Overview
Definition:
Adrenal crisis (Addisonian crisis) is a life-threatening condition characterized by an acute, severe deficiency of glucocorticoids and sometimes mineralocorticoids, leading to circulatory collapse, shock, and multi-organ dysfunction
In pediatrics, it is often the initial presentation of primary adrenal insufficiency or occurs in children with known adrenal disorders during periods of stress.
Epidemiology:
Primary adrenal insufficiency occurs in approximately 1-2 per 100,000 live births
Congenital adrenal hyperplasia (CAH) is the most common cause of primary adrenal insufficiency in neonates and infants, particularly the salt-wasting forms
Autoimmune adrenalitis is more common in older children
The incidence of adrenal crisis is difficult to quantify but is a significant complication of untreated or inadequately treated adrenal insufficiency.
Clinical Significance:
Prompt recognition and aggressive management of adrenal crisis are critical for survival in pediatric patients
It is a medical emergency requiring immediate intervention with glucocorticoid replacement and supportive care
Understanding the pediatric-specific nuances of presentation, diagnosis, and management, including age-appropriate dosing, is vital for residents preparing for DNB and NEET SS examinations, as this is a frequently tested topic with significant clinical implications.
Clinical Presentation
Symptoms:
Sudden onset of severe weakness
Profound lethargy and somnolence
Vomiting and diarrhea
Abdominal pain, often severe and diffuse
Fever
Hypotension and shock
Hypoglycemia
Irritability or confusion
In infants, poor feeding, failure to thrive, and dehydration are common
Cyanosis may be present in severe cases.
Signs:
Profound hypotension, often unresponsive to fluid resuscitation alone
Tachycardia
Poor peripheral perfusion
Cool, clammy skin
Signs of dehydration
Hyperpigmentation of skin and mucous membranes (more common in chronic insufficiency, less so in acute crisis unless it is the first presentation)
Hypotonia
Seizures (due to hypoglycemia or hyponatremia)
Possible signs of underlying cause (e.g., congenital anomalies in CAH).
Diagnostic Criteria:
Diagnosis is primarily clinical, supported by laboratory findings
A high index of suspicion is essential
Definitive diagnosis is made by demonstrating deficient cortisol production and a lack of response to ACTH stimulation, although this test is typically deferred until the patient is stable
Electrolyte abnormalities (hyponatremia, hyperkalemia) are hallmark laboratory findings
Hypoglycemia is also common.
Diagnostic Approach
History Taking:
Inquire about history of adrenal insufficiency, congenital adrenal hyperplasia, autoimmune disorders, or endocrine conditions
Ask about recent illness, surgery, trauma, or any stressor
Family history of adrenal disorders
Medication history, especially steroid use (to rule out secondary adrenal insufficiency).
Physical Examination:
Assess vital signs carefully, noting hypotension, tachycardia, and signs of shock
Evaluate hydration status
Examine for hyperpigmentation
Perform a thorough abdominal exam for tenderness
Assess neurological status for lethargy, confusion, or seizures
Look for any dysmorphic features suggestive of genetic syndromes.
Investigations:
Immediate bedside glucose monitoring
Serum electrolytes (sodium, potassium, chloride, bicarbonate)
Serum cortisol (random, typically low)
Serum ACTH (may be elevated in primary insufficiency, low/normal in secondary)
Renin and aldosterone levels (to assess mineralocorticoid status, if indicated)
Blood gas analysis (for acid-base status)
Complete blood count (may show mild anemia or eosinophilia)
Imaging: Adrenal ultrasound or CT scan if adrenal hemorrhage or mass is suspected
Cosyntropin stimulation test (250 mcg IV) to assess ACTH reserve: measure baseline cortisol and cortisol 30-60 minutes after injection
A peak cortisol < 18-20 mcg/dL (500-550 nmol/L) indicates adrenal insufficiency
Interpretation: Low cortisol with high ACTH suggests primary adrenal failure
low cortisol with low/normal ACTH suggests secondary adrenal failure.
Differential Diagnosis:
Septic shock
Hypovolemic shock
Diabetic ketoacidosis
Congenital adrenal hyperplasia (initial presentation)
Inborn errors of metabolism
Sepsis with adrenal involvement
Hypoglycemia of other causes
Gastroenteritis with severe dehydration.
Management
Initial Management:
Immediate fluid resuscitation with isotonic saline (e.g., 0.9% NaCl) at 20 mL/kg bolus, repeated as needed
Glucose administration (e.g., D10W) if hypoglycemia is present
Initiate intravenous hydrocortisone therapy immediately, without waiting for diagnostic test results
Address any precipitating factors.
Medical Management:
Intravenous Hydrocortisone: For acute crisis, administer hydrocortisone 1-2 mg/kg IV/IM every 6-8 hours
Once stable, can switch to oral hydrocortisone
Pediatric Dosing of Hydrocortisone: Infants and children: 1-2 mg/kg IV/IM every 6-8 hours
Maximum dose is typically 100 mg per dose
Adolescents: 100 mg IV/IM as a bolus, followed by continuous infusion of 200 mg/24 hours, or 50-100 mg IV/IM every 6-8 hours
Transition to oral therapy: Once hemodynamically stable and tolerating oral intake, switch to oral hydrocortisone
Typical maintenance dose for children is 4-7 mg/kg/day divided into 2-3 doses, with the largest dose in the morning
Stress dosing: During illness or stress, oral hydrocortisone doses need to be increased (e.g., 3-5 times maintenance dose, or 10-15 mg/kg/day, divided q4-6h)
Mineralocorticoid Replacement: For primary adrenal insufficiency (e.g., Addison's disease, CAH), fludrocortisone is usually required
Dose: 0.05-0.1 mg orally once daily
Monitor electrolytes and blood pressure
Adjust dose as needed
Sodium bicarbonate may be used to treat severe metabolic acidosis if present.
Surgical Management:
Generally not applicable, as adrenal crisis is a medical emergency
Surgical intervention may be considered for complications like adrenal hemorrhage with retroperitoneal hematoma, though this is rare.
Supportive Care:
Continuous monitoring of vital signs, fluid balance, and glucose levels
Monitor electrolytes and acid-base status closely
Mechanical ventilation may be required if respiratory compromise occurs
Strict intake and output monitoring
Provide adequate nutritional support once tolerated
Education for parents/guardians on stress dosing, sick day rules, and emergency preparedness is paramount.
Complications
Early Complications:
Circulatory collapse and shock
Hypoglycemic seizures
Hyponatremic or hyperkalemic cardiac arrhythmias
Acute kidney injury
Sepsis due to immunocompromise or poor perfusion
Disseminated intravascular coagulation (DIC).
Late Complications:
Recurrent adrenal crises if adherence to medication and stress dosing is poor
Growth failure in children with inadequately treated adrenal insufficiency
Osteoporosis
Cardiovascular disease
Psychiatric disturbances.
Prevention Strategies:
Meticulous adherence to prescribed glucocorticoid and mineralocorticoid replacement therapy
Comprehensive education of patients and caregivers on sick day rules, stress dosing protocols, and recognizing early signs of crisis
Provision of emergency hydrocortisone injection kits (IM/IV) and instruction on their use
Regular follow-up with pediatric endocrinologist
Early recognition and management of precipitating factors like infections or stress.
Prognosis
Factors Affecting Prognosis:
Timeliness of diagnosis and initiation of treatment
Severity of the crisis
Presence of precipitating factors and their management
Underlying cause of adrenal insufficiency
Patient's overall health status and presence of comorbidities
Adherence to long-term therapy and patient/caregiver education.
Outcomes:
With prompt and aggressive management, most children can recover from adrenal crisis
However, it remains a life-threatening condition, and mortality can occur if treatment is delayed or inadequate
Long-term outcomes depend on consistent management of adrenal insufficiency and prevention of future crises.
Follow Up:
Regular endocrinology follow-up is essential for dose adjustments, monitoring growth and development, and reinforcing education
Patients and families should have a clear emergency plan and access to medical advice at all times
Monitoring for potential complications of long-term steroid therapy is also necessary.
Key Points
Exam Focus:
Emergency treatment of pediatric adrenal crisis requires immediate IV hydrocortisone (1-2 mg/kg/dose q6-8h) and aggressive fluid resuscitation
Differentiate primary vs
secondary adrenal insufficiency based on ACTH levels
Stress dosing is crucial for preventing crises during illness.
Clinical Pearls:
Always consider adrenal crisis in any child presenting with shock, severe dehydration, vomiting, and abdominal pain, especially if there are electrolyte abnormalities
Never withhold hydrocortisone while awaiting diagnostic tests in a suspected crisis
Educate families on always carrying emergency hydrocortisone.
Common Mistakes:
Delaying hydrocortisone administration
Underestimating fluid and glucose requirements
Inadequate stress dosing during illness
Misinterpreting electrolyte patterns without considering the clinical context
Forgetting mineralocorticoid replacement in primary adrenal insufficiency.