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J Cyst Fibros. 2019 Oct;18:S95-S104. doi: 10.1016/j.jcf.2019.08.024.

Female reproductive health in cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

Kara S Hughan, Tanicia Daley, Maria Socorro Rayas, Andrea Kelly, Andrea Roe

Affiliations

  1. Division of Pediatric Endocrinology and Diabetes, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, 4401 Penn Avenue, Pittsburgh, PA, USA. Electronic address: [email protected].
  2. Division of Pediatric Endocrinology and Metabolism, Emory Children's Pediatric Institute, Emory University School of Medicine, 1400 Tullie Road, Atlanta, GA, USA.
  3. Division of Pediatric Endocrinology and Diabetes, University of Texas Health San Antonio, 7703 Floyd Curl, San Antonio, TX, USA.
  4. Division of Pediatric Endocrinology and Diabetes, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, 2716 South Street, Philadelphia, PA, USA.
  5. Division of Family Planning, Department of Obstetrics and Gynecology, Perelman School of Medicine at the University of Pennsylvania, 1000 Courtyard, 3400 Spruce Street, Philadelphia, PA, USA.

PMID: 31679735 DOI: 10.1016/j.jcf.2019.08.024

Abstract

Women with cystic fibrosis (CF) are living longer and healthier lives, and opportunities for childbearing are increasingly promising. However, this population can also face sexual and reproductive health concerns, including menstrual irregularities, unplanned pregnancies, infertility and pregnancy complications. Additionally, more women are entering menopause and are at risk for the consequences of estrogen deficiency. The exact mechanisms involved in female reproductive health conditions in CF are not clearly understood, but are thought to include cystic fibrosis transmembrane regulator (CFTR)-mediated abnormalities, changes in female sex hormones, and other CF health-related factors. In the era of CFTR modulator therapy, new data are necessary to understand the impact of CFTR modulation on contraceptive effectiveness, fertility, and pregnancy outcomes to help guide future clinical care. This article reviews the current scientific knowledge of major reproductive health issues for women with CF.

Copyright © 2019 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Keywords: Amenorrhea; Contraception; Cystic fibrosis; Fertility; Menstruation; Pregnancy; Reproductive health

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