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Cystic Fibrosis in Adults

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Airway Diseases

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Abstract

Cystic fibrosis (CF) occurs due to a mutation in a single gene encoding the cystic fibrosis transmembrane conductance regulator protein (CFTR) located on chromosome 7 and affects multiple physiological systems. Mutation of the CFTR gene causes either an absence or abnormal function of this transmembrane ion channel on the apical surface of epithelial cells throughout the human body and impacts the movement of several small ions, in particular, Cl- and bicarbonate [1]. Though dysfunction of CFTR can lead to multisystem disease, it is the recurrent pulmonary infections and pancreatic involvement that shapes the clinical course of the disease. The median life expectancy of those with CF has improved over the last few years, thanks to anticipatory care and treatment facilitated through early diagnosis. Established therapies for acute and chronic pulmonary infections, the provision of a high fat, high calorie diet, and follow-up in specialized CF centers by dedicated multidisciplinary teams have all contributed to this increased survival. Over the last 7 decades, CF has changed from being a poorly understood genetic disorder often resulting in death in childhood to a multifaceted disease calling for multidisciplinary management as patients advance into adulthood. According to registry data from multiple countries, the proportion of adult patients now exceeds children (Table 1) [2], an observation realized prior to the advent of CFTR modulators, small molecule therapies enhancing the proper configuration and function of the CFTR protein. The gene implicated in the pathogenesis of CF has been intensively studied, yet there remain significant difficulties in diagnosing and managing CF, particularly in regions of the world where CFTR modulators are not available. Many complications develop in adult patients as their disease progresses. In addition to slowing down or preventing disease progression, such as in childhood, managing disease-related complications (e.g., CF-related diabetes (CFRD), allergic bronchopulmonary aspergillosis (ABPA), chronic airway infections, respiratory failure, pulmonary hypertension, pneumothorax, liver disease, etc.) and treatment-related adverse events (drug toxicity, adverse reactions) requires a comprehensive approach to care in adulthood.

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Authors and Affiliations

  1. Faculty of Medicine, Department of Internal Medicine, Division of Intensive Care, Hacettepe University, Ankara, Turkey

    Berrin Er

  2. Faculty of Medicine, Department of Pulmonary Diseases, Division of Allergy and Clinical Immunology, Hacettepe University, Ankara, Turkey

    Ebru Damadoglu

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  1. Berrin Er
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  2. Ebru Damadoglu
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Correspondence to Berrin Er .

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Editors and Affiliations

  1. Department of Otorhinolaryngology, Eskişehir Osmangazi University, Eskisehir, Türkiye

    Cemal Cingi

  2. Department of Pulmonology, Celal Bayar University, Manisa, Türkiye

    Arzu Yorgancıoğlu

  3. Department of Otorhinolaryngology, Kırıkkale University, Kırıkkale, Türkiye

    Nuray Bayar Muluk

  4. Federal University of Bahia School of Medicine and ProAR Foundation, Salvador - Bahia, Brazil

    Alvaro A. Cruz

Section Editor information

  1. Department of Pulmonology, Gazi University School of Medicine, Ankara, Turkey

    I. K. Oguzulgen MD

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Er, B., Damadoglu, E. (2023). Cystic Fibrosis in Adults. In: Cingi, C., Yorgancıoğlu, A., Bayar Muluk, N., Cruz, A.A. (eds) Airway Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-22483-6_86-1

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