Features of pubertal development of adolescent girls with cystic fibrosis

Author:

Goryainova Anastasia V.1ORCID,Karachentsova Irina V.2,Sibirskaya Elena V.3,Sharkov Sergey M.4ORCID,Golubkova Varvara M.5,Bondarenko Olesya V.5

Affiliation:

1. Russian Children’s Clinical Hospital, Pirogov Russian National Research Medical University

2. Russian Children’s Clinical Hospital, Pirogov Russian National Research Medical University; Pirogov Russian National Research Medical University

3. Russian Children’s Clinical Hospital, Pirogov Russian National Research Medical University; Moscow State University of Medicine and Dentistry named after A.I. Evdokimov

4. Morozov Children Municipal Clinical Hospital; I.M. Sechenov First Moscow State Medical University

5. Pirogov Russian National Research Medical University

Abstract

This review is devoted to an urgent and insufficiently studied problem - the characteristics of the pubertal development of girls with cystic fibrosis (CF), the deviations in the period of menarche, the stages of the puberty, anatomical and physiological features of the female reproductive system in this monogenic disease. The increase in life expectancy, as well as the spread and increasing availability of targeted therapies, highlight the need for further research on this topic. Early diagnosis of disorders of pubertal development due to timely prescribed therapy (hormonal) allows not only correcting the timing of the onset of menarche in CF adolescent girls, but also increasing the chances of conceiving and bearing a child.

Publisher

National Medical Research Center for Childrens Health

Subject

General Engineering

Reference54 articles.

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2. Cystic Fibrosis Foundation. Patient Registry Annual Data Report – 2019. Available at: https://www.cff.org/sites/default/files/2021-10/2019-Patient-Registry-Annual-Data-Report.pdf

3. Rousset-Jablonski C., Reynaud Q., Nove-Josserand R., Durupt S., Durieu I. Gynecological management and follow-up in women with cystic fibrosis. Rev. Mal. Respir. 2018; 35(6): 592–603. https://doi.org/10.1016/j.rmr.2017.08.004

4. Kazmerski T.M., Prushinskaya O.V., Hill K., Nelson E., Leonard J., Mogren K., et al. Sexual and reproductive health of young women with cystic fibrosis: a concept mapping study.

5. Acad. Pediatr. 2019; 19(3): 307–14. https://doi.org/10.1016/j.acap.2018.08.011

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