Cah Adrenal Crisis Protocol

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name: cah-adrenal-crisis-protocol
description: Bedside protocol for recognising and managing adrenal crisis in an infant, child, or adolescent with classical congenital adrenal hyperplasia (CAH) — including triage clues, fluid resuscitation, IV hydrocortisone bolus and maintenance dosing (age-stratified), glucose and electrolyte correction, and stepped-down disposition. Also covers home sick-day rules (minor vs moderate–severe illness) and the disease-card instructions parents should follow before reaching hospital. Use when a clinician asks "is this an adrenal crisis", "how to manage adrenal crisis in CAH", "stress dose steroids for a sick CAH child", "IV hydrocortisone bolus dose neonate", "double dose hydrocortisone sick day", "vomiting child with CAH what to do", or any acute decompensation in a known CAH patient. Source: Dabas et al., Indian Pediatrics 2020;57:159–164 (Box I and Web Box I).
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# 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.

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## 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.

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## 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.

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## STEP 3 — Resuscitate (A → B → C)

1. **Airway / Breathing** — oxygen if hypoxic; intubate if obtunded.
2. **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.
3. **Newborns:** continue maintenance at **1.5–2× normal** with **half-normal saline in 5% dextrose** (0.45% NaCl + D5W).

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## STEP 4 — Correct hypoglycaemia

- Check capillary glucose immediately.
- If low: **10% dextrose 5 mL/kg IV** bolus. Recheck in 15 min.

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## 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.

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## 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.

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## 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.

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## 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.

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## 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.

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## 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.

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## 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.