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.
Highlights
Primary adrenal insufficiency (PAI), first described by Thomas Addison in 1855, is characterized by the inability of the adrenal cortex to produce enough glucocorticoids and/or mineralocorticoids
The prevalence of PAI is around 82-144/million and the most common causes are autoimmunity (Addison’s disease [AD]) in adults and genetic causes in children, especially enzymatic defects [1]
Infection triggers the release of cytokines such as interleukin 1 (IL-1), tumor necrosis factor α (TNFα), and interleukin 6 (IL-6), which stimulate the hypothalamus-pituitary-adrenal (HPA) axis to increase the cortisol levels
Summary
Primary adrenal insufficiency (PAI), first described by Thomas Addison in 1855, is characterized by the inability of the adrenal cortex to produce enough glucocorticoids and/or mineralocorticoids. Further studies among hypopituitarism patients demonstrated excessively high mortality, possibly due to inadequate glucocorticoid replacement therapy These data stimulated additional studies about mortality rate due to adrenal insufficiency [5]. Prior to the introduction of newborn screening (NBS) programs for CAH, the neonatal mortality was higher, especially among SW males This increased mortality was suggested by both the low proportion of the SW form in relation to the SV form and the low male-to-female ratio in the Hospital das Clínicas cohort, as well as in other unscreened populations [9,10,11]. Three retrospective studies comprising 1,191 English and Swedish CAH patients evidenced increased mortality in all age groups, varying from 2- to 5-fold in comparison with the general population [15,16,17] In all these studies on mortality in adrenal insufficiency, the main causes of death were cardiovascular disease, AC, infections, and cancer. These data highlight the necessity of continuous education of patients, relatives, caregivers, and physicians, emphasizing the importance of stress hydrocortisone doses during adverse events [23]
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