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Background: Reproductive life planning is key, now that people with cystic fibrosis (pwCF) may live into their 60s. This study explores contraceptive use, pregnancy trends, and whether concomitant cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy reduces contraceptive effectiveness.

Methods: Females with CF aged 18-45 years from 10 U.

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Objective: To develop and try out a convenient and accessible for use technology for testing the pulmonary function in children and adults, including in patients with chronic bronchopulmonary pathology.

Material And Methods: The article presents the results of Lazarev sound-breathing test (LST) in different cohorts of children and adults, including pregnant women and children suffering from chronic bronchopulmonary diseases, in particular cystic fibrosis.

Results: A direct dependence of the LST indicators' level from the age of children and adolescents has been obtained.

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Pregnancy in People With Cystic Fibrosis Treated With Highly Effective Modulator Therapy.

Obstet Gynecol

January 2025

Division of Pulmonary and Critical Care Medicine, the Division of Pediatric Pulmonology, and the Division of Maternal Fetal Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

Article Synopsis
  • New medications called highly effective modulator therapy are improving health for many people with cystic fibrosis (CF), leading to more pregnancies among these individuals.
  • The safety of these medications during pregnancy is unclear, as limited data show potential risks, such as health declines in patients when therapy is stopped and possible cataract development in offspring.
  • Ongoing studies are expected to shed light on these safety concerns, highlighting the importance of thorough counseling for people with CF considering pregnancy.
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As cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies including elexacaftor/tezacaftor/ivacaftor (ETI) have become widely used in eligible patients with cystic fibrosis (CF), the use of these medications in pregnant people has become a critical area of investigation. Since these medications appear generally safe to both mother and fetus when taken by pregnant people with CF, interest has pivoted to the use of ETI in CF carrier mothers to decrease morbidity and mortality from meconium ileus (MI) in fetuses with cystic fibrosis. Here we discuss three infants at our institution with ultrasound findings of MI who were exposed to prenatal ETI through CF carrier mothers for the purposes of treating MI and lowering risk of intestinal complications from this severe manifestation of CF.

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