Primary Adrenal Insufficiency
Key Points
Key Points
PAI is defined by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids.
PAI is a severe and potentially life-threatening condition due to the central role of these hormones in energy, salt, and fluid homeostasis.
Cortisol deficiency results in a decrease in feedback to the hypothalamic-pituitary axis and subsequent enhanced stimulation of the adrenal cortex by elevated levels of plasma ACTH. Consequent to disruption of adrenal mineralocorticoid synthesis, renin release by the juxtaglomerular cells of the kidneys increases. This is of clinical, diagnostic, and therapeutic relevance because PAI needs to be distinguished from secondary adrenocortical insufficiency due to insufficient production of ACTH and without impact on the renin-angiotensin-aldosterone system.
Diagnosis
Diagnosis
...ld Be Tested and How...
...e Society (ES) recommends diagnostic testing...
...mends confirmatory testing with th...
...timal Diagnostic Tes...
...the standard dose (250 μg for adults and chil...
...s the low-dose (1 μg) corticotropin test f...
...cotropin stimulation test is not feasible,...
...ends measurement of plasma ACTH to esta...
...ds the simultaneous measurement of plasma...
...hat the etiology of PAI should be determi...
.... Clinical Features of Adrenal Insufficiency...
...re 1. Diagnostic approach to the pati...
...Major Etiologies of PAI and Associated FeaturesHa...
Treatment
...reatmen...
...Primary Adrenal Insufficiency in Adul...
...tients with severe adrenal insufficiency sympt...
...rticoid Replacement Regime...
...recommends glucocorticoid therapy in all p...
...s using hydrocortisone (15–25 mg) or...
...e to hydrocortisone, ES suggests usi...
...uggests against using dexamethason...
...monitoring glucocorticoid replacement using clini...
...ts against hormonal monitoring of glucocorticoid...
...icoid Replacement in PAI...
...hat all patients with confirmed aldosterone d...
...ecommends monitoring mineralocortic...
...develop hypertension while receiving flu...
...od pressure remains uncontrolled, ES su...
Dehydroepiandrosterone Replac...
...sts a trial of dehydroepiandrosterone (DH...
...nitial period of 6 months of DHEA replacement. I...
...nitoring DHEA replacement by measuring m...
...t During Pregnancy...
...gests that pregnant patients with PAI be monitore...
...t, based on the individual clinical course...
...omen with PAI, ES suggests using hydroc...
...nd recommends against using dexamethasone because...
...commends hydrocortisone stress dosing during t...
...eatment and Monitoring During Childho...
...PAI, ES suggests treatment with hydroco...
...dren with PAI, ES suggests avoiding synt...
...gests monitoring glucocorticoid re...
...h PAI and confirmed aldosterone def...
...and Prevention of Adrenal Crisis in Patie...
ES recommends that patients with suspected adrenal...
...cortisone is unavailable, ES suggests pre...
...vention of adrenal crisis, ES suggests ad...
...s patient education concerning glucocort...
...ds that all patients should be equipped with a...
...commends that every patient should...
...ditional Monitoring Requirem...
...suggests that adults and children with P...
...t PAI patients be evaluated annually for sym...
...sts periodic screening for autoimmune diseases...
...ts patient education about increasing the...
...sts genetic counseling for patients with...
...Management of PAI in Specific SituationsHav...
...asures for Prevention of Adrenal CrisisHavi...