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The birth weight and length were 3330 g and 51.5 cm, respectively. Apgar scores were 9 and 9 at 1 and 5 min, respectively. This patient showed hyperpigmentation and perineal hypospadias: both testes of 2 mL were palpable in a bifid severely hyperpigmented scrotum. At the age of 3 days, they were screened for hormonal abnormalities: their tests showed markedly elevated 17OHP levels (86 ng/mL n.v. 10 ng/mL), ACTH 250 pg/mL (n.v. 8–53), cortisol 42 µg/L (n.v. 60–230), and DHEAS 1970 mcg/L (560–2360). Testosterone and delta4androstenedione (delta4A) were 1.3 ng/mL (n.v. 17-OHP = 17 hydroxyprogesterone (defined in the abstract). ACTH = Adreno Cortico Tropic Hormone (defined in the abstract).

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