Abstract

Introduction: Pure form of gonadal dysgenesis, 46 XY - a form of malformation characterized by the presence of female genital organs: hypoplastic uterus, fallopian tubes and dysgenetic gonads, usually represented by fibrous tissue. According to the literature, the above-described pathology can form because various genetic defects at the stage of differentiation from the bipotential gonad into the testicle from 3 to 8 weeks of gestation. Methods and results: A patient with complaints of the absence of secondary sexual characteristics and menstruation, who applied to our Center in order to clarify the diagnosis.

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