Adrenal crisis and mortality rate in adrenal insufficiency and congenital adrenal hyperplasia

Lousada, Lia Mesquita; Mendonca, Berenice B.; Bachega, Tania A. S. S.

doi:10.20945/2359-3997000000392

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Archives of Endocrinology and Metabolism

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Review • Arch. Endocrinol. Metab. 65 (4) • 2021 • https://doi.org/10.20945/2359-3997000000392 copy

Adrenal crisis and mortality rate in adrenal insufficiency and congenital adrenal hyperplasia

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Primary adrenal insufficiency (PAI) is characterized by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. Addison's disease (AD) and congenital adrenal hyperplasia (CAH) are the most frequent disorders in adults and children, respectively. Despite the diagnostic advances and the availability of glucocorticoid and mineralocorticoid replacements, adrenal crisis (AC) is still a potentially lethal condition contributing to the increased mortality, not only during the first year of life, but also throughout life. Failure in increasing glucocorticoid doses during acute stress, when greater amounts of glucocorticoids are required, can lead to AC and an increase morbimortality rate of PAI. Considering a mortality rate of 0.5 per 100 patient years, up to 1,500 deaths from AC are expected in Brazil in the coming decade, which represents an alarming situation. The major clinical features are hypotension and volume depletion. Nonspecific symptoms such as fatigue, lack of energy, anorexia, nausea, vomiting, and abdominal pain are common. The main precipitating factors are gastrointestinal diseases, other infectious disease, stressful events (e.g., major pain, surgery, strenuous physical activity, heat, and pregnancy), and withdrawal of glucocorticoid therapy. Suspected AC requires immediate therapeutic action with intravenous (iv) hydrocortisone, fluid infusion, monitoring support, and antibiotics if necessary. AC is best prevented through patient education, precocious identification and by adjusting the glucocorticoid dosage in stressor situations. The emergency card, warning about acute glucocorticoid replacement, has high value in reducing the morbidity and mortality of AC.

Keywords
Primary adrenal insufficiency; congenital adrenal hyperplasia; mortality; adrenal crisis; emergency care

Topic	Treatment
1	Volume expansion: 1,000 mL iv isotonic saline within the first hour. Children: 20 mL/kg isotonic saline. Can repeat up to a total of 60 mL/kg within 1 h for shock.
2	100 mg iv hydrocortisone followed by 200 mg/d hydrocortisone as a continuous infusion for 24 h or every 6 h, reduced to 100 mg/d hydrocortisone the following day. Children: 50-100 mg/m² iv hydrocortisone bolus followed by 50-75-100 mg/m²/d hydrocortisone every 6 h or 2-3 mg/m²/h as a continuous infusion for 24 h. *Alternative simpler scheme of bolus stress doses of hydrocortisone: children < 15 kg (0-2 years), 25 mg; 15-25 kg (2-6 years), 50 mg; and > 25 kg (>6 years), 100 mg.
3	For hypoglycemia: 0.5-1 g/kg dextrose or 2-4 mL/kg of 25% dextrose solution (maximum single dose 25 g) infused slowly at a rate of 2 to 3 mL/min. Alternatively, 5-10 mL/kg of 10% dextrose solution for children < 12 years old
4	Cardiac and hemodynamic monitoring. Low heparin doses. Antibiotics if necessary.
5	Switch to oral regimen depending on clinical state.

Clinical Condition	Treatment
Fever and illness - home management	Hydrocortisone replacement doses doubled (>38 °C) or tripled (>39 °C) until recovery and increased consumption of electrolyte-containing fluids as tolerated
Oral intake prevented by vomiting or trauma	Adults: 100 mg IM or SC hydrocortisone (children: 50 mg/m²)
Minor to moderate surgical stress	Doubled or tripled hydrocortisone oral doses or 25-75 mg/24 h iv hydrocortisone (usually 1 to 2 d) in adults (50 mg/m² in children)
Major surgery with general anesthesia, trauma, delivery, or disease that requires intensive care	100 mg IV hydrocortisone followed by 200 mg/d hydrocortisone as a continuous infusion for 24 h or every 6 h, reduced to 100 mg/d hydrocortisone the following day. Children: 50-100 mg/m² hydrocortisone bolus at the induction of anesthesia, followed by 50-100 mg/m²/d hydrocortisone divided q 6 h. Decrease to 50 mg/m²/d hydrocortisone on the second day and 25 mg/m²/d on the third day.

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E-mail: aem.editorial.office@endocrino.org.br

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[1] Correspondence to: Lia Mesquita Lousada, Laboratório de Hormônios e Genética Molecular (LIM/42) Av. Dr. Enéas de Carvalho Aguiar, 155, 2º andar, bloco 6, Cerqueira César 05403-900 – São Paulo, SP, Brasil.