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
...iagnosis...
Who Should Be Tested...
...rine Society (ES) recommends diagno...
...recommends confirmatory testing with the corti...
...mal Diagnostic Tests...
...suggests the standard dose (250 μg for...
...sts the low-dose (1 μg) corticotropin tes...
...corticotropin stimulation test is...
...mmends measurement of plasma ACTH to establish PAI...
ES recommends the simultaneous measur...
...that the etiology of PAI should be...
...al Features of Adrenal Insufficiency and Adrenal...
...re 1. Diagnostic approach to the pat...
...r Etiologies of PAI and Associated FeaturesHav...
Treatment
...eatmen...
...Primary Adrenal Insufficiency in Adults...
...with severe adrenal insufficiency symptoms...
...corticoid Replacement Regimen...
ES recommends glucocorticoid therapy in all...
...ggests using hydrocortisone (15–25 mg) or...
...an alternative to hydrocortisone, ES suggests usin...
...nst using dexamethasone for the tr...
...s monitoring glucocorticoid replacement u...
...sts against hormonal monitoring of...
...ocorticoid Replacement in PAI...
...ommends that all patients with confirmed al...
...recommends monitoring mineralocorticoi...
...patients who develop hypertension while rece...
...ssure remains uncontrolled, ES suggests...
...epiandrosterone Replacement...
...gests a trial of dehydroepiandrosterone (DHEA) r...
...ts an initial period of 6 months of DHEA replace...
...itoring DHEA replacement by measuring...
...reatment During Pregnan...
...t pregnant patients with PAI be monitored...
...gests that, based on the individua...
...pregnant women with PAI, ES suggests using hy...
...nd recommends against using dexamethasone bec...
...ends hydrocortisone stress dosing during...
...Monitoring During Childhood...
...ildren with PAI, ES suggests treatment...
...children with PAI, ES suggests avoiding sy...
...monitoring glucocorticoid replacement b...
...PAI and confirmed aldosterone deficien...
...agement and Prevention of Adrenal Crisis in Pati...
...ends that patients with suspected adrenal cri...
If hydrocortisone is unavailable, ES suggests pre...
...tion of adrenal crisis, ES suggests...
...gests patient education concerning g...
...nds that all patients should be equipped w...
ES recommends that every patient should b...
...nal Monitoring Require...
...ests that adults and children with PAI...
ES suggests that PAI patients be evaluated ann...
...periodic screening for autoimmune diseas...
...ts patient education about increasing the do...
...ests genetic counseling for patients...
...anagement of PAI in Specific SituationsHavin...
...Measures for Prevention of Adrenal...