Binjurebarkshyperplasi

Binjurebarkshyperplasi, or adrenal cortex hyperplasia, is a condition in which there is an increased number of cells in the adrenal cortex, resulting in thickening and enlargement of the adrenal glands. It is usually bilateral, affecting both glands, and can arise as a primary condition or secondary to other disorders that stimulate the adrenal cortex.

Causes and types include congenital adrenal hyperplasia (CAH) due to enzymatic defects in cortisol synthesis, such as 21-hydroxylase or 11β-hydroxylase deficiencies. These defects lead to reduced cortisol production and increased adrenocorticotropic hormone (ACTH) stimulation, causing adrenal hyperplasia. Adrenal hyperplasia can also occur as a secondary response to prolonged ACTH elevation from pituitary or ectopic sources, or as part of broader adrenal disorders such as Cushing’s syndrome. In some cases, treatment-related or drug-induced mechanisms contribute to hyperplasia.

Pathophysiology involves increased trophic signaling to the adrenal cortex, often from ACTH, which drives proliferation of cortical cells in the zona fasciculata and zona reticularis and alters steroid production. The clinical features depend on the underlying cause and the steroid profile produced by the hyperplastic tissue. CAH commonly presents in infancy with salt-wasting crises or ambiguous genitalia in females, and later may show signs of androgen excess. Some defects cause hypertension due to mineralocorticoid precursors.

Diagnosis relies on biochemical testing and imaging. Hormone assays may reveal elevated ACTH and precursors with low cortisol; specific patterns such as elevated 17-hydroxyprogesterone point to 21-hydroxylase deficiency. Imaging (CT or MRI) shows bilateral adrenal enlargement. Genetic testing may confirm enzyme defects.

Treatment addresses the underlying cause: glucocorticoid therapy to suppress excess ACTH in CAH, mineralocorticoid replacement as needed, and careful monitoring. Surgery is rarely indicated, except for specific anatomical or genital concerns. Prognosis depends on early detection and effective hormonal control. Epidemiology varies by condition but congenital forms are rare.