Striking adrenal glands enlargement in a patient with 21-hydroxylase deficiency during stress: a case report

Abstract

Abstract Background: Adrenal cortex cells proliferate to produce sufficient cortisol in response to stress by activating the hypothalamic-pituitary adrenal (HPA) axis. In humans, such morphological changes are too mild to be observed. 21-hydroxylase deficiency was the most common type of congenital adrenal hyperplasia (CAH), and chronically elevated adrenocorticotropic hormone level stimulated by cortisol insufficiency can result in adrenal cortex hyperplasia. Here, we report a case of a remarkable enlargement of the bilateral adrenal glands in a short period of time due to the acute stress in a patient with 21-hydroxylase deficiency. It allows doctors to recognize how great potential the adrenal glands have to secrete compensated adrenocortical glucocorticoids in response to certain stress from the perspective of adrenal alteration in morphology and function. Case Description: A 40-year-old man was received chest computed tomography (CT) scan for determining the acute respiratory infection, then significant bilateral adrenal glands enlargement was found accidentally, which the adrenal glands volume was 2.5 times than the past glands six years before. Nonclassical 21-hydroxylase deficiency was diagnosed by genetic testing, and impaired adrenocortical function was determined by a corticotropin stimulation test. The re-examined CT showed that the adrenal glands had returned to the baseline within a month without any treatment. The bilateral adrenal enlargement in the short term was explained by a physiological response to the acute stress, after excluding other pathological causes. Conclusions: For bilateral adrenal enlargement, in addition to adrenal tumors, infections and autoimmune diseases, physiological adrenal hyperplasia under acute stress should also be considered, especially in patients with adrenocortical dysfunction.