Addison's disease (also known as chronic adrenal insufficiency, hypocortisolism or hypocorticism) is a rare endocrine disorder in which the adrenal gland produces insufficient amounts of steroid hormones (glucocorticoids and often mineralocorticoids). It may develop in children as well as adults, and may occur as the result of a large number of underlying causes. The condition is named after Dr Thomas Addison, the British physician who first described the condition in his 1855 On the Constitutional and Local Effects of Disease of the Suprarenal Capsules. The adjective "Addisonian" is used for features of the condition, as well as patients with Addison's disease.
The condition is generally diagnosed with blood tests, medical imaging and additional investigations. Treatment is with replacement of the hormones (oral hydrocortisone and fludrocortisone). If the disease is caused by an underlying problem, this is addressed. Regular follow-up and monitoring for other health problems is necessary.
Causes:
Causes of adrenal insufficiency can be grouped by the way in which they cause the adrenals to produce insufficient cortisol.
Adrenal dysgenesis-All causes in this category are genetic, and generally very rare. These include mutations to the SF1 transcription factor, congenital adrenal hypoplasia (AHC) due to DAX-1 gene mutations and mutations to the ACTH receptor gene.
Impaired steroidogenesis-To form cortisol, the adrenal gland requires cholesterol, which is then converted biochemically into steroid hormones. Interruptions in the delivery of cholesterol include Smith-Lemli-Opitz syndrome and abetalipoproteinemia.
Adrenal destruction:Autoimmune destruction of the adrenal cortex (often due to antibodies against the enzyme 21-Hydroxylase) is a common cause of Addison's in teenagers and adults.
Treatment:
Treatment for Addison's disease involves replacing the missing cortisol (usually in the form of hydrocortisone tablets) in a dosing regimen that mimics the physiological concentrations of cortisol.
Treatment for an acute attack, an Addisonian crisis, usually involves intravenous (into blood veins) injections of Cortisone (cortisol), Saline solution (basically a salt water, same clear IV bag as used to treat dehydration) and Glucose.
Surgeries may require significant adjustments to medication regimens prior to, during, and following any surgical procedure. The best preparation for any surgery, regardless of how minor or routine it may normally be, is to speak to one's primary physician about the procedure and medication implications well in advance of the surgery.
The condition is generally diagnosed with blood tests, medical imaging and additional investigations. Treatment is with replacement of the hormones (oral hydrocortisone and fludrocortisone). If the disease is caused by an underlying problem, this is addressed. Regular follow-up and monitoring for other health problems is necessary.
Causes:
Causes of adrenal insufficiency can be grouped by the way in which they cause the adrenals to produce insufficient cortisol.
Adrenal dysgenesis-All causes in this category are genetic, and generally very rare. These include mutations to the SF1 transcription factor, congenital adrenal hypoplasia (AHC) due to DAX-1 gene mutations and mutations to the ACTH receptor gene.
Impaired steroidogenesis-To form cortisol, the adrenal gland requires cholesterol, which is then converted biochemically into steroid hormones. Interruptions in the delivery of cholesterol include Smith-Lemli-Opitz syndrome and abetalipoproteinemia.
Adrenal destruction:Autoimmune destruction of the adrenal cortex (often due to antibodies against the enzyme 21-Hydroxylase) is a common cause of Addison's in teenagers and adults.
Treatment:
Treatment for Addison's disease involves replacing the missing cortisol (usually in the form of hydrocortisone tablets) in a dosing regimen that mimics the physiological concentrations of cortisol.
Treatment for an acute attack, an Addisonian crisis, usually involves intravenous (into blood veins) injections of Cortisone (cortisol), Saline solution (basically a salt water, same clear IV bag as used to treat dehydration) and Glucose.
Surgeries may require significant adjustments to medication regimens prior to, during, and following any surgical procedure. The best preparation for any surgery, regardless of how minor or routine it may normally be, is to speak to one's primary physician about the procedure and medication implications well in advance of the surgery.
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