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. 2022 Apr 11;18(6):337–352. doi: 10.1038/s41574-022-00655-w

Fig. 2. Adrenal hyperplasia with extensive adrenocortical reticularis.

Fig. 2

Nodular cortical adrenal hyperplasia is common in congenital adrenal hyperplasia (CAH). Intermingled zonation, adrenomedullary dysplasia and hyperplasia, predominantly involving the cells in the adrenocortical reticularis, are found in the untreated or undertreated CAH-affected adrenal. Immunostaining demonstrates strong expression of the zona reticularis enzyme CYB5A, which results in efficient androgen production. Increased CYB5A expression in the CAH-affected adrenal leads to increased activity of steroid 17α-hydroxylase-17,20-lyase (CYP17A1), a key enzyme involved in adrenocortical androgen production. The CAH-adrenal also efficiently makes biologically active 11-oxygenated (11-OH) androgens. Androgens and androgen precursors are highlighted in the deep blue boxes. Dashed lines indicate processes that are blunted in CAH. 17-OHP, 17-hydroxyprogesterone; DHEA, dehydroepiandrosterone; DHEA-S, DHEA sulfate.