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Autoimmune polyglandular syndrome type 1 (NORD)
Adrenal insufficiency: Pathology review
Adrenal masses: Pathology review
Cushing syndrome and Cushing disease: Pathology review
Diabetes insipidus and SIADH: Pathology review
Diabetes mellitus: Pathology review
Hyperthyroidism: Pathology review
Hypopituitarism: Pathology review
Hypothyroidism: Pathology review
Multiple endocrine neoplasia: Pathology review
Neuroendocrine tumors of the gastrointestinal system: Pathology review
Parathyroid disorders and calcium imbalance: Pathology review
Pituitary tumors: Pathology review
Thyroid nodules and thyroid cancer: Pathology review
Primary adrenal insufficiency
0 / 10 complete
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metabolic acidosis in p. 616
presentation p. 717
Addison disease p. 355
Addison disease p. 355
Primary adrenal insufficiency, is a rare endocrine disorder that happens when the adrenal gland isn’t able to produce enough of the hormones that the body needs, particularly aldosterone and cortisol. The reason it’s called “primary” is that the underlying problem is localized to the adrenal gland itself, rather than a problem of a hormone that acts on the adrenal gland or elsewhere in the body. Primary adrenal insufficiency can develop acutely or chronically, and a really famous example of someone having this condition is John F. Kennedy, who was diagnosed at age 30.
Now, there are two adrenal glands, one above each kidney, and each one has an inner layer called the medulla and an outer layer called the cortex which is subdivided into three more layers, the zona glomerulosa, zona fasciculata, and the zona reticularis. The outermost layer is the zona glomerulosa, and it’s full of cells that make the hormone aldosterone.
Aldosterone is part of a hormone family or axis which work together and are called the renin-angiotensin-aldosterone system. Together these hormones decrease potassium levels, increase sodium levels, and increase blood volume and blood pressure.
First it stimulates the sodium/potassium ion pumps of the principal cells to work even harder. These pumps drive potassium from the blood into the cells and from there it flows down its concentration gradient into the tubule to be excreted as urine. At the same time, the pumps drive sodium in the opposite direction from the cell into the blood, which allows more sodium to flow from the tubule into the cell down its concentration gradient.
Since water often flows with sodium through a process of osmosis, water also moves into the blood, which increases blood volume and therefore blood pressure.
The other function of aldosterone is to stimulate the proton ATPase pumps in alpha-intercalated cells which causes more protons to get excreted into the urine. Meanwhile, ion exchangers on the basal surface of the cell move the negatively charged bicarbonate into the extracellular space, causing an increase in pH.
Primary adrenal insufficiency, also known as Addison's disease, is a chronic condition in which the adrenal glands fail to produce enough cortisol and mineralocorticoid hormones. This can be caused by an autoimmune disorder, infection, or causes of damage to the adrenal glands. Symptoms may include fatigue, weight loss, muscle weakness, low blood pressure, and darkening of the skin. Treatment involves replacement of cortisol and mineralocorticoid hormones, as well as treating any underlying cause.
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