ADRENAL CRISIS

Dr. Zaheen Zehra.N1st year Resident

Dept of Paediatrics.

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.

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)

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.

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.

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.

Other Causes of CAH

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

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.

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.

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.

Clinical Manifestations• Symptoms:Fatigue.Anoxeria, Weight lossNausea, VomitingSalt cravingMyalgia or Joint Pain.• Signs:Low blood pressure.Skin or mucosal hyperpigmentation

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

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.

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.

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.

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.

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

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?

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

THANK YOU