Cryopreservation of Ovarian Cortex in Girls With Turner Syndrome (CRYO-X0)

Assistance Publique - Hôpitaux de Paris

Status

Active, not recruiting

Conditions

Turner Syndrome

Ovarian Insufficiency

Treatments

Procedure: Ovariectomy

Study type

Interventional

Funder types

Other

Identifiers

NCT01410045

P081204

Details and patient eligibility

About

Ovarian insufficiency is common in Turner syndrome related to premature and rapid follicular apoptosis and spontaneous pregnancies are rare in this population. Ovarian cryopreservation has been used in an effort to preserve fertility in patients undergoing treatments which lead to premature and severe ovarian insufficiency. This study aims to assess the relevance of ovarian tissue cryopreservation in girls with Turner syndrome. Based on ovarian follicular density as primary outcome and karyotypic, clinical and hormonal markers as secondary outcomes, analysis of the study will allow to select the patients to whom the procedure would benefit the most.

Full description

Turner syndrome (TS) is characterized by the absence of all or part of a normal second X chromosome and occurs in about 1/2,500 live-born girls. Spontaneous fertility is rare among patients with TS related to premature apoptosis of ovarian follicles. Spontaneous puberty and fertility has been reported mostly in patients with mosaic karyotype or small X deletions.

There is robust evidence that follicles can be observed in ovaries in girls with TS. However, follicular density and quality seems to be largely influenced by karyotype, ovarian morphology and endocrine competence. There are no clear-cut clinical or hormonal markers to assess the ovarian reserve in girls with TS but markers of ovarian function used in women with premature ovarian insufficiency are measured. In TS, it is now fundamental to be able to evaluate the prognosis of the ovarian function and the degree of fertility to provide the relevant information to girls and their parents and to discuss possibilities of motherhood if any.

Ovarian cryopreservation has been used in an effort to preserve fertility in patients undergoing treatments which lead to premature and severe ovarian insufficiency. This study aims to assess the relevance of ovarian tissue cryopreservation in girls with Turner syndrome. Based on ovarian follicular density as primary outcome and karyotypic, clinical and hormonal markers as secondary outcomes, analysis of the study will allow to screen the patients to whom the procedure would benefit the most.

Girls who will be operated will accept to come for a follow-up visit at one and 12 months after the surgery. It is expected to have clinical and hormonal information through a long follow up performed by the referred paediatrician.

Results of the study will allow us to select patients with TS who will benefit the most of this fertility preservation procedure based on karyotypic, clinical and hormonal profile.

Enrollment

47 patients

Sex

Female

Ages

1 to 25 years old

Volunteers

No Healthy Volunteers

Inclusion and exclusion criteria

Inclusion criteria :

girls aged from 1 to 25 years included,
with Turner syndrome or mosaic
patients aged more than 18 will only have ovarian insufficiency dated less than 5 years
without any severe disease, particularly of cardiovascular type
whose agreement to participate to the study has been signed by the parents
whose agreement to participate to the study has been signed by majority age patient

Exclusion criteria :