Leydig Syndrome

Leydig syndrome

Leydig syndrome (Leydig syndrome) or Tay-Sachs syndrome. A rare hereditary disease in which the death of cells that produce androgens in the body occurs. The disease develops under the influence of a mutation in the sulfotransferase 2 gene, located on the X chromosome. In boys, the disease manifests itself as hypogonadism and atrophy of the genital organs, and in girls – increased secondary sexual characteristics. Treatment includes the prescription of hormonal drugs testosterone sodium phenylbutyrate. Symptoms and treatment of the disease. Signs of the disease appear suddenly. Patients become moody, complain of weakness and get tired quickly. Due to disruption of androgen synthesis, boys lag behind in the development of the reproductive apparatus. Prolapse of the scrotum and underdevelopment of the external genitalia. The external difference of the genital organs remains for life. Lack of facial and body hair. Girls have a voice timbre that is unusual for their gender. Rapid enlargement of the mammary glands, secondary hair growth, which has a masculine character. Excess body and chest weight. Static underdevelopment of the pelvic bones. During the first year of a girl's life, they experience menstrual dysfunction and the appearance of vaginal discharge. Treatment of Leydig syndrome is carried out in several stages: Treatment with testosterone. Correction of gonadal deficiency and replacement of the deficiency hormone. Girls undergo tubal ligation because the ovaries are not able to produce the hormones necessary for the full development of the uterus and cyclic