This document discusses adrenal crisis and congenital adrenal hyperplasia. It presents four case studies: 1. A 10-day-old neonate with ambiguous genitalia and symptoms of adrenal insufficiency was diagnosed with congenital adrenal hyperplasia. 2. A 2-year-old boy with a history of URI presented with lethargy and hypotension and was found to have metabolic acidosis, suggesting adrenal crisis. 3. A 1-year-old child with fever, cough and cold for 3 days developed septic shock that did not respond to fluids and vasopressors, but did respond to stress-dose hydrocortisone, indicating

1. ADRENAL CRISIS
Dr. Zaheen Zehra.N
1st year Resident
Dept of Paediatrics.

3. CASE- 1
• 10 days old neonate was brought to the
hospital with complaints of not accepting
feeds well, vomiting and decreased activity.
On examination, the peripheries were cold,
lethargic, oral cavity was dry and skin turgor
was prolonged. Baby also had ambiguous
genitalia. Blood investigations showed
Hyponatremia, hyperkalemia and metabolic
acidosis.

4. Congenital Adrenal Hyperplasia
• More than 90% of CAH cases are caused due
to 21-Hydroxylase deficiency.
• Defective conversion of 17-
hydroxyprogesterone to cortisol due to 21-
hydroxylase deficiency.
• Defect in production of Cortisol and
aldosterone (requires 21-hydroxylation)

8. Clinical features
• Classic CAH:
1. “Salt wasting” form.
2. “Simple virilizing disease”
3. Genital Ambiguity.
4. Failure to thrive.
• Non classical CAH:
1. Late onset 12-Hydroxylase deficiency.
2. Premature pubarche.
3. Accelerated bone age.
4. Hirusitism, Infertility, Acne, Irregular
Periods.

9. Investigations
• Elevated serum concentration of 17-
hydroxyprogesterone ( >3500ng/dL in a
neonate. Normal- 1200ng/dL)
• Hyponatremia.
• Hyperkalemia.
• Hypoglycemia.
• High serum conc of Aldostenedione,
testosterone. 21-deoxycortisol and
progesterone.
• USG -KUB: Adrenal limb width >4mm and
Lobulated surface.

10. Treatment
• Therapy is directed towards providing
glucocorticoid in sufficient doses to reduce the
associated excessive CRH and ACTH secretion
and hyperandrogenemia.
• Hydrocortisone(glucocorticoid replacement):
15 to 20mg/m2/day divided thrice a day
• Fludrocortisone(mineralocorticoid
replacement): 0.1-0.3mg twice daily.
• Sodium chloride: one gram or 4mEq/kg/day.

11. Other Causes of CAH
• 11 beta-Hydroxylase deficiency.
• 3 beta- Hyroxylase deficiency.
• 17- Hydroxylase deficiency.
• Lipoid Adrenal Hyperplasia.
• Deficiency of P450 Oxidoreductase.

12. Case 2
• 2 years old boy was presented with fast
breathing and lethargy. He has history of URI.
Developmentaly normal child. On
examination, child has hyperpigmentation,
Hypotension. Blood gas shows Metabolic
acidosis.

13. Etiology
• Inherited Etiology:
1. Inborn defects of Steroidogenesis.
2. Adrenal Hypoplasia Congenita.
3. Adrenoleukoystrophy.
4. Familial Glucocorticoid Deficiency.
5. Disorders of Cholestrol synthesis and
metabolism.

14. Etiology
• Acquired Etiology:
1. Infection: Tuberculosis, Meningococcemia.
2. Drugs: Ketoconazole, Etomidate.
Drugs such as Rifampicin,
Phenobarbitone and Phenytoin reduce
effectiveness and bioavailability of
corticosteroid replacement therapy.
3. Hemorrhage into Adrenal glands.

15. Clinical Manifestations
• Symptoms:
Fatigue.
Anoxeria, Weight loss
Nausea, Vomiting
Salt craving
Myalgia or Joint Pain.
• Signs:
Low blood pressure.
Skin or mucosal hyperpigmentation

16. Lab Findings
• Hyponatremia.
• Hyperkalemia.
• Hypoglycemia.
• Ketosis.
• Low Random cortisol level.
• Eosinophilia, Lymphocytosis.
• High ACTH level.
• High plasma renin activity.

17. Treatment
• Immediate and Vigorous.
• Initial fluid resuscitation: IVF of 5% glucose in
0.9% saline should be administered to correct
hypoglycemia, hyponatremia & hypovolemia.
• Hyperkalemia correction.
• Inj. Hydrocortisone (water soluble form) IV
bolus and a similar total amount divided in 6
hour intervals for 1st 24 hours.
• Hypothyroidism should not be treated first.

18. Treatment
• After acute manifestations are under control,
most patients require chronic replacement
therapy for their Cortisol & Aldosterone
deficiencies.
• Hydrocortisone P/O 10mg/m2/24 hr in 3
divided doses.
• Equivalent doses of Prednisone or
Prednisolone may be given.

19. Treatment
• ACTH levels may be used to monitor adequacy
of glucocorticoid replacement therapy.
• If Aldosterone deficiency is present,
Fludrocortisone P/O 0.05-0.2 mg daily is given.

20. Case 3
• 1 year old child was brought with c/o fever,
cough and cold for 3 days, lethargy, poor
feeding, fast breathing. Child on examination
showed Tachycardia, Prolonged CFT,
Hypotension and vomiting. Child was
managed as septic shock. Despite giving fluid
boluses and inotropes/vasopressors, Blood
pressure was low and features of shock
persisted.

21. Septic shock secondary to Adrenal
insufficiency
• Initial Treatment: Hydrocortisone infusion
given at stress doses(50mg/m2/24hr)
• Upto 50mg/kg/day of hydrocortisone can be
used to reverse shock.
• Death from absolute adrenal insufficiency and
septic shock within 8 hours.
• Obtain serum cortisol level at the time of
administration of hydrocortisone

22. Case 4
• 4 years old is a known case of steroid resistant
nephrotic syndrome, presented with severe
abdominal pain. Diagnosed as Bacterial
peritonitis. What is the stress dose of
hydrocortisone given in this child?

23. • Stress dose of hydrocortisone:
1. Minor illness: 50mg/m2/day
2. Major illness: 100mg/m2/day

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