Congenital Adrenal Hyperplasia

Two hormones are critical in normal sex differentiation. The testes of normal 46,XY males secrete both Mí¼llerian Inhibiting Substance (also known as MIS or antimí¼llerian hormone) and masculinizing androgenic hormones, while the ovaries of a normal 46,XX female secrete neither. In CAH, the absence of a critical enzyme allows a 46,XX fetus to produce androgens, resulting in ambiguous external genitals. A CAH individual may have an oversized clitoris and fused labia. -pbs.org
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