Table of Contents

CYSTIC AND IDIOPATHIC PULMONARY FIBROSIS RISK FACTORS, MANAGEMENT AND LONG-TERM HEALTH OUTCOMES ; CYSTIC AND IDIOPATHIC PULMONARY FIBROSIS RISK FACTORS, MANAGEMENT AND LONG-TERM HEALTH OUTCOMES ; CONTENTS ; PREFACE; Chapter 1 CLINICAL MANIFESTATIONS OF CYSTIC FIBROSIS AND THEIR MANAGEMENT ; Abstract; List of Abbreviations; Introduction; Epidemiology; Molecular Biology; CFTR Gene; CFTR Biosynthesis; Structure of CFTR; Overview of CFTR Function; Pathophysiology; Types of Mutations; Class I Mutations; Class II Mutations; Class III Mutations; Class IV Mutations; Class V Mutations Class VI MutationsEffect of CFTR Dysfunction on Various Tissues; Sweat Glands; Lung and Small Airways; Paranasal Sinuses; Pancreas; Intestines; Liver and Gallbladder; Reproductive Tract; Bones; Other Organ Systems; Management; Clinical Features; Differential Diagnosis; Overview of Treatment; Treatment of Pulmonary Disease; Treatment of Gastrointestinal Disease; Treatment of Endocrine Abnormalities; Other Aspects of Treatment; Novel Treatment Strategies; Outcome Measures; Pulmonary Function Testing; Imaging-Based Measures; Sputum Biomarkers; Blood-Based Biomarkers; Potential Difference Clinical Trials of Ivacaftor in Patients with CFTR Mutation Other Than G551DTargeted Therapies for Patients with Phe508del CFTR Mutation; Targeted Therapies for Patients with Class I Mutation; Gene Therapy; Conclusion; References; Biographical Sketch; Chapter 5 DISTINCT PATTERNS OF PULMONARY INJURY AND FIBROSIS INDUCED BY INTRATRACHEAL AND SUBCUTANEOUS BLEOMYCIN IN THE MOUSE: RELEVANCE FOR DISTINCT FORMS OF HUMAN LUNG FIBROSIS ; Abstract; List of Abbreviations; Introduction; Results Selection of Patients for Lung TransplantationCriteria for Referral; Contraindications; Pre-Transplant Evaluation; Listing for Transplantation; Preparation for Transplantation; Surgical Issues and Technical Considerations; Post-Transplant Management; Peri-Operative Management and Complications; Delayed Onset and Chronic Complications; Post-Transplant Surveillance; Outcomes; Conclusion; References; Chapter 4 NEW CYSTIC FIBROSIS THERAPY ; Abstract; Introduction; Evolution of CF Therapy; Nutritional Management; Clinical Trials of Ivacaftor in Patients with the Gly551Asp CFTR Mutation Sweat Chloride TestPrognosis and Risk Factors; Conclusion; Conflict of Interests; References; Biographical Sketch; Chapter 2 LIVER DISEASE IN CYSTIC FIBROSIS: PREVALENCE, RISK FACTORS, AND OUTCOMES ; Abstract; Introduction; Objective; Methods; Screening for Liver Disease; Physical Examination; Liver Biochemistry Studies; Echographic Examination; Hepatobiliary Scintigraphy; Analysis of Clinical and CFTR Genotype Data; Statistic Analysis; Results; Discussion; Conclusion; References; Chapter 3 LUNG TRANSPLANTATION FOR CYSTIC FIBROSIS ; Abstract; Introduction