Adrenogenital syndrome with salt loss is a rare disease characterized by a combination of signs of hyperandrogenism (virilization) and salt loss.
The cause of the disease is a tumor or hyperplasia of the adrenal glands that produces excess amounts of androgens. Increased levels of androgens lead to virilization - the development of male secondary sexual characteristics in women.
At the same time, there is a loss of salts (primarily sodium and chlorine) in the urine. This is due to the fact that excess androgens suppress the secretion of aldosterone, the hormone responsible for sodium reabsorption in the renal tubules.
Clinically, the syndrome is manifested by hypertension, edema, weakness, convulsions, and dehydration. Characteristic signs of virilization in women are an enlarged clitoris, male pattern baldness, acne, increased muscle mass, and deepening of the voice.
Diagnosis is based on the detection of high levels of 17-ketosteroids and androgens in the urine and blood against the background of decreased aldosterone levels and loss of salts.
Treatment consists of removing the adrenal tumor or suppressing androgen synthesis. Replacement therapy with glucocorticoids and mineralocorticoids is carried out.
The prognosis for timely treatment is favorable. Without treatment, progression of symptoms and death may occur.
Adrenogenital salt-losing syndrome is a fairly rare and little-studied disease that is characterized by impaired production of cortisol (the stress hormone) in the adrenal cortex. As a result, the work of all organs and systems that require such a hormone is disrupted. This disease manifests itself in the form of cortex deficiency