1. Glucocorticoid? Children with p450c 2 1 CAH deficiency should be given glucocorticoid, such as hydrocortisone (HC) or cortisone acetate, once diagnosed. Especially for newborns, the initial therapeutic dose should be large enough to inhibit ACTH secretion. The general oral dose for children is10 ~ 20 mg/(m2 d), which is slightly higher than the physiological requirement. The total dose is generally divided into two or three times, morning 1/2, afternoon and evening 1/4. Usually, patients with CAH need large doses of glucocorticoid to suppress ACTH and androgen levels. In order to achieve a better therapeutic effect and maintain the adrenal cortex in a state of low response, it is necessary to give a large dose of HC [20 ~ 25mg/(m2 d)] for a period of time to make the adrenal gland shrink to a proper degree, and then give a dose close to the physiological needs. The dosage of glucocorticoid should still be adjusted according to the comprehensive analysis of height rate, bone maturity, 17-OHP, androstenedione, testosterone and blood cortisol. If the patient has entered adulthood (> 16 years old), at this time, the epiphysis has been closed, and 0.25 ~ 0.5 mg dexamethasone can be given once before going to bed to inhibit ACTH secretion in the morning. Under stress, the dose of glucocorticoid should be increased to 2 ~ 3 times of the original dose to avoid the crisis of adrenal hypofunction. Female patients need lifelong glucocorticoid replacement therapy; Male patients with pure male CAH have reached their final height by adulthood, so treatment can be interrupted. However, some glucocorticoids should be supplemented according to the severity of stress; Salt loss, both men and women should be treated for life. For true precocious puberty, LHRH-a was given at the same time, with a dose of 4 μ g/(m2 d). Giving mineralocorticoid (such as 9α-FHC) at the same time of glucocorticoid therapy can obviously improve the salt loss state, and is beneficial to improve other clinical symptoms and signs, reduce the dosage of glucocorticoid, and avoid causing Cushing's face and growth disorder. Newborns and infants have poor tolerance to salt loss, so they need a large dose of 9α-FHC 0. 1.5 ~ 0.3 mg/d, and sometimes they need to add 1 ~ 2 g salt to their diet every day. The infant dose is 0.05 ~ 0.1.5 mg/d. Treatment of acute adrenocortical failure (1) Correct dehydration: mild and moderate dehydration, intravenous drip of 5% ~ 10% glucose saline 20 ~ 40 ml/kg within the first 2 hours. (2) Correcting hyponatremia: the sodium supplement amount (mmol/L)=( 135-a measured value) ×0.6× body weight, half of the total amount was given at the 8th ~12h, and the remaining half was added with maintenance amount; 9α-FHC 0.05 ~ 0. 1 mg/d can be taken orally; Mild hyponatremia, 0. 1 ~ 0.2g/kg NaCl can be taken orally for 2 years old without additional salt. (3) Correcting severe hyperkalemia: 0.5g/kg glucose and 0.3U/kg insulin were added intravenously. (4) HC 1 00 ~ 200mg/(m2 d) or cortisone acetate125 ~ 250mg/(m2 d) should be supplemented orally for three times, and the dosage should be reduced after1week, and then reduced to the maintenance dosage after 3 ~ 4 weeks. 3. Surgical treatment? Different degrees of 2 1-OHD lead to different degrees of masculinity of external genitalia in female patients. In mild cases, only the clitoris is slightly enlarged after birth, which is covered by the normal development of vulva without surgery. If clitoral hypertrophy has affected gender judgment, clitoris plastic surgery should be performed within 2 years as soon as possible. Some children still have different degrees of labial fusion and vaginal stenosis, so after puberty, before marriage when necessary, vaginal shaping and expansion are often needed.