VIRILE FORM OF CONGENITAL ADRENAL CORTEX DYSFUNCTION IN AN ADULT PATIENT WITH LOW COMPLIANCE
Abstract
The article presents a case study of a virile form of congenital dysfunction of the adrenal cortex in a 26-year-old patient. An abnormal structure of the external genitalia was revealed at birth, during the newborn period congenital dysfunction of the adrenal cortex was diagnosed (female, 46XX). Hormone therapy with steroids was initiated in the first weeks of life. Since the age of 3 this therapy was stopped, and then he did not receive medical attention until 10 years old. At the age of 10 the decision to change gender has been made by during hospitalization. Until adulthood, he received testosterone and steroid therapy, and underwent surgical procedures (gonadectomy, male-type external genital plastic surgery, testicular prosthetics). He stopped therapy at the age of 18. A special feature of the case is the choice of male sex in a patient with genotype 46XX and low compliance since childhood. The present case shows the need for psychological support and dynamic monitoring of patients with this disease throughout their lives.
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