rospedРоссийский педиатрический журнал имени М.Я. СтуденикинаM.Ya. Studenikin Russian Pediatric JournalФГАУ «НМИЦ здоровья детей» Минздрава России10.46563/1560-9561-2022-25-4-230-235lcgetgrosped-214Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL INVESTIGATIONSОсобенности полового развития девочек-подростков с муковисцидозомFeatures of pubertal development of adolescent girls with cystic fibrosishttps://orcid.org/0000-0002-8302-1207ГоряиноваАнастасия ВасильевнаGoryainovaAnastasia V.

Канд. мед. наук, врач-педиатр, гастроэнтеролог, сотрудник педиатрического отд-ния Российской детской клинической больницы.

e-mail: dr.goryainova@gmail.com

dr.goryainova@gmail.comКараченцоваИрина ВасильевнаKarachentsovaIrina V.

Канд. мед. наук, доцент каф. акушерства и гинекологии педиатрического факультета ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава России, гл. внештатный специалист — гинеколог детского и юношеского возраста РДКБ.

e-mail: 5053104@list.ru

MD, PhD, Associate Professor of the Department of Obstetrics and Gynecology, Faculty of Pediatrics, Pirogov Russian National Research Medical University, 119571, Moscow, Russian Federation; obstetrician-gynecologist of the surgical gynecological department of Russian Children’s Clinical Hospital, Pirogov Russian National Research Medical University, 119571, Moscow, Russian Federation.

e-mail: 5053104@list.ru 

5053104@list.ruСибирскаяЕлена ВикторовнаSibirskayaElena V.

Доктор мед. наук, проф. каф. репродуктивной медицины и хирургии МГМСУ, гл. специалист гинеколог детского и юношеского возраста Московской области, руководитель центра охраны репродуктивного здоровья МО на базе ГБУЗ МО Долгопрудненской центральной городской больницы, зав. хирургическим гинекологическим отд-нием Российской детской клинической больницы.

e-mail: elsibirskaya@yandex.ru

elsibirskaya@yandex.ruhttps://orcid.org/0000-0002-9563-6815ШарковСергей МихайловичSharkovSergey M.

Доктор мед. наук, проф. каф. детской хирургии и урологии-андрологии им. Л.П. Александрова, ФГАОУ ВО «Первый МГМУ им. И.М. Сеченова» Минздрава России (Сеченовский Университет), зав. оперблоком Морозовской детской городской клинической больницы ДЗ города Москвы.

e-mail: sharkdoc@mail.ru

sharkdoc@mail.ruГолубковаВарвара МихайловнаGolubkovaVarvara M.

Студентка 6-го курса ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава России.

e-mail: golubkova.bb@gmail.com

golubkova.bb@gmail.comБондаренкоОлеся ВикторовнаBondarenkoOlesya V.

Студентка 6-го курса ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава России.

e-mail: olessia1998@yandex.ru

olessia1998@yandex.ruРоссийская детская клиническая больница ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииRussian Children’s Clinical Hospital, Pirogov Russian National Research Medical UniversityРоссийская детская клиническая больница ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России; ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииRussian Children’s Clinical Hospital, Pirogov Russian National Research Medical University; Pirogov Russian National Research Medical UniversityРоссийская детская клиническая больница ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России; ФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова» Минздрава РоссииRussian Children’s Clinical Hospital, Pirogov Russian National Research Medical University; Moscow State University of Medicine and Dentistry named after A.I. EvdokimovГБУЗ «Морозовская детская городская клиническая больница» Департамента здравоохранения города Москвы; ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)Morozov Children Municipal Clinical Hospital; I.M. Sechenov First Moscow State Medical UniversityФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииPirogov Russian National Research Medical University202227092023254230235Copyright © Горяинова А.В., Караченцова И.В., Сибирская Е.В., Шарков С.М., Голубкова В.М., Бондаренко О.В., 20232023Горяинова А.В., Караченцова И.В., Сибирская Е.В., Шарков С.М., Голубкова В.М., Бондаренко О.В.Goryainova A.V., Karachentsova I.V., Sibirskaya E.V., Sharkov S.M., Golubkova V.M., Bondarenko O.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.rosped.ru/jour/article/view/214

Обзор посвящён актуальной и недостаточно изученной проблеме — особенностям полового развития девочек-подростков с муковисцидозом, срокам становления менархе, отклонениям в этапах пубертатного периода, анатомо-физиологическим особенностям женской репродуктивной системы при данном моногенном заболевании. Увеличение продолжительности жизни, а также распространение и повышение доступности таргетной терапии определяет необходимость дальнейших исследований данной темы. Ранняя диагностика нарушения полового развития за счёт своевременно назначенной терапии (гормональной) позволяет не только корригировать сроки наступления менархе у девочек-подростков с муковисцидозом, но и увеличить шансы зачатия и вынашивания ребёнка.

Участие авторов

Участие авторов:Горяинова А.В., Караченцова И.В., Сибирская Е.В., Шарков С.М. — концепция и дизайн исследования;Горяинова А.В., Голубкова В.М., Бондаренко О.В., Караченцова И.В. — сбор и обработка материала, написание текста; Бондаренко О.В., Голубкова В.М., Караченцова И.В. — редактирование.Все соавторы — утверждение окончательного варианта статьи, ответственность за целостность всех частей статьи.

Финансирование

Финансирование. Исследование не имело финансовой поддержки.

Конфликт интересов

Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов. 

Поступила 16

Поступила 16.06.2022Принята к печати 29.06.2022Опубликована 30.08.2022

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.

Contribution

Contribution:Goryainova A.V., Karachentsova I.V., Sibirskaya E.V., Sharkov S.M. — research concept and design of the study;Goryainova A.V., Golubkova V.M., Bondarenko O.V., Karachentsova I.V. — collection and processing of material;Goryainova A.V., Golubkova V.M., Bondarenko O.V., Karachentsova I.V. — text writing;Bondarenko O.V., Go­lubkova V.M., Karachentsova I.V. — editing.All co-authors — approval of the final version of the article, responsibility for the integrity of all parts of the article.

Acknowledgment

Acknowledgment. The study had no sponsorship. 

Conflict of interest

Conflict of interest. The authors declare no conflict of interest. 

Received

Received: June 16, 2022Accepted: June 29, 2022Published: 30 August, 2022

девочки-подросткименархепубертатный периодмуковисцидозполовое развитиеmenarchepubertycystic fibrosispubertal developmentReferencesКаширская Н.Ю., Кондратьева Е.И., Красовский С.А., Старинова М.А., Воронкова А.Ю., Амелина Е.Л. и др. Регистр больных муковисцидозом в Российской Федерации - 2019 год. М.: ИД Медпрактика-М; 2021Kashirskaya N.Yu., Kondrat’eva E.I., Krasovskiy S.A., Starinova M.A., Voronkova A.Yu., Amelina E.L., et al. Register of Patients with Cystic Fibrosis in the Russian Federation – 2019 [Registr bol’nykh mukovistsidozom v Rossiyskoy Federatsii – 2019 god]. Moscow: ID Medpraktika-M; 2021. (in Russian)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.pdfCystic 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.pdfRousset-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.2017.08.004Rousset-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.2017.08.004Kazmerski 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.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.Acad. Pediatr. 2019; 19(3): 307-14. https://doi.org/10.1016/j.acap.2018.08.011Acad. Pediatr. 2019; 19(3): 307–14. https://doi.org/10.1016/j.acap.2018.08.011Arjmand B., Larijani B., Sheikh Hosseini M., Payab M., Gilany K., Goodarzi P., et al. The horizon of gene therapy in modern medicine: advances and challenges. Adv. Exp. Med. Biol. 2020; 1247: 33-64. https://doi.org/10.1007/5584_2019_463Arjmand B., Larijani B., Sheikh Hosseini M., Payab M., Gilany K., Goodarzi P., et al. The horizon of gene therapy in modern medicine: advances and challenges. Adv. Exp. Med. Biol. 2020; 1247: 33–64. https://doi.org/10.1007/5584_2019_463Kushary S., Ali N., Spencer J.B., Dokson J., Hunt W.R. Assessment of a novel genetic counselling intervention to inform assisted reproductive technology treatments and other family-building options in adults with cystic fibrosis. Reprod. Biomed. Soc. Online. 2021; 13: 37-45. https://doi.org/10.1016/j.rbms.2021.05.001Kushary S., Ali N., Spencer J.B., Dokson J., Hunt W.R. Assessment of a novel genetic counselling intervention to inform assisted reproductive technology treatments and other family-building options in adults with cystic fibrosis. Reprod. Biomed. Soc. Online. 2021; 13: 37–45. https://doi.org/10.1016/j.rbms.2021.05.001Goetz D.M., Savant A.P. Review of CFTR modulators 2020. Pediatr. Pulmonol. 2021; 56(12): 3595-606. https://doi.org/10.1002/ppul.25627Goetz D.M., Savant A.P. Review of CFTR modulators 2020. Pediatr. Pulmonol. 2021; 56(12): 3595–606. https://doi.org/10.1002/ppul.25627Shteinberg M., Taylor-Cousar J.L., Durieu I., Cohen-Cymberknoh M. Fertility and pregnancy in cystic fibrosis. Chest. 2021; 160(6): 2051-60. https://doi.org/10.1016/j.chest.2021.07.024Shteinberg M., Taylor-Cousar J.L., Durieu I., Cohen-Cymberknoh M. Fertility and pregnancy in cystic fibrosis. Chest. 2021; 160(6): 2051–60. https://doi.org/10.1016/j.chest.2021.07.024Wang X.F., Zhou C.X., Shi Q.X., Yuan Y.Y., Yu M.K., Ajonuma L.C., et al. Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm. Nat. Cell Biol. 2003; 5(10): 902-6. https://doi.org/10.1038/ncb1047Wang X.F., Zhou C.X., Shi Q.X., Yuan Y.Y., Yu M.K., Ajonuma L.C., et al. Involvement of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm. Nat. Cell Biol. 2003; 5(10): 902–6. https://doi.org/10.1038/ncb1047Ahmad A., Ahmed A., Patrizio P. Cystic fibrosis and fertility. Curr. Opin. Obstet. Gynecol. 2013; 25(3): 167-72. https://doi.org/10.1097/GCO.0b013e32835f1745Ahmad A., Ahmed A., Patrizio P. Cystic fibrosis and fertility. Curr. Opin. Obstet. Gynecol. 2013; 25(3): 167–72. https://doi.org/10.1097/GCO.0b013e32835f1745Chan H.C., Shi Q.X., Zhou C.X., Wang X.F., Xu W.M., Chen W.Y., et al. Critical role of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm. Mol. Cell. Endocrinol. 2006; 250(1-2): 106-13. https://doi.org/10.1016/j.mce.2005.12.032Chan H.C., Shi Q.X., Zhou C.X., Wang X.F., Xu W.M., Chen W.Y., et al. Critical role of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm. Mol. Cell. Endocrinol. 2006; 250(1-2): 106–13. https://doi.org/10.1016/j.mce.2005.12.032Xu W.M., Shi Q.X., Chen W.Y., Zhou C.X., Ni Y., Rowlands D.K., et al. Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility. Proc. Natl Acad. Sci. USA. 2007; 104(23): 9816-21. https://doi.org/10.1073/pnas.0609253104Xu W.M., Shi Q.X., Chen W.Y., Zhou C.X., Ni Y., Rowlands D.K., et al. Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility. Proc. Natl Acad. Sci. USA. 2007; 104(23): 9816–21. https://doi.org/10.1073/pnas.0609253104Rode B., Dirami T., Bakouh N., Rizk-Rabin M., Norez C., Lhuillier P., et al. The testis anion transporter TAT1 (SLC26A8) physically and functionally interacts with the cystic fibrosis transmembrane conductance regulator channel: a potential role during sperm capacitation. Hum. Mol. Genet. 2012; 21(6): 1287-98. https://doi.org/10.1093/hmg/ddr558Rode B., Dirami T., Bakouh N., Rizk-Rabin M., Norez C., Lhuillier P., et al. The testis anion transporter TAT1 (SLC26A8) physically and functionally interacts with the cystic fibrosis transmembrane conductance regulator channel: a potential role during sperm capacitation. Hum. Mol. Genet. 2012; 21(6): 1287–98. https://doi.org/10.1093/hmg/ddr558Weenen C., Laven J.S.E., Von Bergh A.R.M., Cranfield M., Groome N.P., Visser J.A., et al. Anti-Müllerian hormone expression pattern in the human ovary: potential implications for initial and cyclic follicle recruitment. Mol. Hum. Reprod. 2004; 10(2): 77-83. https://doi.org/10.1093/molehr/gah015Weenen C., Laven J.S.E., Von Bergh A.R.M., Cranfield M., Groome N.P., Visser J.A., et al. Anti-Müllerian hormone expression pattern in the human ovary: potential implications for initial and cyclic follicle recruitment. Mol. Hum. Reprod. 2004; 10(2): 77–83. https://doi.org/10.1093/molehr/gah015Dewailly D., Robin G., Peigne M., Decanter C., Pigny P., Catteau-Jonard S.Interactions between androgens, FSH, anti-Müllerian hormone and estradiol during folliculogenesis in the human normal and polycystic ovary. Hum. Reprod. Update. 2016; 22(6): 709-24. https://doi.org/10.1093/humupd/dmw027Dewailly D., Robin G., Peigne M., Decanter C., Pigny P., Catteau-Jonard S. Interactions between androgens, FSH, anti-Müllerian hormone and estradiol during folliculogenesis in the human normal and polycystic ovary. Hum. Reprod. Update. 2016; 22(6): 709–24. https://doi.org/10.1093/humupd/dmw027Bradley G.M., Blackman S.M., Watson C.P., Doshi V.K., Cutting G.R. Genetic modifiers of nutritional status in cystic fibrosis. Am. J. Clin. Nutr. 2012; 96(6):1299-308. https://doi.org/10.3945/ajcn.112.043406Bradley G.M., Blackman S.M., Watson C.P., Doshi V.K., Cutting G.R. Genetic modifiers of nutritional status in cystic fibrosis. Am. J. Clin. Nutr. 2012; 96(6):1299–308. https://doi.org/10.3945/ajcn.112.043406Ratchford T.L., Teckman J.H., Patel D.R. Gastrointestinal pathophysiology and nutrition in cystic fibrosis. Expert Rev. Gastroenterol. Hepatol. 2018; 12(9): 853-62. https://doi.org/10.1080/17474124.2018.1502663Ratchford T.L., Teckman J.H., Patel D.R. Gastrointestinal pathophysiology and nutrition in cystic fibrosis. Expert Rev. Gastroenterol. Hepatol. 2018; 12(9): 853–62. https://doi.org/10.1080/17474124.2018.1502663Mauch R.M., Kmit A.H., Marson F.A., Levy C.E., Barros-Filho A.A., Ribeiro J.D. Association of growth and nutritional parameters with pulmonary function in cystic fibrosis: a literature review. Rev. Paul. Pediatr. 2016; 34(4): 503-9. https://doi.org/10.1016/j.rpped.2015.12.002Mauch R.M., Kmit A.H., Marson F.A., Levy C.E., Barros-Filho A.A., Ribeiro J.D. Association of growth and nutritional parameters with pulmonary function in cystic fibrosis: a literature review. Rev. Paul. Pediatr. 2016; 34(4): 503–9. https://doi.org/10.1016/j.rpped.2015.12.002Dos Santos A.L.M., de Melo Santos H., Nogueira M.B., Távora H.T.O., de Lourdes Jaborandy Paim da Cunha M., de Melo Seixas R.B.P., et al. Cystic fibrosis: clinical phenotypes in children and adolescents. Pediatr. Gastroenterol. Hepatol. Nutr. 2018; 21(4): 306-14. https://doi.org/10.5223/pghn.2018.21.4.306Dos Santos A.L.M., de Melo Santos H., Nogueira M.B., Távora H.T.O., de Lourdes Jaborandy Paim da Cunha M., de Melo Seixas R.B.P., et al. Cystic fibrosis: clinical phenotypes in children and adolescents. Pediatr. Gastroenterol. Hepatol. Nutr. 2018; 21(4): 306–14. https://doi.org/10.5223/pghn.2018.21.4.306Горяинова А.В., Поликарпова С.В., Семыкин С.Ю., Каширская Н.Ю., Михалаки П.И. Активность тобрамицина в отношении штаммов Pseudomonas aeruginosa, выделенных у пациентов с муковисцидозом. Доктор.Ру. 2021; 20(3): 17-23. https://doi.org/10.31550/1727-2378-2021-20-3-17-23Connett G.J., Pike K.C. Nutritional outcomes in cystic fibrosis – are we doing enough? Paediatr. Respir. Rev. 2015; 16(Suppl. 1): 31–4. https://doi.org/10.1016/j.prrv.2015.07.015Горинова Ю.В., Симонова О.И., Лазарева А.В., Черневич В.П., Смирнов И.Е. Опыт длительного применения ингаляций раствора тобрамицина при хронической синегнойной инфекции у детей с муковисцидозом. Российский педиатрический журнал. 2015; 18(3): 50-3.Goryainova A.V., Polikarpova S.V., Semykin S.Yu., Kashirskaya N.Yu., Mikhalaki P.I. Tobramycin activity for Pseudomonas Aeruginosa spp. isolated in cystic fibrosis patients. Doktor.Ru. 2021; 20(3): 17–23. https://doi.org/10.31550/1727-2378-2021-20-3-17-23 (in Russian)Смирнов И.Е., Тарасова О.В., Лукина О.Ф., Кустова О.В., Сорокина Т.Е., Симонова О.И. Структурно-функциональное состояние легких при муковисцидозе у детей. Российский педиатрический журнал. 2015; 18(2): 11-7.Gorinova Yu.V., Simonova O.I., Lazareva A.V., Chernevich V.P., Smirnov I.E. Experience of the sustainable use of inhalations of tobramycin solution in chronic Pseudomonas aeruginosa infection in children with cystic fibrosis. Rossiyskiy pediatricheskiy zhurnal. 2015; 18(3): 50–3. (in Russian)Смирнов И.Е., Кучеренко А.Г., Егоров М.С., Смирнова Г.И., Уртнасан Ц., Симонова О.И. и др. Матриксные металлопротеиназы при муковисцидозе у детей. Российский педиатрический журнал. 2018; 21(3): 145-51. https://doi.org/10.18821/1560-9561-2018-21-3-145-151Smirnov I.E., Tarasova O.V., Lukina O.F., Kustova O.V., Sorokina T.E., Simonova O.I. Structural and functional state of the lungs in cystic fibrosis in children. Rossiyskiy pediatricheskiy zhurnal. 2015; 18(2): 11–7. (in Russian)Smirnov I.E., Kucherenko A.G., Egorov M.S., Smirnova G.I., Urtnasan Ts., Simonova O.I., et al. Matrix metalloproteinases in children with cystic fibrosis. Rossiyskiy pediatricheskiy zhurnal. 2018; 21(3): 145-51. https://doi.org/10.18821/1560-9561-2018-21-3-145-151 (in Russian)Smirnov I.E., Kucherenko A.G., Egorov M.S., Smirnova G.I., Urtnasan Ts., Simonova O.I., et al. Matrix metalloproteinases in children with cystic fibrosis. Rossiyskiy pediatricheskiy zhurnal. 2018; 21(3): 145–51. https://doi.org/10.18821/1560-9561-2018-21-3-145-151 (in Russian)Neinstein L.S., Stewart D., Wang C.I., Johnson I. Menstrual dysfunction in cystic fibrosis. J Adolesc. Health Care. 1983; 4(3): 153-7. https://doi.org/10.1016/s0197-0070(83)80367-2Neinstein L.S., Stewart D., Wang C.I., Johnson I. Menstrual dysfunction in cystic fibrosis. J Adolesc. Health Care. 1983; 4(3): 153–7. https://doi.org/10.1016/s0197-0070(83)80367-2Schulze K.J., O’Brien K.O., Germain-Lee E.L., Booth S.L., Leonard A., Rosenstein B.J. Calcium kinetics are altered in clinically stable girls with cystic fibrosis. J. Clin. Endocrinol. Metab. 2004; 89(7): 3385-91. https://doi.org/10.1210/jc.2003-031879Schulze K.J., O’Brien K.O., Germain-Lee E.L., Booth S.L., Leonard A., Rosenstein B.J. Calcium kinetics are altered in clinically stable girls with cystic fibrosis. J. Clin. Endocrinol. Metab. 2004; 89(7): 3385–91. https://doi.org/10.1210/jc.2003-031879Umławska W., Sands D., Zielińska A. Age of menarche in girls with cystic fibrosis. Folia Histochem. Cytobiol. 2010; 48(2): 185-90. https://doi.org/10.2478/v10042-010-0051-xUmławska W., Sands D., Zielińska A. Age of menarche in girls with cystic fibrosis. Folia Histochem. Cytobiol. 2010; 48(2): 185–90. https://doi.org/10.2478/v10042-010-0051-xGaudino R., Dal Ben S., Cavarzere P., Volpi S., Piona C., Boner A., et al. Delayed age at menarche in chronic respiratory diseases. Eur. J. Clin. Invest. 2021; 51(5): e13461. https://doi.org/10.1111/eci.13461Gaudino R., Dal Ben S., Cavarzere P., Volpi S., Piona C., Boner A., et al. Delayed age at menarche in chronic respiratory diseases. Eur. J. Clin. Invest. 2021; 51(5): e13461. https://doi.org/10.1111/eci.13461de Souza Dias Lopes P., Machado S.H., Lucena I.R.S., Marostica P.J.C. Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging. Arch. Endocrinol. Metab. 2021; 65(5): 632-9. https://doi.org/10.20945/2359-3997000000404de Souza Dias Lopes P., Machado S.H., Lucena I.R.S., Marostica P.J.C. Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging. Arch. Endocrinol. Metab. 2021; 65(5): 632–9. https://doi.org/10.20945/2359-3997000000404Dal Ben S., Gaudino R., Cavarzere P., Volpi S., Antoniazzi F., Boner A., et al. Age at menarche in girls with cystic fibrosis and asthma. Minerva Endocrinol. (Torino). 2022. https://doi.org/10.23736/S2724-6507.21.03451-5Dal Ben S., Gaudino R., Cavarzere P., Volpi S., Antoniazzi F., Boner A., et al. Age at menarche in girls with cystic fibrosis and asthma. Minerva Endocrinol. (Torino). 2022. https://doi.org/10.23736/S2724-6507.21.03451-5Hughan K.S., Daley T., Rayas M.S., Kelly A., Roe A. Female reproductive health in cystic fibrosis. J. Cyst. Fibros. 2019; 18(Suppl. 2): 95-104. https://doi.org/10.1016/j.jcf.2019.08.024Hughan K.S., Daley T., Rayas M.S., Kelly A., Roe A. Female reproductive health in cystic fibrosis. J. Cyst. Fibros. 2019; 18(Suppl. 2): 95–104. https://doi.org/10.1016/j.jcf.2019.08.024Heltshe S.L., Godfrey E.M., Josephy T., Aitken M.L., Taylor-Cousar J.L. Pregnancy among cystic fibrosis women in the era of CFTR modulators. J. Cyst. Fibros. 2017; 16(6): 687-94. https://doi.org/10.1016/j.jcf.2017.01.008Heltshe S.L., Godfrey E.M., Josephy T., Aitken M.L., Taylor-Cousar J.L. Pregnancy among cystic fibrosis women in the era of CFTR modulators. J. Cyst. Fibros. 2017; 16(6): 687–94. https://doi.org/10.1016/j.jcf.2017.01.008Braun C., Bacchetta J., Braillon P., Chapurlat R., Drai J., Reix P. Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography. Osteoporos.Int. 2017; 28(11): 3179-88. https://doi.org/10.1007/s00198-017-4179-9Braun C., Bacchetta J., Braillon P., Chapurlat R., Drai J., Reix P. Children and adolescents with cystic fibrosis display moderate bone microarchitecture abnormalities: data from high-resolution peripheral quantitative computed tomography. Osteoporos. Int. 2017; 28(11): 3179–88. https://doi.org/10.1007/s00198-017-4179-9Edenborough F.P. Women with cystic fibrosis and their potential for reproduction. Thorax. 2001; 56(8): 649-55. https://doi.org/10.1136/thorax.56.8.649Edenborough F.P. Women with cystic fibrosis and their potential for reproduction. Thorax. 2001; 56(8): 649–55. https://doi.org/10.1136/thorax.56.8.649Frayman K.B., Chin M., Sawyer S.M., Bell S.C. Sexual and reproductive health in cystic fibrosis. Curr. Opin. Pulm. Med. 2020; 26(6): 685-95. https://doi.org/10.1097/MCP.0000000000000731Frayman K.B., Chin M., Sawyer S.M., Bell S.C. Sexual and reproductive health in cystic fibrosis. Curr. Opin. Pulm. Med. 2020; 26(6): 685–95. https://doi.org/10.1097/MCP.0000000000000731Kazmerski T.M., Sawicki G.S., Miller E., Jones K.A., Abebe K.Z., Tuchman L.K., et al. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J. Cyst. Fibros. 2018; 17(1): 57-63. https://doi.org/10.1016/j.jcf.2017.07.017Kazmerski T.M., Sawicki G.S., Miller E., Jones K.A., Abebe K.Z., Tuchman L.K., et al. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J. Cyst. Fibros. 2018; 17(1): 57–63. https://doi.org/10.1016/j.jcf.2017.07.017Kazmerski T.M., Hill K., Prushinskaya O., Nelson E., Greenberg J., Pitts S.A., et al. Perspectives of adolescent girls with cystic fibrosis and parents on disease-specific sexual and reproductive health education. Pediatr. Pulmonol. 2018; 53(8): 1027-34. https://doi.org/10.1002/ppul.24015Kazmerski T.M., Hill K., Prushinskaya O., Nelson E., Greenberg J., Pitts S.A., et al. Perspectives of adolescent girls with cystic fibrosis and parents on disease-specific sexual and reproductive health education. Pediatr. Pulmonol. 2018; 53(8): 1027–34. https://doi.org/10.1002/ppul.24015Umławska W., Susanne C. Growth and nutritional status in children and adolescents with cystic fibrosis. Ann. Hum. Biol. 2008; 35(2): 145-53. https://doi.org/10.1080/03014460701824132Umławska W., Susanne C. Growth and nutritional status in children and adolescents with cystic fibrosis. Ann. Hum. Biol. 2008; 35(2): 145–53. https://doi.org/10.1080/03014460701824132Sands D., Umławska W., Zielińska A. A cross-sectional study of growth, nutritional status and body proportions in children and adolescents at a medical center specializing in the treatment of cystic fibrosis in Poland. Arch. Med. Sci. 2015; 11(1): 155-63. https://doi.org/10.5114/aoms.2015.49207Sands D., Umławska W., Zielińska A. A cross-sectional study of growth, nutritional status and body proportions in children and adolescents at a medical center specializing in the treatment of cystic fibrosis in Poland. Arch. Med. Sci. 2015; 11(1): 155–63. https://doi.org/10.5114/aoms.2015.49207Mędza A., Kaźmierska K., Wielgomas B., Konieczna L., Olędzka I., Szlagatys-Sidorkiewicz A., et al. DeltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis do not differ with regard to nutritional status. Nutrients. 2021; 13(5): 1402. https://doi.org/10.3390/nu13051402Mędza A., Kaźmierska K., Wielgomas B., Konieczna L., Olędzka I., Szlagatys-Sidorkiewicz A., et al. DeltaF508 CFTR hetero- and homozygous paediatric patients with cystic fibrosis do not differ with regard to nutritional status. Nutrients. 2021; 13(5): 1402. https://doi.org/10.3390/nu13051402Galli-Tsinopoulou A., Moudiou T., Mamopoulos A., Karamouzis M., Nousia-Arvanitakis S. Multifollicular ovaries in female adolescents with cystic fibrosis. Fertil. Steril. 2006; 85(5): 1484-7. https://doi.org/10.1016/j.fertnstert.2005.10.051Galli-Tsinopoulou A., Moudiou T., Mamopoulos A., Karamouzis M., Nousia-Arvanitakis S. Multifollicular ovaries in female adolescents with cystic fibrosis. Fertil. Steril. 2006; 85(5): 1484–7. https://doi.org/10.1016/j.fertnstert.2005.10.051Kelestimur F., Unluhizarci K., Baybuga H., Atmaca H., Bayram F., Sahin Y. Prevalence of polycystic ovarian changes and polycystic ovary syndrome in premenopausal women with treated type 2 diabetes mellitus. Fertil. Steril. 2006; 86(2): 405-10. https://doi.org/10.1016/j.fertnstert.2006.01.019Kelestimur F., Unluhizarci K., Baybuga H., Atmaca H., Bayram F., Sahin Y. Prevalence of polycystic ovarian changes and polycystic ovary syndrome in premenopausal women with treated type 2 diabetes mellitus. Fertil. Steril. 2006; 86(2): 405–10. https://doi.org/10.1016/j.fertnstert.2006.01.019Hassa H., Tanir H.M., Yildiz Z.Comparison of clinical and laboratory characteristics of cases with polycystic ovarian syndrome based on Rotterdam’s criteria and women whose only clinical signs are oligo/anovulation or hirsutism. Arch. Gynecol. Obstet. 2006; 274(4): 227-32. https://doi.org/10.1007/s00404-006-0173-8Hassa H., Tanir H.M., Yildiz Z. Comparison of clinical and laboratory characteristics of cases with polycystic ovarian syndrome based on Rotterdam’s criteria and women whose only clinical signs are oligo/anovulation or hirsutism. Arch. Gynecol. Obstet. 2006; 274(4): 227–32. https://doi.org/10.1007/s00404-006-0173-8Cirillo F., Catellani C., Sartori C., Lazzeroni P., Morini D., Nicoli A., et al. CFTR and FOXO1 gene expression are reduced and high mobility group box 1 (HMGB1) is increased in the ovaries and serum of women with polycystic ovarian syndrome. Gynecol. Endocrinol. 2019; 35(10): 842-6. https://doi.org/10.1080/09513590.2019.1599349Cirillo F., Catellani C., Sartori C., Lazzeroni P., Morini D., Nicoli A., et al. CFTR and FOXO1 gene expression are reduced and high mobility group box 1 (HMGB1) is increased in the ovaries and serum of women with polycystic ovarian syndrome. Gynecol. Endocrinol. 2019; 35(10): 842–6. https://doi.org/10.1080/09513590.2019.1599349Wu M., Bettermann E.L., Arora N., Hunt W.R., McCracken C., Tangpricha V. Relationship between estrogen treatment and skeletal health in women with cystic fibrosis. Am. J. Med. Sci. 2020; 360(5): 581-90. https://doi.org/10.1016/j.amjms.2020.06.005Wu M., Bettermann E.L., Arora N., Hunt W.R., McCracken C., Tangpricha V. Relationship between estrogen treatment and skeletal health in women with cystic fibrosis. Am. J. Med. Sci. 2020; 360(5): 581–90. https://doi.org/10.1016/j.amjms.2020.06.005Garg V., Shen J., Li C., Agarwal S., Gebre A., Robertson S., et al. Pharmacokinetic and drug-drug interaction profiles of the combination of tezacaftor/ivacaftor. Clin. Transl. Sci. 2019; 12(3): 267-75. https://doi.org/10.1111/cts.12610Garg V., Shen J., Li C., Agarwal S., Gebre A., Robertson S., et al. Pharmacokinetic and drug-drug interaction profiles of the combination of tezacaftor/ivacaftor. Clin. Transl. Sci. 2019; 12(3): 267–75. https://doi.org/10.1111/cts.12610Roe A.H., Traxler S., Schreiber C.A. Contraception in women with cystic fibrosis: a systematic review of the literature. Contraception. 2016; 93(1): 3-10. https://doi.org/10.1016/j.contraception.2015.07.007Roe A.H., Traxler S., Schreiber C.A. Contraception in women with cystic fibrosis: a systematic review of the literature. Contraception. 2016; 93(1): 3–10. https://doi.org/10.1016/j.contraception.2015.07.007Roe A.H., Merjaneh L., Oxman R., Hughan K.S. Gynecologic health care for females with cystic fibrosis. J. Clin. Transl. Endocrinol. 2021; 26: 100277. https://doi.org/10.1016/j.jcte.2021.100277Roe A.H., Merjaneh L., Oxman R., Hughan K.S. Gynecologic health care for females with cystic fibrosis. J. Clin. Transl. Endocrinol. 2021; 26: 100277. https://doi.org/10.1016/j.jcte.2021.100277Perrissin-Fabert M., Stheneur C., Veilleux-Lemieux M., Taddeo D., Marcotte J.E., Boutin A., et al. Hormonal Contraception Effects on Pulmonary Function in Adolescents with Cystic Fibrosis. J. Pediatr. Adolesc. Gynecol. 2020; 33(6): 681-5. https://doi.org/10.1016/j.jpag.2020.07.014Perrissin-Fabert M., Stheneur C., Veilleux-Lemieux M., Taddeo D., Marcotte J.E., Boutin A., et al. Hormonal Contraception Effects on Pulmonary Function in Adolescents with Cystic Fibrosis. J. Pediatr. Adolesc. Gynecol. 2020; 33(6): 681–5. https://doi.org/10.1016/j.jpag.2020.07.014Haupt M.E., Kwasny M.J., Schechter M.S., McColley S.A. Pancreatic enzyme replacement therapy dosing and nutritional outcomes in children with cystic fibrosis. J. Pediatr. 2014; 164(5): 1110-5.e1. https://doi.org/10.1016/j.jpeds.2014.01.022Haupt M.E., Kwasny M.J., Schechter M.S., McColley S.A. Pancreatic enzyme replacement therapy dosing and nutritional outcomes in children with cystic fibrosis. J. Pediatr. 2014; 164(5): 1110–5.e1. https://doi.org/10.1016/j.jpeds.2014.01.022Khalaf R.T., Green D., Amankwah E.K., Peck J., Carr V., Goldenberg N.A., et al. Percutaneous endoscopic gastrostomy tubes may be associated with preservation of lung function in patients with cystic fibrosis. Nutr. Clin. Pract. 2019; 34(2): 290-6. https://doi.org/10.1002/ncp.10219Khalaf R.T., Green D., Amankwah E.K., Peck J., Carr V., Goldenberg N.A., et al. Percutaneous endoscopic gastrostomy tubes may be associated with preservation of lung function in patients with cystic fibrosis. Nutr. Clin. Pract. 2019; 34(2): 290–6. https://doi.org/10.1002/ncp.10219McDonald C.M., Bowser E.K., Farnham K., Alvarez J.A., Padula L., Rozga M. Dietary macronutrient distribution and nutrition outcomes in persons with cystic fibrosis: an evidence analysis center systematic review. J. Acad. Nutr. Diet. 2021; 121(8): 1574-90.e3. https://doi.org/10.1016/j.jand.2020.03.016McDonald C.M., Bowser E.K., Farnham K., Alvarez J.A., Padula L., Rozga M. Dietary macronutrient distribution and nutrition outcomes in persons with cystic fibrosis: an evidence analysis center systematic review. J. Acad. Nutr. Diet. 2021; 121(8): 1574–90.e3. https://doi.org/10.1016/j.jand.2020.03.016Shaikhkhalil A.K., Freeman A.J., Sathe M. Variations in nutrition practices in cystic fibrosis: a survey of the DIGEST Program. Nutr. Clin. Pract. 2021; 36(6): 1247-51. https://doi.org/10.1002/ncp.10605Shaikhkhalil A.K., Freeman A.J., Sathe M. Variations in nutrition practices in cystic fibrosis: a survey of the DIGEST Program. Nutr. Clin. Pract. 2021; 36(6): 1247–51. https://doi.org/10.1002/ncp.10605

The authors declare that there are no conflicts of interest present.