Advances in Cystic Fibrosis, An Issue of Clinics in Chest Medicine

1st Edition, Volume 43-4 - November 9, 2022
Editors: Clemente J. Britto, Jennifer L. Taylor-Cousar
Language: English
Hardback ISBN:
9 7 8 - 0 - 3 2 3 - 8 4 9 6 1 - 6
eBook ISBN:
9 7 8 - 0 - 3 2 3 - 8 4 9 6 2 - 3

In this issue of Clinics in Chest Medicine, guest editors Drs. Clemente J. Britto and Jennifer L. Taylor-Cousar bring their considerable expertise to Advances in Cystic Fibrosis.… Read more

Advances in Cystic Fibrosis, An Issue of Clinics in Chest Medicine

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In this issue of Clinics in Chest Medicine, guest editors Drs. Clemente J. Britto and Jennifer L. Taylor-Cousar bring their considerable expertise to Advances in Cystic Fibrosis. Top experts in the field cover key topics such as genetics of cystic fibrosis (CF): clinical implications; innate and adaptive immunity in CF; novel applications of biomarkers and personalized medicine to define the spectrum of CF; emerging approaches to understand CF pathogenesis; and more.

Cover image
Title page
Table of Contents
Copyright
Contributors
Forthcoming Issues
Preface
Toward a Broader Understanding of Cystic Fibrosis Epidemiology and Its Impact on Clinical Manifestations
Key points
Introduction
Diagnosis
Incidence
Prevalence
Variability in phenotype
Survival
Changing outcomes and clinical manifestations
Summary
Clinics care points
Genetics of Cystic Fibrosis: Clinical Implications
Key points
Introduction
Classification of cystic fibrosis transmembrane conductance regulator variants by molecular mechanism
Genotype–phenotype correlation
Diagnostics
Treatment
Summary
Clinics care points
Update on Innate and Adaptive Immunity in Cystic Fibrosis
Key points
Immune barrier function and host defense in cystic fibrosis lungs
Altered innate immune responses and hyper-inflammation in cystic fibrosis
Phagocyte function and host defense in cystic fibrosis
Adaptive immunity in cystic fibrosis
Potential new players modifying the immune response in patients with cystic fibrosis
Immunity and modifier genes in cystic fibrosis
Summary
Clinics care points
Novel Applications of Biomarkers and Personalized Medicine in Cystic Fibrosis
Key points
Introduction
Discussion
Summary
Clinics care points
Emerging Approaches to Monitor and Modify Care in the Era of Cystic Fibrosis Transmembrane Conductance Regulators
Key points
Introduction
Monitoring clinical status in the highly effective modulator therapy-treated patient
Summary
Clinics care points
The Impact of Highly Effective Modulator Therapy on Cystic Fibrosis Microbiology and Inflammation
Key points
Introduction: cystic fibrosis and cystic fibrosis transmembrane conductance regulator modulators
Pulmonary damage in CF is due to interactions between airway inflammation, infection, abnormal anion secretion, and impaired mucociliary clearance
Changes in inflammation and infection after initiation of highly effective cystic fibrosis transmembrane conductance regulator modulator therapy
Effects of highly effective cystic fibrosis transmembrane conductance regulator modulator therapy on inflammation and infection in human subjects
Concluding remarks
Clinics care points
Novel Approaches to Multidrug-Resistant Infections in Cystic Fibrosis
Key points
Introduction
Discussion
Clinics care points
Clinics care points
Clinics care points
Clinics care points
Summary
Financial support
Update on Clinical Outcomes of Highly Effective Modulator Therapy
Key points
Introduction
Summary of controlled trials of ivacaftor in adults and adolescents with cystic fibrosis
Real-world observational studies of ivacaftor in adults and adolescents with cystic fibrosis
Summary of controlled trials of elexacaftor, tezacaftor, and ivacaftor in adults and adolescents with cystic fibrosis
Real-world observational studies of elexacaftor, tezacaftor, and ivacaftor in adults and adolescents with cystic fibrosis
Studying cystic fibrosis transmembrane conductance regulator modulators in young children and infants with cystic fibrosis
Summary of controlled trials of ivacaftor in young children with cystic fibrosis
Real-world observational studies of ivacaftor in young children with cystic fibrosis
Summary of controlled clinical trials of elexacaftor, tezacaftor, and ivacaftor in young children with cystic fibrosis
Real-world observational studies of elexacaftor, tezacaftor, and ivacaftor in young children with cystic fibrosis
Broader considerations prompted by cystic fibrosis transmembrane conductance regulator modulator trial results
Summary
Clinics care points
Author disclosures
Nontuberculous Mycobacterial Infections in Cystic Fibrosis
Key points
Introduction
Infection prevention
Summary
Clinics care points
Non-Modulator Therapies: Developing a Therapy for Every Cystic Fibrosis Patient
Key points
Introduction
Clinics care points
Disclosure
Updates in Nutrition Management of Cystic Fibrosis in the Highly Effective Modulator Era
Key points
Introduction
Discussion
Summary
Clinics care points
Update in Advancing the Gastrointestinal Frontier in Cystic Fibrosis
Key points
Introduction
Gastrointestinal manifestations of cystic fibrosis
Summary
Clinics care points
Transitions of Care in Cystic Fibrosis
Key points
Introduction
Barriers and challenges associated with cystic fibrosis transition
Models of transition/landscape of transition
Establishment of formal, standardized programs, and models of care
Mechanisms to enhance the training and establishment of programs for transition
Transition in post–COVID-19 era/future directions
Summary
Clinics care points
Endocrine Complications of Cystic Fibrosis
Key points
Introduction
Cystic fibrosis-related diabetes
Cystic fibrosis-related bone disease
Growth restriction in cystic fibrosis
Summary
Clinics care points
Management of Mental Health in Cystic Fibrosis
Key points
Background
Clinical relevance
Therapeutic options
Discussion
Clinics care points
Disclosure
Family Planning and Reproductive Health in Cystic Fibrosis
Key points
Introduction
Sexual activity and function
Sexually transmitted infections and contraception
Pregnancy planning
Fertility
Assisted reproductive technology
Pregnancy
Parenthood
Conclusions and care recommendations
Clinics care points
Disclosure
Update on Lung Transplantation for Cystic Fibrosis
Key points
Introduction
Summary
Clinics care points
Disclosure

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Advances in Cystic Fibrosis, An Issue of Clinics in Chest Medicine

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Clemente J. Britto

Jennifer L. Taylor-Cousar