Primary adrenal insufficiency
Primary adrenal insufficiency
The cause of primary adrenal insufficiency, better known as Addison's disease, lies at the level of the adrenal gland itself. Addison's disease is caused by destruction of the adrenal cortex. This is mostly due to autoimmune destruction. Tuberculosis or adrenal haemorrhage can also destroy the adrenal cortex. Adrenal insufficiency involves both the zona fasciculata (glucocorticoids) and the zona glomerulosa (mineralocorticoids). Symptoms that can occur are hypotension, loss of appetite, hypovolemia, and low blood glucose levels. In addition,
hyponatriemia and hyperkalemia are due to hypoaldosteronism. Both glucocorticoids and mineralocorticoids are needed to control the symptoms.
An enzymatic disorder can also be the underlying cause of primary adrenal insufficiency. The cortisol synthesis in the adrenal gland involves many different hormones. In case of a (genetic) dysfunction of one of the enzymes, the cortisol production can be completely inhibited. On the other hand, the metabolic product prior to this non-functional conversion will be highly elevated.
I. Mutation of the ACTH receptor is a cause of primary adrenal insufficiency. II. Primary adrenal insufficiency is a cause of decreased sex hormones.
Extra info: Mutation of the ACTH receptor is a rare cause of adrenal insufficiency, which makes it impossible for ACTH to bind the receptor in the adrenal gland. The zona reticularis of the adrenal gland produces small (neglectable) amounts of androgens. The decreased production of androgens as a result of primary adrenal insufficiency results in minimally decreased sex steroid concentrations in the plasma in women. In men, the testes have much higher testosterone production capacity.
