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Atrophy of the adrenal cortex
3%
6/212
Diffuse hyperplasia of the adrenal cortex
87%
185/212
Atrophy of the adrenal medulla
0%
0/212
Adrenal adenoma
4%
8/212
Atrophy of the adrenal gland
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The patient described above has a pituitary adenoma which, as a result of excess secretion of ACTH, mediates hyperplasia of the adrenal cortex. Cushing syndrome is either due to endogenous or exogenous causes. This patient is suffering from a endogenous ACTH-dependent form where a pituitary adenoma is secreting excess ACTH. Of note, this high ACTH pituitary form is known as Cushing disease, while all other forms are referred to as Cushing syndrome. The effect is increased stimulation of cortical cells (predominantly fasciculata cells) leading to hyperplasia of the cortex. Akcay et al. discuss Cushing disease. They report that ACTH-dependent Cushing's is more common than the non-ACTH dependent type. Low versus high-dose dexamethasone testing is described for the diagnostic workup of Cushing's disease. If cortisol production is not suppressed with low-dose dexamethasone but is with high-dose, the problem is most likely pituitary overproduction of ACTH; however, if neither low or high-dose dexamethasone suppress cortisol production, the problem is most likely not pituitary in origin but rather favors primary adrenal overproduction. Bode et al. discuss the clinical finding of hirsutism in women in which the differential includes medication side-effects, polycystic ovary syndrome, adrenal hyperplasia, thyroid dysfunction, Cushing syndrome, and androgen-secreting tumors. Illustration A shows the classic moon-face profile as well as the buffalo hump seen in Cushing's. Illustration B shows a histological specimen of adrenal cortex hyperplasia. Incorrect Answers: Answer 1: Atrophy of the adrenal cortex is more consistent with exogenous glucocorticoid administration. Answer 3: Atrophy of the adrenal medulla is not consistent with a pituitary ACTH-secreting adenoma. Answer 4: A pituitary, not adrenal, adenoma was found to be causing the disease. An adrenal adenoma that is funcitional would result in contralateral atrophy of the other adrenal from decreased ACTH production and stimulation. Answer 5: Atrophy of the adrenal gland is not consistent with a pituitary ACTH-secreting adenoma.
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