Last question answer - D. Male and Ambiguous genitalia. The baby in the stem of this question lacks 5-a-reductase, resulting in low dihydrotestosterone (DHT) levels. Testosterone is not affected in patients with 5-a-reductase, resulting in a normal internal urogenital tract. DHT is responsible for the external urogenital tract; without it, genetically male babies will not develop normal external genitalia, and may even be mistaken for females.
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A 14-year-old girl is brought in by her mother for follow-up after being diagnosed with recurrent peptic ulcers. The patient reports having irregular menstrual cycles, and her last menstrual cycle was 3 months ago. Laboratory investigations show gastrin 200 (less than 180), parathyroid hormone 112 pg/ml (10-55), calcium 10.8 mg/dl (8.5-10.2), and prolactin 40 (less than 25). An MRI of the head shows an 8 mm tumor in the pituitary gland. The patient denies any family history of endocrine diseases. Genetic testing shows a positive MEN1 gene mutation. Which of the following is the most likely first presentation of this patient's condition during childhood? [normal values are in the brackets]
1.Sjogren syndrome
2.VIPoma
3.Cushing disease
4.Glaucoma