Adrenal hyperplasia in women

The adrenal cortex is the external tissue located in the adrenal gland region and is a type of endocrine tissue. Adrenal hyperplasia mainly occurs in newborns. It is a congenital disease. Its symptoms are mainly manifested in the masculinization of girls' physiological signs and the excessive secretion of sex hormones in boys, which makes them prone to precocious puberty. So what should women do when they suffer from adrenal hyperplasia?

1. Adrenal hyperplasia

Congenital adrenal hyperplasia is also known as adrenogenital syndrome or adrenal perversion. It is mainly due to defects in the enzymes necessary for the biosynthesis of adrenal cortical hormones, resulting in abnormal synthesis of corticosteroids. In most cases, the adrenal glands secrete insufficient glucose-regulating hormones and salt-regulating hormones but excessive androgens, resulting in varying degrees of adrenal cortical insufficiency clinically, accompanied by masculinization in girls and precocious puberty in boys. In addition, there may be various syndromes such as hyponatremia or hypertension. Congenital adrenal hyperplasia (CAH) is a group of diseases caused by enzyme defects in the synthesis of adrenal cortical hormones. It is an autosomal recessive genetic disease. Those that cause masculinization are also called adrenal sexual dysfunction syndrome. The incidence of typical CHA is about 10/100,000, while the incidence of atypical CHA is about 10 times that of typical CHA and is race-specific.

Treatment of congenital adrenal hyperplasia

1. Correct water and electrolyte imbalance in a timely manner (for children with salt-losing syndrome)

Normal saline can be used for intravenous rehydration, and 0.45% sodium chloride and sodium bicarbonate solution can be used for metabolic acidosis. Avoid using solutions containing potassium. Severe salt-wasting type requires intravenous infusion of hydrocortisone. If hyponatremia and dehydration are difficult to correct, deoxycorticosterone acetate (DOCA) can be injected intramuscularly or fludrocortisone can be taken orally. After dehydration is corrected, glucocorticoids are changed to oral administration and maintained for a long time, while sodium chloride is taken orally at the same time. The amount can be adjusted appropriately according to the condition of the disease.

2. Long-term treatment

(1) Glucocorticoids

On the one hand, glucocorticoid treatment can compensate for the insufficient secretion of cortisol by the adrenal glands, and on the other hand, it can inhibit the excessive release of ACTH, thereby reducing the excessive production of androgens. Therefore, it can improve symptoms such as masculinization and precocious puberty, and ensure the normal growth and development of children.

(2) Mineralocorticoids

Mineralocorticoids can synergize with the effects of glucocorticoids to further reduce the secretion of ACTH. Fludrocortisone can be taken orally. After the symptoms improve, the dosage can be gradually reduced and the medication can be stopped. Long-term use may cause high blood pressure. 0.1 mg of fludrocortisone is equivalent to 1.5 mg of hydrocortisone, and its amount should be calculated in the dosage of cortisol to avoid excessive cortisol.