The kidney cannot be diseased. Once a disease occurs, it is quite serious, including imbalance of renal hormones and instability of adrenaline, which can lead to adrenal syndrome. After kidney disease occurs, the patient will not only suffer from physical fatigue and coma, but also adverse reactions such as body edema and nausea and vomiting. Introduction: CAH patients are congenitally deficient in the enzyme that synthesizes cortisol in the adrenal glands, which leads to impaired cortisol synthesis and excessive secretion of ACTH in the blood, high or low levels of sex hormones, and then abnormalities in the morphology and function of sexual organs as the main clinical manifestations. CAH can be divided into several types according to the different enzymes that are lacking, among which 21-hydroxylase deficiency is the most common. Hydroxylase deficiency accounts for more than 80% of CAH. According to the degree of deficiency, it can be divided into simple virilization type and virilization with salt wasting type. The simple virilizing type is caused by partial deficiency of 21-hydroxylase, accounting for 50% to 80% of 21-hydroxylase deficiency. The main manifestations in females are abnormal development of external genitalia, virilization, breast aplasia during puberty, and amenorrhea. In males, it manifests as precocious genital giant deformity, rapid enlargement of the penis and immature testicles. Patients often have clinical manifestations such as low resistance and pigmentation of the skin and mucous membranes. Masculinization with salt loss is due to severe It is caused by hydroxylase deficiency, and in addition to the symptoms of simple virilization, there may also be hyperkalemia, hyponatremia, hypotension and metabolic acidosis. In severe cases, crisis symptoms such as circulatory failure may occur. 11β-hydroxylase deficiency accounts for about 5% of CAH. In addition to masculinization, patients often suffer from symptoms of hypertension and hypokalemia due to the accumulation of 11-deoxycorticosterone in the blood. Features: The treatment of CAH is mainly based on the supplementation of glucocorticoids. Those with severe salt loss also need to supplement with salt hormones such as deoxycorticosterone acetate (DOCA) or 9α-fluorocortisone. Adrenal sex hormone-secreting tumors are rare and include both virilizing and feminizing adrenocortical tumors. Treatment is mainly surgical. |
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