Two adolescents with chronic adrenal insufficiency, presumably secondary to the cytomegalic type of congenital adrenal hypoplasia, developed a gonadotropin deficiency at puberty, which was both delayed and incomplete. Endocrine studies demonstrated in plasma low LH levels which failed to increase after LH-RH, higher levels of FSH, low basal testosterone concentration but increasing normally after HCG stimulation. Azoospermia was demonstrated in one case. In parallel with those previously reported, these two new cases show that the association between gonadotropin deficiency and congenital adrenal hypoplasia is not accidental, even if the relationship between the two components of the syndrome is still unclear.
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