- Lethargy, poor feeding, or irritability19- Vomiting, diarrhoea, abdominal pain20- Dehydration, hypotension, shock21- Hypoglycaemia, seizures22- A preceding minor illness (URI, gastroenteritis, fever, trauma, surgery, missed doses)2324**Diagnosis is clinical** — symptoms are non-specific. Do **not** wait for biochemistry.2526Red-flag features for ICU triage: shock, altered sensorium, seizures, severe hyponatraemia / hyperkalaemia with ECG changes, hypoglycaemia.2728---2930## STEP 2 — Home rules (what parents should have already done)3132This is what the **disease card** instructs caregivers — confirm on arrival and continue:3334| Scenario | Action |35|---|---|36| Routine well days | Continue regular oral hydrocortisone + fludrocortisone. **Never stop steroids.** |37| **Minor illness** — URI, acute diarrhoea, mild fever | **Double** the usual oral hydrocortisone dose. Continue fludrocortisone. |38| **Moderate–severe illness** — vomiting, fever > 38.5 °C, lethargy, poor feeding, dehydration, surgery, trauma | **3–5×** the usual oral hydrocortisone dose. Switch to IV if vomiting or hospitalised. **Withhold fludrocortisone** while on stress HC doses. |39| Pre-hospital, sick child | Single IM/IV hydrocortisone bolus **50–75 mg/m²** if available, then bring to hospital. |40| Hospitalised | IV hydrocortisone 50–75 mg/m² stat, then resume protocol below. |4142After the acute episode resolves, taper back to the maintenance HC dose and resume fludrocortisone.4344---4546## STEP 3 — Resuscitate (A → B → C)47481. **Airway / Breathing** — oxygen if hypoxic; intubate if obtunded.492. **Circulation** — wide-bore IV access. If shock signs:50 - **Isotonic saline 20 mL/kg over 10 min**, repeat as needed up to **60 mL/kg**.51 - Further fluids guided by perfusion, urine output, electrolytes.523. **Newborns:** continue maintenance at **1.5–2× normal** with **half-normal saline in 5% dextrose** (0.45% NaCl + D5W).5354---5556## STEP 4 — Correct hypoglycaemia5758- Check capillary glucose immediately.59- If low: **10% dextrose 5 mL/kg IV** bolus. Recheck in 15 min.6061---6263## STEP 5 — Give IV hydrocortisone (stress dose)6465| Patient | Bolus | Maintenance (next 24 h) |66|---|---|---|67| **Neonate** | **~25 mg IV bolus** | **5–6 mg IV every 6 h** |68| Infant / child | **50–100 mg/m² IV bolus** | **50–100 mg/m²/day** divided **q6h** (4 doses) |69| Pragmatic age-based fallback | Infants 25 mg, children 50 mg, adolescents 100 mg | Same maintenance rule |7071- **Hydrocortisone provides both glucocorticoid AND sufficient mineralocorticoid activity** at these stress doses — no separate fludrocortisone needed while IV HC is running.72- Continue IV until the child is tolerating oral intake; then step down to oral HC at 3–5× maintenance, then taper back to baseline over 24–48 h once illness resolves.7374---7576## STEP 6 — Check and correct dyselectrolytaemia7778- Serum **Na, K, glucose, urea, creatinine, venous blood gas**.79- Typical pattern in SW-CAH crisis: **hyponatraemia, hyperkalaemia, metabolic acidosis, hypoglycaemia**.80- Hyperkalaemia: ECG, calcium gluconate / insulin-dextrose / salbutamol as per standard paediatric protocols.81- Avoid sodium bicarbonate unless severe acidosis (pH < 7.1) — risk of worsening hypokalaemia later.8283---8485## STEP 7 — Monitor8687- Continuous vitals, hourly urine output, sensorium.88- Repeat electrolytes and glucose **2–4 hourly** until stable.89- Watch for fluid overload, especially in neonates.9091---9293## STEP 8 — Step-down and discharge9495- Once vomiting stops and the child is tolerating fluids → switch to oral HC at **3–5× maintenance** dose.96- Once afebrile and clinically well → taper to baseline maintenance over 24–48 h.97- **Resume fludrocortisone** when back on oral HC at maintenance.98- Before discharge: confirm parents understand sick-day rules, replenish any IM emergency hydrocortisone supply, re-issue the disease card if missing.99100---101102## STEP 9 — Investigate the trigger103104- Septic screen (urine, blood culture if febrile).105- Stool studies if diarrhoea.106- Review compliance — missed doses, expired stock, wrong preparation (pre-dissolved liquid).107- Review fludrocortisone dose — overdose can precipitate hypokalaemia and crises in some patients.108109---110111## Quick reference card112113- **Suspect crisis →** treat empirically, do not wait for labs.114- **Fluids:** NS 20 mL/kg bolus, up to 60 mL/kg if shocked.115- **Hydrocortisone IV bolus:**116 - Neonate ≈ **25 mg**117 - Infant / child **50–100 mg/m²**118- **Maintenance:** 50–100 mg/m²/day IV q6h; neonate ≈ 5–6 mg q6h.119- **Glucose:** 10% dextrose 5 mL/kg if hypoglycaemic.120- **Withhold** fludrocortisone while on stress HC.121- **Home:** double for minor illness, 3–5× for moderate–severe; never stop steroids.122123---124125## Related skills126127- [[cah-infant-hydrocortisone-dosing]] — chronic maintenance dosing.128- [[cah-fludrocortisone-salt-supplementation]] — mineralocorticoid management.129- [[cah-sick-day-rules]] — parent-facing version of Step 2.
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