Patient, female, 17 years old, presented with “no menstruation”. Physical examination: T: 36.5°C, P: 78 beats/min, R: 20 beats/, Bp: 150/93mmHg. Height: 155cm, weight: 60kg, skin: dark and rough. Lower lip hair was more black. Laryngeal nodes were obvious and there was no obvious development of breasts. Gynecological examination: pubic hair was dense and male; clitoris was enlarged, about 3 cm long and 1.2 cm in diameter; the lower part of the labia majora on both sides were fused, and the urethral opening was seen, but the vaginal opening was not seen. The uterus was detected on anal examination. Ultrasonography Uterus: length 40mm, thickness 29mm, width 35mm, lining thickness 3mm; left ovary 20mm×15mm×18mm, right ovary 23mm×16mm×12mm. Endocrinology Measurements FSH: 5.6IU/L, LH: 4.3IU/L, PRL: 15pg/ml, estradiol: 56pg/ml, testosterone: 3.8ng/ ml, 17-hydroxyprogesterone: 36 ng/ml, cortisol: 3.2 μg/dl (8:00 AM), 11-deoxycorticosterone: 98 ng/dl. Chromosomal karyotype analysis 46, XX. Why did the patient present with hypertension? ANSWER: The chromosome karyotype is 46,XX and the gonad is ovarian, so the patient’s biological sex is female. The patient has signs of hyperandrogenism: a laryngeal knot, more hair on the lower lip, an enlarged clitoris and elevated blood testosterone levels. The patient was 17 years old at the time of presentation and had never had a menstrual period, so a diagnosis of primary amenorrhea could be made, which in this case was caused by hyperandrogenism. Partial fusion of the labia majora is associated with excessive androgen levels in the fetus during mid-pregnancy. There are two glands in the female body that secrete androgens: the ovaries and the adrenal cortex. According to the two-cell dual gonadotropin theory, androgen secretion by the ovaries is mainly regulated by LH. Androgen secretion by the adrenal cortex is regulated by ACTH, which not only promotes the secretion of adrenal cortical cortisol, but also promotes the secretion of adrenal cortical androgens. Normal testosterone level is less than 0.66ng/ml in adult women and less than 0.8ng/ml in pubertal girls. clinically, testosterone level in functional hyperandrogenism is often <1.5ng/ml, and testosterone level in organic hyperandrogenism is often >1.5ng/ml. functional hyperandrogenism is often without masculinizing signs, while organic hyperandrogenism is often characterized by masculine signs, such as laryngeal nodules and aphthous stomache, and aphthous stomaches. The organic androgenic hyperandrogenizers tend to have signs of masculinization, such as laryngeal nodes, enlarged clitoris, and so on. Organic causes of hyperandrogenism in women include: 21-hydroxylase deficiency and 11β-hydroxylase deficiency in congenital adrenocortical hyperplasia; androgen-secreting tumors such as ovarian blast cell tumors, ovarian supportive-mesenchymal cell tumors, and ovarian steroid cell tumors, etc. The main point of diagnosis of 21-hydroxylase deficiency and 11β-hydroxylase deficiency is that the patient has a significantly elevated level of 17-hydroxyprogesterone. Progesterone levels are significantly elevated, and ultrasound or other imaging is required to identify androgen-secreting tumors.21-Hydroxylase defects or 11β-hydroxylase defects have blood 17-hydroxyprogesterone levels; 11β-hydroxylase defects have elevated levels of 11-deoxycorticosterone, whereas 21-hydroxylase defects do not. Because 11-deoxycorticosterone has aldosterone-like effects that can lead to elevated blood pressure, 11β-hydroxylase defects tend to have hypertension. Based on the clinical presentation and various ancillary tests, we can determine that the patient has 11β-hydroxylase defect. How to treat it? Answer: 11β-hydroxylase defect is mainly treated with glucocorticoids. If the blood pressure is not normalized after using glucocorticosteroids, antihypertensive drugs need to be added. Glucocorticoids: Hydrocortisone is usually used in children at a dose of 10 to 20 mg/m2 per day in 2 to 3 divided doses. Adults use hydrocortisone 37.5mg per day, divided into 2 to 3 doses; prednisone 7.5mg per day, divided into 2 doses; or dexamethasone 0.4 to 0.75mg, taken once a day before bed. Lifelong medication is required. In stressful situations, the dose needs to be increased 1 to 2 times. During surgery or trauma, if the patient cannot take it orally, change to intramuscular injection or intravenous administration. Antihypertensive medication: After glucocorticoid treatment, if the patient’s blood pressure remains high, antihypertensive medication needs to be added. Surgical treatment: Abnormalities of the external genitalia can be corrected surgically. The purpose of the surgery is to reduce the size of the clitoris, expose the vaginal opening, and make the vagina open. The head of the clitoris is rich in nerve endings, which are very important for maintaining sexual pleasure, so nowadays, clitoral body excision is done to preserve the head of the clitoris and its blood vessels and nerves. Fertility problems: In the vast majority of patients, glucocorticoid treatment restores normal ovulation and therefore normal conception is possible. For female patients, lifelong medication is required and should not be stopped during pregnancy. After glucocorticoid treatment, if the patient does not resume ovulation, clomiphene, HMG and HCG can be used to induce ovulation. Clinical points The key to determining whether the etiology of hyperandrogenism is organic or functional is the blood testosterone level and the presence or absence of signs of masculinization; the organic etiology of hyperandrogenism in females includes 21-hydroxylase defects and 11β-hydroxylase defects in congenital adrenocortical hyperplasia; ovarian blastocytoma, ovarian supportive – mesenchymal stromal cell tumors, ovarian steroid cell tumors and other Androgen-secreting tumors; the key to differentiating between 21-hydroxylase defects and 11β-hydroxylase defects is blood 11-deoxycorticosterone levels and the presence of hypertension.