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We report the case of a patient who was initially assigned as a male, and in whom the diagnosis of CAH was delayed (17 years of age). He was shown to have female pseudohermaphrodites. Before treatment, plasma testosterone level was: 15 ng/mL (N: 0.1-0.7), 17 OH progesterone (17 OHP): 45 ng/mL (N: 0.1-1.1) and FSH, LH values were below the normal range. While taking hydrocortisone, a rapid onset of new clinical, biological and radiological findings were observed after three months: rapid menarche and thelarche occurred, plasma testosterone and 17 OHP levels decreased, respectively 1 and 3.7 ng/mL, plasma FSH and LH were respectively 4.1 mUI/mL (N: 1.5-7) and 14.3 m UI/mL (N: 1.1-11.7). Polycystic ovaries were shown at sonography. Authors try to discuss the mechanism of the new events observed and specially those related to the polycystic ovarian disease. In a female with untreated CAH at adult age, menarche can rapidly occur once appropriate treatment was started. |
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