Cystic Fibrosis, An Issue of Clinics in Chest Medicine

Jon Koff

Description

Dr. Jon Koff has assembled and expert team of authors of the topic of Cystic Fibrosis. Articles include: Epidemiology and Pathobiology, Genetics and genetic medicine in Cystic Fibrosis, Innate and Adaptive Immunity in Cystic Fibrosis, Microbiome in Cystic Fibrosis, Diagnostic Testing in Cystic Fibrosis, Treating Pseudomonas in Cystic Fibrosis, Diagnosis of Adult Patients with Cystic Fibrosis, Transition from Pediatrics to Adult Care, Lung Transplant in Cystic Fibrosis, and more!

Sujets

• Médecine
• Spécialités médicales
• Médecine Générale
• Internal Medicine
• Medical

Informations

• Publié par: Elsevier
• Date de parution: 27 février 2016
• Nombre de lectures: 0
• EAN13: 9780323416429
• Langue: English
• Poids de l'ouvrage: 2 Mo

Extrait

Cystic Fibrosis
Clinics In Chest Medicine
Editor
Jonathan L. Koff, MD
Department of Medicine, Yale University, New Haven, CT, USA

Clinics Review Articles

www.chestmed.theclinics.com

March 2016 Volume 37 Number 1
Table of Contents
Cover image
Title page
Copyright
Contributors
Editor
Authors
CME Accreditation Page
Forthcoming Issues
Forthcoming Issues
Recent Issues
Preface. Cystic Fibrosis
Epidemiology of Cystic Fibrosis
Key points
Changes in incidence and prevalence
Cystic fibrosis-related conditions
Diagnosis
Classification of mutant forms of cystic fibrosis transmembrane conductance regulator and their implications
Treatments directed at the basic defect
Clinical manifestations of cystic fibrosis
Outcomes in cystic fibrosis: improvements and challenges
Increasing adult population of cystic fibrosis patients
Cystic fibrosis centers: impact on outcomes
References
Genetics of Cystic Fibrosis: Clinical Implications
Key points
Introduction
Cystic fibrosis transmembrane conductance regulator protein mutations and the disease spectrum
Cystic fibrosis transmembrane conductance regulator protein mutations divided into functional classes
Modifiers
Genetics and diagnosis
Genetics and therapies
Small molecules directed at fixing mutant cftr
Approved agents: (ivacaftor and ivacaftor/lumacaftor)
Adverse events
Emerging therapies
Emerging read-through agents: ataluren
Summary
References
Innate and Adaptive Immunity in Cystic Fibrosis
Key points
Innate immunity
Adaptive immunity
Summary
References
Diagnostic Testing in Cystic Fibrosis
Key points
Introduction
Screening protocols for cystic fibrosis
Diagnostic testing for cystic fibrosis transmembrane conductance regulator protein defects
Diagnostic criteria for cystic fibrosis
Cystic fibrosis transmembrane conductance regulator protein-related metabolic syndrome
Cystic fibrosis transmembrane conductance regulator protein-related disorders
Summary
References
Diagnosis of Adult Patients with Cystic Fibrosis
Key points
Nature of the problem
Diagnostic criteria
Clinical manifestations
Clinical findings
Diagnostic dilemmas
Outcomes for patients with cystic fibrosis diagnosed as adults
Summary
References
The Microbiome in Cystic Fibrosis
Key points
Introduction
Overview of methods and considerations in lung microbiome investigation
The cystic fibrosis respiratory microbiome
Functional features of the cystic fibrosis microbiome
The gastrointestinal microbiome in cystic fibrosis
Future directions in cystic fibrosis microbiome research
References
The Approach to Pseudomonas aeruginosa in Cystic Fibrosis
Key points
Introduction
Prevention
Early eradication therapy
Maintenance therapy for chronic infection
Treatment of acute pulmonary exacerbations
Summary
References
Nontuberculous Mycobacterial Infections in Cystic Fibrosis
Key points
Introduction
Epidemiology
Diagnosis of nontuberculous mycobacteria lung disease in cystic fibrosis
Screening for nontuberculous mycobacteria in the cystic fibrosis population
Treatment of nontuberculous mycobacteria lung disease
Nonpharmacologic treatment options
Surgical treatment options
Treatment outcomes and impact of nontuberculous mycobacteria lung disease
Recommendations for follow-up
Lung transplantation in cystic fibrosis and nontuberculous mycobacteria
Infection prevention
Summary/discussion
References
Nutritional Issues in Cystic Fibrosis
Key points
Introduction
Effect of nutritional status on cystic fibrosis lung disease and survival
Lung transplantation and nutritional status
Pathogenesis of disordered nutrition in cystic fibrosis
Nutritional assessment
Energy intake, vitamin and mineral supplementation
Behavioral consideration
Oral supplementation
Appetite stimulation
Enteral supplementation
Complications of cystic fibrosis
Summary
References
Gastrointestinal Disorders in Cystic Fibrosis
Key points
Introduction
Gastroesophageal reflux disease
Pancreatic insufficiency and pancreatitis
Dysmotility and small intestinal bacterial overgrowth
Distal intestinal obstruction syndrome and constipation
Gastrointestinal malignancy
Summary and future considerations
References
Cystic Fibrosis Transitions of Care: Lessons Learned and Future Directions for Cystic Fibrosis
Key points
Development and implementation of a Cystic Fibrosis Care Center Network transition policy
Improving cystic fibrosis transitions
Clinical outcomes: measuring success in transitions
Transition as a quality improvement process
Future needs and current capacity for measuring quality improvement activities
Summary
Acknowledgments
References
Lung Transplantation for Cystic Fibrosis
Key points
Background
Guidelines for referral and evaluation
Selection of candidates for lung transplantation
Type of transplantation
Comorbidities in cystic fibrosis and impact on transplant candidacy and outcomes
Mechanical support as a bridge to lung transplantation
Survival and quality of life concerns
Opportunities for improved transplant outcomes
Summary
References
Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis
Key points
Introduction
Pathophysiology
Patient evaluation
Pharmacologic treatment options
Nonpharmacologic treatment options
Surgical treatment options
Experimental therapies
Summary/discussion
References
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance
Key points
Introduction
Experimental data showing acquired cystic fibrosis transmembrane conductance regulator dysfunction from smoking
Acquired cystic fibrosis transmembrane conductance regulator dysfunction in patients with chronic obstructive pulmonary disease
Mediators of cystic fibrosis transmembrane conductance regulator dysfunction in cigarette smoke
Secondhand smoking
Acquired cystic fibrosis transmembrane receptor dysfunction and asthma
Therapeutic approaches
Summary
Funding
Conflicts of interest
References
Index
Copyright
ELSEVIER
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CLINICS IN CHEST MEDICINE Volume 37, Number 1
March 2016 ISSN 0272-5231, ISBN-13: 978-0-323-41641-2
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