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

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.

Diagnosis and Management of Adrenal Crisis in 46XX Congenital Adrenal Hyperplasia Infant

“…Hyperkalemia of more than 6 mmol/L can induce fatal cardiac arrhythmias and requires aggressive therapy with intravenous sodium bicarbonate, calcium gluconate, or IV dextrose plus insulin (Parham et al 2006, Miller et al 2008 Education is very important in the management of adrenal insufficiency and the prevention of AC. Patients and families should be informed about CAH, general CAH management, and acute complications which should be taken at home to prevent an adrenal crisis, particularly related to glucocorticoids adjustments in stressful events and AC prevention including administration of emergency glucocorticoid particularly in illness, fever, and any type of stress (Lousada et al 2021). Furthermore, the general practitioners, pediatricians, nurses, and health workers must be aware of these cases.…”

Impact of Newborn Screening on Clinical Presentation of Congenital Adrenal Hyperplasia

“…Studies in Japan before the NBS and in the UK showed that up to 20% of patients with the SW form of 21OHD developed learning difficulties and intellectual disabilities [16,22,23]. The risk of developing severe salt-wasting increases during the second week of life, and, therefore, an early diagnosis and intervention, preferably during the first week after birth, is of great importance [24,25].…”

Endocrine Emergencies in the Medical Intensive Care Unit

“…Cortisol has a half-life of 90 minutes; hence, the adrenal crisis presents within a few hours of cortisol deprivation. As cortisol has multiorgan effects thanks to its pleiotropic nature, the various consequences of its deficiency as explained physiologically are listed below33,34,39: Cortisol has anti-inflammatory effects via suppression of production of various cytokines; hence, adrenal insufficiency results in a rapid surge in cytokine levels, which presents as fevers, myalgia, and loss of appetite. Cortisol promotes demarginalization of neutrophils from endothelial cells into the circulation and promotes apoptosis of eosinophils and lymphocytes, so a cortisol-deficient state leads to neutropenia, eosinophilia, and lymphocytosis. Cortisol is a requisite of the synergistic action of catecholamines; its absence causes vasodilation and hypotension. Cortisol is a stress hormone and in a fight or flight response it amplifies production of glucose, free fatty acids, and amino acids; hence, in adrenal crisis hypoglycemia and reduced free fatty acids are noted. In primary adrenal insufficiency, loss of aldosterone function further exacerbates hypotension, as the body cannot retain sodium and water. …”