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. On examination, sexual development corresponded to stage 1 according to Taner. The external genital organs are formed according to the female type. The vagina ends blindly, up to 3.5 cm deep. According to visual methods, the uterus, ovary were absent. According to the hormonal profile, hypergonadotropic hypogonadism, hypoestrogenism, low testosterone levels with normal levels of other androgens were noted. Karyotype - 46, XY. Laparoscopy was performed. Bilateral removal of the sex glands. According to histological findings: The gonads on both sides are represented by testicular tissue, in which all tubules are replaced by Sertoli cells. The right epididymis was normal structure. According to the results of full exome sequencing in the DHH gene (NM_021044.4) mutation at exon 2, the variant in the homozygous state c.419T> G, p.L140R (513x) were found. The patient was prescribed replacement therapy and additional examination in order to exclude peripheral neuropathy. Conclusion: The above clinical observation shows the importance of multidisciplinary management, morphological and molecular verification of the diagnosis for choosing the right tactics for managing such patients.

Chromosome 16 deletion syndrome is find out in 3 of 10,000 newborns. The deletion is found in 1 in 100 people with autism; caused by the absence of a part in a certain region of chromosome 16, designated as p11.2. Methods and results: A 16 year old patient girl. Menarche at age 12. Menses regularly, profuse, painful. With regard to mental retardation and behavioral disorders was observed from an early age but never consulted by geneticist. At the age of 16, due to a cycle disorder –anomaly uterine bleedind, she was first examined by a gynecologist. A malformation of the genital organs was revealed: doubling of the uterus and vagina. Was sent to our Center to choose a tactics of treatment.

Nonwoven polycaprolactone materials produced by electrospinning are perspective internal prosthetic implants. Seeding these implants with multipotent mesenchymal stromal cells stimulates the replacement of the prosthesis with recipient's own connective tissue. Electrospinning method was used for producing polycap-rolactone matrices differing in thickness, pore diameter, fiber size, and biomechanical properties. Labeled cells were seeded on scaffolds in three ways: (1) static, (2) dynamic, and (3) directed flow of the cell suspension generated by capillary action. Cell distribution on the surface and the interior of the scaffolds was studied; the metabolic activity of cells was measured by MTT assay. Static seeding method yielded fully confluence of cells covered the entire scaffold surface, but the cells were located primarily in the upper third of the matrix. Dynamic method proved to be effective only for scaffolds of thickness greater than 500 microns, irrespective of the pore diameter. The third method was effective only for scaffolds with the pore diameter of 20-30 microns, regardless of the material thickness. Resorbable nonwoven polycaprolactone electrospun materials have appropriate biomechanical properties and similar to native tissue matrix structures for internal prosthesis. The choice of the most effective cell seeding method depends on the spatial characteristics - the material thickness, pore diameter, and fibers size, which are determined by the electrospinning conditions.

Actuality: Teratoma is a group of germ cell tumors, developing from pluripotent embryonic gonadae cells capable to undergo somatic and trophoblastic differentiation and histogenetically be a source of various tumors. Teratoma may be localized primarily in the ovaries. While scrutinizing available literature, we found no data on clinical characteristic of adolescents with this pathology. Objective: To provide clinical and anamnestic analysis of patients and undertake structural and morphological analysis of ovarian teratomas based on medical records of adolescent girls admitted to The Research Center for Obstetrics, Gynecology and Perinatology in 2015. Materials and methods: patients from 7 to 17 years old with ovarian teratomas. A retrospective study using clinical-anamnestic, laboratory, and instrumental methods of research. Results: In 2015, 61 patients with ovarian tumors were surgically treated. Of these, 42.6% had ovarian teratoma. There were 3 immature teratomas with malignant component, and 23 patients – mature tumors. The average age of girls was 12.8 ± 0.7 years. 9 (17.0%) patients were asymptomatic. However, the majority (66.7%) complained of periodic pelvic pain, lower back (5.9%), menstruation desorders (35%). According to medical records in one case severe preeclampsia was reported during mother's pregnancy, and twice – neonatal pathology (acute hypoxia, prematurity). 18 girls had average age of menarche 12.8 ± 0.7 years and 9 patients (33.3%) were prepubertal. In 8 (66.6%) of menstruating girls menstrual cycle disorders like delay up to 60 days were observed. According to pelvic ultrasound examination in all cases signs of teratomas were observed. Three girls had teratoma associated with PCOS, four had gidatids, two -malformations of uterus. At gynecological examination teratomas appeared as sensitive tight-elastic masses. Blood tests for tumor markers showed a 2.5-fold increase of CA-125 in two girls with immature ovarian teratomas. In 22 patients the removal of teratoma by ovarian resection preserving healthy tissue was performed during laparoscopy. Three patients underwent laparotomy because of gigant size of tumor and/or malignancy. Postoperative period was uneventful. The results of the histological study showed that teratomas consisted of skin and its appendages (27%), glia with neurons (26%), cartilage (10%), less adipose tissue (6%), tooth tissue, the epithelium of the intestinal and respiratory tract (3%) Conclusion: Perhaps the study of ovarian reserve in these patients will mark the prognosis of fertility in the future.