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The 21st Annual North American CF Conference Session List

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Complete Conference Online Access / CM-ROM Set - Days 1-3 (Digital Audio with synchronized PowerPoint)
Special Educational Online Access / CD-ROM - SHORT COURSES (Digital Audio with synchronized PowerPoint)
Special Online Access / CD-ROMs by Day (Digital Audio with synchronized PowerPoint)

Complete Conference Online Access / CM-ROM Set - Days 1-3 (Digital Audio with synchronized PowerPoint)

SET Complete Conference CD-ROM Set - Days 1-3

Special Educational Online Access / CD-ROM - SHORT COURSES (Digital Audio with synchronized PowerPoint)

SCON Special Educational CD-ROM (Wednesday, October 3, 2007) - 9 SHORT COURSES

Special Online Access / CD-ROMs by Day (Digital Audio with synchronized PowerPoint)

DY1 Day 1 - Thursday, October 4, 2007

DY2 Day 2 - Friday, October 5, 2007

DY3 Day 3 - Saturday, October 6, 2007

P1 From Basic Science to the Clinic: Where Are We & What Is Still Missing

P1-0 Welcome & Introductions

P1-1 Plenary Session I: "From Basic Science to the Clinic: Where Are We & What Is Still Missing?"

S1 CFTR Structure: Putting It All Together

S1-0 Introduction

S1-1 CFTR Structure: What We Can Learn from Electron Microscopy

Structure of the Human CFTR NBD1 Domain as a Homodimer: Insights and Applications

S1-3 Novel Approaches for Expression, Stabilization and Crystallization of CFTR

S1-4 Modeling Full Length CFTR: From Sequence to Structure

Is It All Genetics?

Non-genetic Influences on CF Disease Severity

Air Pollution As a Modifier of Lung Function

Gene-environment Interactions in Susceptibility to Tuberculosis Disease

Interactions Among Environmental and Genetic Factors that Affect CF Lung Disease Severity

Physics of Mucus Transport

Rheology and Optimization of Adhesive Locomotion

Computational Model of Mucociliary Clearance - Relevance to Therapy

Polymer Physics of the Periciliary Layer

Mucociliary Clearance: Physics to Physiology

Pros and Cons: Gastrostomy Tubes in Patients with CF and Acid Suppression Improves PERT Function

We Do Not Need to Use Gastrostomy Tubes (GT) to Improve Outcomes in Patients with CF

We Need to Use Gastrostomy Tubes to Improve Outcomes in Patients with CF

Acid Suppression Improves Effectiveness of Pancreatic Enzyme Replacement Therapy

Use of Acid Suppressive Agents Should Not be a Standard Part of CF Care

Identification of Novel Therapeutic Targets in the Post-genomic Era

The Response of Bronchial Epithelial Cells to Pseudomonas aeruginosa: Beyond Gene Expression Profiling

Therapeutic Targets Defined by Pseudomonas aeruginosa Adaptation to the CF Airway

S5-3 Are Pulmonary Phospholipids and Fatty Acids Attractants or Foods for Pseudomonas aeruginosa?

Neuraminidase Inhibitors to Prevent P. aeruginosa Infection

Pain Management in the Cystic Fibrosis Patient

Pain Management in the Cystic Fibrosis Patient: The Spectrum of Pain in the Hospitalized Patient with CF

Case Studies Panel Discussion

A Developmental Approach to Adolescent Pain

Controversial Issues in Aerosol Therapy

Introduction to Aerosol Science

The Combination of Drug & Device Used for Respiratory Medications Should Be FDA Approved: Pro

The Combination of Drug & Device Used for Respiratory Medications Should Be FDA Approved: Con

Cleaning Controversies

W1 Modifier Genes

W10 Making the CF Diagnosis

W1-0 Introduction

W10-0 Introduction

W10-1 Use and Interpretation of CFTR Mutation Analysis in the Clinical Setting: An International Consensus

W10-2 CF Foundation Consensus on CF Diagnosis

W10-3 Redefining the Diagnostic Parameters for Cystic Fibrosis

W10-4 Abstract 185. The Reference Range for Sweat Chloride Testing and Genotype Analysis for Newborns Who Are Heterozygote Carriers of Cystic Fibrosis

W10-5 Abstract 187. CFTR Screening Does Not Identify All CFTR Coding Region Mutations in CF Patients

W10-6 Abstract 188. Diagnosing Cystic Fibrosis in Adults: Is Sweat Testing Still the Gold Standard?

W10-7 Discussion & Wrap-up

W1-1 Abstract 179. Variants in the Endothelin Receptor A Gene Associate with Increased Expression Levels, Increased Cell Proliferation, and More Severe Pulmonary Disease in CF Females

W1-2 Abstract 180. The Beta-Defensin Repeat Region Affects Cystic Fibrosis Lung Disease Severity

W1-3 Abstract 181. A 6 Megabase Region on the Long Arm of Chromosome 5 Displays Linkage to Cross-sectional and Longitudinal Measures of Cystic Fibrosis Lung Disease Severity

W1-4 Abstract 182. TCF7L2, a Risk Gene for Type 2 Diabetes, Is Associated with Cystic Fibrosis-related Diabetes

W1-5 Abstract 183. Assessing Mucin Gene Polymorphisms to Determine Their Role As Modifiers of Different CF Phenotypes

W1-6 Abstract 184. Assessment of Genetic Modifiers for Phenotypic Severity of Beta-ENaC Transgenic Mice

W1-7 Discussion & Wrap-up

W2-1 Abstract 1. Processing and Function of CFTR-Î"F508 Are Species Dependent

W2-2 Abstract 2. Differences in Interactions of WT and Î"F508 NBD1 with Other Regions of CFTR

W2-3 Abstract 3. SHSPS Target Î"F508 CFTR for ERAD via the Sumo Pathway

W2-4 Abstract 4. Dynasore Prevents CFTR Removal From the Cell Surface

W2-5 Abstract 5. Identification of a Non-native State of NBD1 that Is Affected by Î"F508

W2-6 Abstract 6. CAL-dependent Degradation of CFTR by Trans-golgi Network Localized Snare Protein Syntaxin-6

W2-7 Discussion & Wrap-up

W3 Pushing the Envelope: New Concepts in CF Therapy

W3-0 Introduction

W3-1 Abstract 239. Repeated Administration of Lentiviral Vector to Mouse Nasal Epithelia Enhances Expression without Emergence of Blocking Mucosal Immune Responses

W3-2 Abstract 240. Bronchoscope-guided, Targeted Lobar Aerosolization of HDAD into Nonhuman Primate Lungs Results in Uniform, High Level Pulmonary Transduction, Long Term Transgene Expression and Negligible Toxicity

W3-3 Abstract 241. Airway Stem/Progenitor Cell Integration

W3-4 The Path to the Clinic for VX-770, a Novel CFTR Potentiator

W3-5 Abstract 242. High Throughput Screening Assay for Airway Mucin Secretion

W3-6 CF - HCO3- = Mucoviscidosis

W3-7 Discussion & Wrap-up

W4 CF Airway Defense and Redox State

W4-0 CF Airway Defense & Redox State: Introduction

W4-1 Abstract 143. Regulation of the LPO Antibacterial System Through Hydrogen Peroxide Production

W4-2 DUOX1 in the Airway Epithelium: Role in Epithelial Repair and Remodeling

W4-3 Abstract 144. Neutrophil Defects in Cystic Fibrosis

W4-4 Abstract 146. Dual Effects of Pyocyanin-induced Oxidative Stress on CFTR Chloride Transport

W4-5 Abstract 147. A Role of Glutathione Transport in the Attenuation of Oxidative Injury by Hypertonic Saline

W4-7 Discussion & Wrap-up

W5 Infectious Diseases Management

W5-0 Introduction

W5-1 Abstract 305. Bacterial Microfloral Diversity of the Cystic Fibrosis Lung by 16S Ribosomal RNA

W5-2 Abstract 306. Antibiotic Susceptibility of Anaerobic Bacteria Cultured from the Sputum of Cystic Fibrosis Patients Grown Planktonically and as Biofilms

W5-3 Abstract 307. "Time to Event" Analysis of Efficacy of an Early Aggressive Eradication Protocol for First Growth Pseudomonas aeruginosa in Cystic Fibrosis

W5-4 Abstract 308. Phase 3 Trial (AIR-CF 1) Measuring Improvement in Respiratory Symptoms in Patients with Cystic Fibrosis Following Treatment with Aztreonam Lysine for Inhalation (AZLI)

W5-5 Abstract 309. A Comparison of Oral Chloramphenicol and Oral Ciprofloxacin in the Treatment of Pseudomonas aeruginosa Infections in Patients with Cystic Fibrosis

W5-6 Abstract 310. Sustained Effect of Prophylactic Treatment with IgY Against P. aeruginosa Infections

W5-7 Discussion & Wrap-up

W6 Adult Issues in CF

W6-0 Introduction

W6-1 Testosterone Replacement in Males with CF

W6-2 Abstract 544. Self-reported Treatment Burden in Adults with Cystic Fibrosis

W6-3 Abstract 545. The Estimated Incidence of Patients with Cystic Fibrosis Having a CF Affected Child: A Survey of Centers Throughout the United States

W6-4 Abstract 546. Why Do So Few Men with Cystic Fibrosis Seek Fertility Treatment?

W6-5 Abstract 547. Cardiac Dysfunction in Adult CF Patients with Severe Lung Disease - A Retrospective CoHort Study

W6-6 CF Transition Care...an Adult Issue Also?

W6-7 Discussion & Wrap-up

W7 Medical & Psychosocial Challenges & Solutions to G-tube Placement Throughout the Lifecycle

W7-0 Introduction

W7-1 Abstract 484. Impact of Improved Nutritional Status with Gastrostomy Feedings on Pulmonary Exacerbations in Cystic Fibrosis

W7-2 Diagnosis & Dialogue: The Role of the Physician in the Tube Feeding Decision

W7-3 Family Centered Approach to the Initiation of Gastrostomy Tube Feedings

W7-4 Abstract 485. Percutaneous Endoscopic Gastrostomy (PEG) In Cystic Fibrosis: Effects on Nutrition and Pulmonary Function

W7-5 Challenges to Gastrostomy Tube Placement in Adolescent with Cystic Fibrosis

W7-6 Adults with Advanced Lung Disease and Gastrostomy Tube Placement

W7-7 Discussion & Wrap-up

W8 Assessment and Treatment of Depression in Pediatric and Adult Patients with CF

W8-0 Introduction

W8-1 Abstract 548. Emotional Distress in Parents of Infants with CFTR-related Disorders Diagnosed Through Neonatal Screening

W8-2 Preliminary Results of the National Depression and Anxiety Screening Study in Two CF Centers in Ohio

W8-3 Evidence-based Treatment for Adolescents with Depression

W8-4 Abstract 549. Physical Health and Anxiety Symptoms: Does Monitoring Mediate the Relationship?

W8-5 Comorbidity of Depression and Cystic Fibrosis in Children and Adolescents: Associations with Medical Adherence

W8-6 Levels of Depression and Anxiety in Hispanic Patients with CF and Their Caregivers

W8-7 Discussion & Wrap-up

W9 Time for Airway Clearance

W9-0 Introduction

W9-1 Exercise and Airway Clearance

W9-2 Abstract 431. Measuring Patient Compliance in Inhalation Studies by Smart Card Technology

W9-3 Strategies to Reduce Time for Airway Clearance

W9-4 Abstract 432. Delivery of Hypertonic Saline from an Adaptive Aerosol Delivery Nebulizer and a Conventional Jet Nebulizer

W9-5 Abstract 434. Aerosolization of Liposomal Amikacin (ArikaceTM) Using Different Nebulizers: Selection of the eFlow

W9-6 Abstract 435. Methodologies for Targeting Aerosolized Drugs to the Conducting Airways

W9-7 Discussion & Wrap-up

P2 CF Drug Development: What's New?

P2-0 Plenary Session II: "CF Drug Development: What's New?"

S10 Mucosal Pathogen Interaction

S10-0 Introduction

S10-1 Mucin Structure and Its Function As the Main Trap for Microbes

S10-2 Small Intestinal Bacterial Overgrowth in the CF Mouse: Roles of Mucus and Motility

S10-3 Microbial Community Assembly and Host-microbial Interactions in the Intestine

S10-4 When Pathogen Meets Host-Clues for Development of Novel Therapeutics

S11 Airway Defenses : New Developments in Host-Pathogen Interactions

S11-0 Introduction

S11-1 Hit Them Where They Eat: Antimicrobial Strategies that Target Bacterial Metabolism

S11-2 Polymicrobial Interactions in CF: Synergies and Antagonisms in Host Response

S11-3 Role of CFTR in the Regulation of Intracellular Acidification and Bacterial Killing in Alveolar Macrophages

S11-4 An Oxidant Driven Microbicidal System in Airway Epithelia

S12 Medical, Neurocognitive, and Psychosocial Effects of Sleep Problems in CF

S12-0 Introduction

S12-1 An Overview of Sleep in the CF Literature

S12-2 Sleep Disturbance and Pulmonary Function in CF Patients

S12-3 Sleep in CF - Is It Broken?

S12-4 Sleep Disordered Breathing in CF and Effects of Treatment

S13 Pulmonary and Nutritional Care of the Young Child with CF

S13-0 Introduction

S13-1 Nutritional Issues in Diagnosis and Management of Infants and Young Children with CF

S13-2 Breathing Lessons: How Can We Keep the Pre-school Lung Healthy?

S13-3 Using Diagnostic Tools in the Young Child: X-rays and Pulmonary Function Tests

S13-4 Guiding Parents on the CF Journey

S14 Update on Quality Improvement and Benchmarking

S14-0 Introduction

S14-1 The Adult CF Benchmarking Project: What Have We Learned?

S14-2 CF Quality Improvement: Effect on Outcomes

S14-3 Improving CF Outcomes at Children's Memorial Hospital: A Case Study

S14-4 The CF Foundation Quality Improvement Initiative: Lessons Learned and Future Directions

S8 CFTR: Biogenesis and Post-ER Quality Control

S8-0 Introduction

S8-1 Cotranslational Folding of Ribosome-bound Nascent CFTR Polypeptides

S8-2 Reconstitution and the Identification of New Factors Required for CFTR/Ste6p* Quality Control in the Endoplasmic Reticulum

S8-3 Membrane/Cytoplasmic Domain Contacts in CFTR

S8-4 ESCRTs in Membrane Trafficking and Quality Control

S9 CF Liver Disease: From the Bench to the Bedside

S9-0 Introduction

S9-1 Pathophysiology of CF-associated Liver Disease: Lessons from the Bench

S9-2 Biomarkers and Non-invasive Imaging for CF associated Liver Disease: Search for the Holy Grail

S9-3 Natural History of CF-associated Liver Disease

S9-4 Treatment Options for CF-associated Liver Disease

W11 Pulmonary Guidelines & Care

W11-0 Introduction

W11-1 Abstract 368. Large U.S. Cohort of Young Pseudomonas-negative CF Patients: Mild Lung Disease, Heterogeneous Management

W11-2 Abstract 369. Lung Function Is Diminished in Infants with CF Diagnosed by Newborn Screening Regardless of Pulmonary Infection Detected in Broncho-alveolar Lavage

W11-3 Abstract 370. Comparison of Antibiotics for Early Pseudomonas Infection in CF: Interim Data Analysis

W11-4 Abstract 371. Improvements in Lung Function and Nutritional Outcomes in Cystic Fibrosis

W11-5 Abstract 372. Effect of Key Phamacological Therapies on Decline in Lung Function in Adults with Cystic Fibrosis

W11-6 Abstract 373. Evaluation of a Pulmonary Exacerbation Score in Adults with Cystic Fibrosis

W11-7 Discussion & Wrap-up

W12 CFTR Structure & Protein Interactions

W12-0 Introduction

W12-1 Abstract 7. PKC Phosphorylation Is Necessary for PKA-dependent Binding of the Domain with the Rest of CFTR

W12-2 Abstract 8. Cooperative Domain Assembly Is Essential for CFTR Folding in Living Cells

W12-2B Abstract 10. Interactions with Nascent CFTR as it Exits the Ribosome

W12-3 Relationships Between CFTR Domain Folding and Chloride Channel Activation

W12-4 Abstract 11. ATP Occlusion at the First Nucleotide Binding Domain (NBD1) of CFTR Is Independent of the Second (NBD2) and Therefore of NBD Dimerization

W12-5 Abstract 12. CFTR Structure: What Can We Learn from Electron Microscopy?

W12-6 Discussion & Wrap-up

W13 Biomarkers of Airway Physiology & Host Defense

W13-0 Introduction

W13-1 Abstract 80. Standardization of Mucociliary and Cough Clearance Methodology

W13-2 Abstract 81. Assessment of Induced Sputum as a Tool to Evaluate Anti-inflammatory Agents in CF

W13-3 Abstract 82. UK CF Gene Therapy Consortium Tracking Study: Change in Sputum Properties in Response to IV Antibiotic

W13-4 Abstract 83. A Novel Proteolytically-derived Neutrophil Chemoattractant in Cystic Fibrosis: Role as Biomarker and Possible Therapeutic Target

W13-5 Abstract 84. Novel Mediators of Persistent Neutrophilic Inflammation in Cystic Fibrosis

W13-6 Abstract 85. A Simple and Non-invasive Method to Assess CF Airways Inflammation: Mass Spectrometric Detection of Purines and Urea in Exhaled Breath Condensate

W13-7 Discussion & Wrap-up

W14 Ion Transport & Mucosal Function

W14-0 Introduction

W14-1 Abstract 86. Chloride-bicarbonate Exchange in Human Bronchial Epithelial (HBE) Cells Treated with Interleukin 17A (IL-17A)

W14-2 Abstract 87. Activation of Nasal Epithelial CLC-2 Channels Rescues Chloride Transport in Cystic Fibrosis Mice

W14-3 Abstract 88. Adenosine-mediated Regulation of CFTR and ASL Homeostasis Requires an Intact Cytoskeleton

W14-4 Abstract 89. Dynamics of Liquid Secretion Responses of Porcine Tracheal Submucosal Glands to Acetylcholine and VIP

W14-5 Abstract 92. Protease Inhibitor Regulation of ENaC Function in the Airway: From in vitro to in vivo

W14-6 Abstract 93. Serca Pump Inhibitors Reduce Amiloride-sensitive Sodium Absorption and ENaC Expression

W14-7 Discussion & Wrap-up

W15 Microbial Diversity & Ecology of CF Airways

W15-0 Introduction

W15-1 Abstract 311. Detection of Anaerobic Bacteria in Sputum from Cystic Fibrosis Patients with an Acute Pulmonary Exacerbation

W15-2 Abstract 312. Molecular Identification of Bacteria in the CF Airway

W15-3 Abstract 313. Microbial Community Analysis of CF Sputum Using the Novel 16S rRNA

W15-4 Analysis of the Lung Bacterial Communities Present in Patients at Two Cystic Fibrosis Centers in America and Britain

W15-5 Abstract 314. The P. aeruginosa Burden of the CF Lung - Uncovering the Black Box

W15-6 Abstract 315. PCR Diagnosis of Respiratory Viruses in Children with Cystic Fibrosis

W15-7 Discussion & Wrap-up

W16 Increasing Social Support & Information Exchange Among Patients & Families

W16-0 Introduction

W16-1 Abstract 550. Perceived Social Support for Caregivers of Children with Cystic Fibrosis

W16-2 What Do Parents of Children with CF Want and Need Regarding Emotional and Spiritual Support from Their Healthcare Teams?

W16-3 Abstract 551. Evaluation of a Community Youth Befriending Service for Young People with CF

W16-4 Thank You for Caring, But Don't Tell Me What to Do: Adolescent Perspectives on Social Support from Peers, Family, and Healthcare Teams

W16-5 Abstract 552. Young Adults without Family Support - How Can We Help?

W16-6 Abstract 553. A DVD Presentation - An Adult Cystic Fibrosis Couples Support Group: Reducing Isolation while Living with Life-threatening Illness

W16-7 Discussion & Wrap-up

W17 QI - Lessons Learned

W17-0 Introduction

W17-1 Abstract 463. Improving Evidenced Based Awareness and Practitioner Use of CF Pulmonary Medications

W17-2 Abstract 464. Optimizing Lung Function in People with Cystic Fibrosis Through Consistent and Aggressive Identification and Treatment of Pulmonary Exacerbations

W17-3 Abstract 465. The Impact of Re-education of Airway Clearance Techniques (REACT) on Adherence and Pulmonary Function in Patients with Cystic Fibrosis

W17-4 Abstract 466. Maintain the Gain: Application of Continuous Quality Improvement to Nutritional Cystic Fibrosis Care

W17-5 Abstract 467. Project 4 or More: Improving Clinic Attendance to Four or More Times a Year at Arkansas Children's Hospital Cystic Fibrosis Care Center

W17-6 Abstract 468. Evaluation of Northeastern Ontario's Clinic Round Table Format

W17-7 Discussion & Wrap-up

W18 Infection Control

W18-0 Introduction

W18-1 Abstract 317. Epidemiology of Anterior Nares Colonization with Staphylococcus aereus in Children with CF and Their Household Members

W18-2 Abstract 318. Implementation of an Eradication Protocol and Revision of Infection Control Guidelines to Reduce MRSA Rates in Pediatric Patients with Cystic Fibrosis

W18-3 Abstract 319. Effectiveness of Alcohol-based Cleansers During CF Clinic Visits in Reducing Patient Hand Contamination by Respiratory Pathogens

W18-4 A Practical Approach to Infection Control

W18-5 Barriers to Adherence to Infection Control as Experienced by Healthcare Professionals Compared with CF Patients and Their Families

W18-6 Infection Control or Social Control: The Psychosocial Impact on CF Patient and Their Families

W18-7 Discussion & Wrap-up

W19 GI, Liver, & Nutrition Research

W19-0 Introduction

W19-1 Abstract 486. Treatment with Ursodeoxycholic Acid, But Not with Cholic Acid Increases Bile Flow Independently of Cystic Fibrosis Transmembrane Regulator in Mice

W19-2 Abstract 487. Ursodeoxycholic Acid (UCDA) Therapy in a Congenic CF-Mouse Model of Liver Disease

W19-3 Abstract 488. Altered Eicosanoid Metabolism in the CF Mouse Small Intestine

W19-4 Abstract 489. Reproducibility of the Malabsorption Blood Test (MBT)

W19-5 Abstract 490. Immunomodulatory Properties of Vitamin D in Scandinavian Cystic Fibrosis Patients

W19-6 Abstract 491. Anemia Is a Serious Comorbid Condition in Cystic Fibrosis and Is Associated with Poor Lung Function

W19-7 Discussion & Wrap-up

W20 Multi-disciplinary Update on Lung Transplant: Pre & Post Care

W20-0 Introduction

W20-1 Nutritional Considerations Pre and Post Lung Transplant

W20-2 Abstract 420. CT Scans from CF Patients Screened for Lung Transplant: The Severe Advanced Lung Disease (SALD) CT Scoring System and Correlation with Survival: Preliminary Results

W20-3 Fit for Transplant: Physiotherapy Issues and Optimisation

W20-4 Abstract 421. A Prospective Randomized Trial Comparing Airway Clearance Strategies Following Lung Transplantation

W20-5 Abstract 423. Cystic Fibrosis Quality of Life Comparison Between Male vs. Female Pediatric Lung Transplant Recipients

W20-6 Abstract 424. Lack of Progression of Liver Disease in CF Patients Following Lung Transplantation - Evidence that Lung Transplantation Is Safe in this Group of Patients

W20-7 Discussion & Wrap-up

P3 Improving Patient Outcomes Using the Tools We Have Now

P3-0 Plenary Session III: "Improving Patient Outcomes Using the Tools We Have Now"

S15 Regulation of Airway Surface Liquid

S15-0 Introduction

S15-1 Aquaporins and Their Role in ASL Volume Homeostasis

S15-2 Regulation of Airway ENaCs by Proteases: An Increasingly Complex Story

S15-3 Control of CFTR-mediated ASL Secretion by Adenosine and ENaC

S15-4 Airway Surface Liquid pH and Innate Defense by the NADPH Oxidase

S16 Outcome Measures to Assess CF Interventions

S16-0 Introduction

S16-1 Invasive and Non-invasive Markers of Airway Inflammation

S16-2 Measures of CFTR Molecular and Clinical Function

S16-3 Lower Airway Markers of Infection and Inflammation

S16-4 Measures of Mucociliary Clearance in Assessment of New CF Therapies

S17 The Inflammatory Process in CF: A Complex Picture with Many Players

S17-0 Introduction

S17-1 Revisiting the Role of CFTR Deficiency in Promoting Inflammation

S17-2 Cytokines of the Innate and Adaptive Immune System in CF Lung Inflammation

S17-3 A Unifying Theory to Explain How CFTR Leads to the Lipid Abnormalities in CF and Altered Inflammatory Signaling

S17-4 A Real-life View of Inflammation in CF: Novel Functional Assays for Immune Cell Analysis

S18 Bone Disease: Pathophysiology & Measurements

S18-0 Introduction

S18-1 Pediatric Bone Health: Starting at the Beginning

S18-2 CF Bone Disease: Is Mutant CFTR Really the Culprit?

S18-3 The Value of High-dose Cholecalciferol Supplementation to Replenish Vitamin D in CF

S18-4 The Influence of Pulmonary Infection on Bone Metabolism in Cystic Fibrosis

S19 Clinical Infectious Diseases

S19-0 Introduction

S19-1 Impact of MRSA on Clinical Status in CF

S19-2 Current Antibiotic Resistance Patterns in CF

S19-3 New Antibiotic Strategies for Treating Pulmonary Infection with Pseudomonas aeruginosa

S19-4 The Challenge of Emerging Pathogens in CF

S20 Herding Cats: Coordinating CF Care Today

S20-0 Introduction

S20-1 Living with the Complicated CF Patient: A Parent's Perspective

S20-2 Juggling, Balancing and Other Circus Acts: Orchestrating Pediatric CF Care

S20-3 Coordinating of Care and the Pursuit of Infection Control

S20-4 Research Coordination: How Does it Fit Into the Big Picture?

W21 Newborn Screening & Genetics

W21-0 Introduction

W21-1 Abstract 189. An Improved Newborn Screening Algorithm in Colorado: IRT/IRT/DNA

W21-2 IRT/Mutation Panel/DNA Sequencing - the California Model for CF Newborn Screening

W21-3 Abstract 190. Development of a DNA-based Cystic Fibrosis Newborn Screening Proficiency Testing Program

W21-4 Borderline Sweat Tests in CF Newborn Screening - Diagnostic Dilemmas and Long Term Follow-up

W21-5 Abstract 191. Adolescents and Young Adults with CF: Knowledge of the Genetic Basis of CF and Their Reproductive Risks

W21-6 Abstract 192. Patient Assessment of Genetic Counseling and Cystic Fibrosis Newborn Screening Program at the University of Minnesota Medical Center, Fairview

W21-7 Discussion & Wrap-up

W22 Molecular Mechanisms for CFTR Correction & Potentiation

W22-0 Introduction

W22-1 Abstract 243. Correction of ?F508 CFTR Trafficking Defect by a Novel Family of Marine Sponge Derived Alkaloids

W22-2 Abstract 244. Rescuing ?F508-CFTR Retention with CFTR Derived Peptides Containing the Di-basic RXR Motif (CF-RXR)

W22-3 Abstract 245. Small Molecular Correctors Increase the Surface Stability of Rescued ?F508 CFTR

W22-4 Abstract 246. Design and Synthesis of a Hybrid Potentiator-corrector Agonist of ?F508-CFTR

W22-5 Abstract 247. N6-(2-Phenylethyl)-ATP Potentiates G551D-CFTR by Binding to the Hydrolysis-incompetent Site (NBD1)

W22-6 Abstract 248. VX-770, A Potent, Selective, and Orally Bioavailable Potentiator of CFTR Gating

W22-7 Discussion & Wrap-up

W23 Airways Epithelial Biology

W23-0 Introduction

W23-1 Abstract 94. Differential Expression of Sarcoendoplasmic Reticulum Calcium Atpases (Sercas) in Cystic Fibrosis (CF) Epithelium

W23-2 Abstract 95. Evaluation of the SCN-/H2O2/Lactoperoxidase System as an Antibacterial Defense in Human Airway Epithelium

W23-3 Abstract 96. Flagellin Activates P38, PI3 Kinase, NF-KB and CFTR-dependent Cl Secretion in Airway Gland Epithelial Cells

W23-4 Abstract 97. Hypertonic Challenge Inhibits Sodium Transport in Human Bronchial Epithelial Cells

W23-5 Abstract 98. The Soluble Protein Plunc Regulates ENaC in Human Bronchial Cell Cultures

W23-6 Abstract 99. Prostasin Expression Is Regulated by Airway Surface Liquid Volume and Is Increased in Cystic Fibrosis

W23-7 Discussion & Wrap-up

W24 National/International CF Registries & QI

W24-0 Introduction

W24-1 Public Reporting of Cystic Fibrosis Outcomes: Methods for Case-Mix Adjustment

W24-2 Abstract 447. A Patient and Family Survey to Collect the Socioeconomic Variables in the Cystic Fibrosis Foundation Patient Registry: Methods and Results

W24-3 Abstract 448. Trends in CF Respiratory Microbiology in the U.S., 1995-2005

W24-4 Abstract 449. Multi-slope Models of Lung Disease as an Alternative to Single Slope Models

W24-5 Abstract 450. Predictors of Hospitalization in Cystic Fibrosis: Is There a Gender Gap?

W24-6 European Registry

W24-7 Discussion & Wrap-up

W25 Interactions of Pathogens & the CF Host

W25-0 Introduction

W25-1 Abstract 320. Persistent Nontypeable Haemophilus influenzae Infection Induces Airway Epithelial Tolerance to Pseudomonas aeruginosa

W25-2 Abstract 321. Human Airway Epithelial Cells Facilitate Biofilm Formation by Pseudomonas aeruginosa

W25-3 Abstract 322. The Pseudomonas aeruginosa Biofilm Matrix: A Mannose- and Galactose-rich Exopolysaccharide Is Anchored in a Helical Pattern on the Cell Surface and Acts as an Extracellular Scaffold Holding Biofilm Cells Together

W25-4 Abstract 323. Pseudomonas aeruginosa Induces Rapid Necrotic Killing of PMNs by Quorum Sensing-controlled Production of Rhamnolipid

W25-5 Abstract 324. Pseudomonas aeruginosa in Cystic Fibrosis: Pyocyanin Negative Strains Are Associated with BPI-ANCA and Progressive Lung Disease

W25-6 Abstract 325. Pseudomonas aeruginosa Type III Secretion and Lung Function Decline in Cystic Fibrosis

W25-7 Discussion & Wrap-up

W26 Models of Palliative Care

W26-0 Introduction

W26-1 End of Life: The Importance of Family-centered Care

W26-2 Advance Directives: Interventions at Emory

W26-3 Abstract 554. Advance Care Planning in Adults with CF

W26-4 End of Life Discussion: Is There Still Hope

W26-5 Developing a Palliative Care Approach to CF

W26-6 Discussion & Wrap-up

W27 Patient-Family Centered Care

W27-0 Introduction

W27-1 Abstract 572. Improving Team Communication on the Inpatient Pulmonary Unit

W27-2 Teaching the Newly Diagnosed Adult: Using a Creative Approach

W27-3 Strengthening Partnerships by Engaging Families as Members of the CF Team

W27-4 Passport for Teens: Helping Families Facilitate Transition

W27-5 Teaching Day for Teens

W27-6 Discussion & Wrap-up

W28 Parenting Challenges & Parent Stress: Overcoming Barriers to Adherence to Treatments

W28-0 Introduction

W28-1 Abstract 555. Effects of Caregiver Depression on Self-reported and Electronically Monitored Enzyme Adherence and Short-term Changes in Weight for Children with CF

W28-2 Abstract 556. Challenges of Living with Cystic Fibrosis (CLCF): Psychometric Evaluation of a New Measure

W28-3 Abstract 557. Effects of Behavioral-family Systems Therapy on Family Conflict and Communication in Adolescents with CF and Their Parents

W28-4 Bridging the Gap Between Research and Application: Provider and Parent Perspectives

W28-5 Helping Families of Young Children Meet Care Goals: Case Presentations

W28-6 Helping the Families of School-age Children Meet Care Goals: Case Presentations

W28-7 Discussion & Wrap-up

W29 Fatty Acids & Inflammation: Alterations in Uptake & Metabolism of Fatty Acids in CF

W29-0 Introduction

W29-1 Introduction to Fatty Acid Metabolism

W29-2 Abstract 492. Increased Metabolism of Linoleic Acid Through the N-6 Pathway in Cultured Cells with a Cystic Fibrosis Phenotype Is Corrected by Docosahexaenoic Acid (DHA) Supplementation

W29-3 Abstract 494. Impaired PPAR? Regulation as a Key Player in CF Fatty Acid Imbalance

W29-4 Abstract 495. Decreased PEMT Activity in CF Mice Is Dependent on Cholin Status

W29-5 Abstract 496. Type of Dietary Fat Intake to Serum Fatty Acid Status in Children with CF

W29-6 Discussion & Wrap-up

W30 Physical Activity & Exercise in CF - What Are the Outcomes?

W30-0 Introduction

W30-1 Abstract 436. Effects of Different Conditioning Programs on Quality of Life in CF

W30-2 Abstract 437. Effects of an Exercise Program on Motor Performance in Preschool Children with CF

W30-3 Abstract 438. Motor Performance in Preschool Children with CF Compared to Healthy Children

W30-4 Abstract 439. Reliability and Validity of the Habitual Activity Estimation Scale (HAES) in Patients with Cystic Fibrosis

W30-5 Abstract 440. Long-term Effect of Habitual Physical Activity on Lung Health in Patients with Cystic Fibrosis

W30-6 Abstract 441. Development of an Exercise Knowledge Test for Children with Cystic Fibrosis

W30-7 Discussion & Wrap-up

SC1 Drug Treatment Options in CF Care: Beyond the Basics

SC1-0 Antimicrobial Therapy in Cystic Fibrosis - Beyond the Basics

SC1-1 Use of Antibiotics in Difficult to Manage CF Patients

SC1-2 Treatment and Prevention of early P. aeruginosa infection

SC1-3 MRSA Infection in Cystic Fibrosis

SC1-4 Optimizing Management of Pulmonary Exacerbation in Cystic Fibrosis

SC2 CF Basic Science for the Clinician

SC2-0 Introduction

SC2-1 CFTR: Gene to Protein - How Mutations in CFTR Lead to Cystic FibrosisA Basic Scientist's View

SC2-2 How does CFTR work as a chloride channel, and how do we study its function?

SC2-3 CF Lung Disease

SC2-4 Therapy for Cystic Fibrosis Based on a Rational Understanding of CFTR

SC3 Advanced Motivational Interviewing & CF: A Practical Workshop for Teams

SC3-0 Introductions

SC3-1 Adherence & the therapeutic relationship

SC3-10 The Next Step

SC3-2 Motivational Interviewing REFRESHER

SC3-3 Expressing empathy

SC3-4 Rolling With Resistance

SC3-5 Change & Supporting Self-Efficacy

SC3-6 MI in practice

SC3-7 Information Exchange

SC3-8 Non Diirective Skills

SC3-9 Signs of Resistance

SC4 Quality Improvement in CF Care: Steal Shamelessly, Share Generously

SC4-0 CF Strategic QI Initiative

SC4-1 Overview of Clinical Microsystems Thinking

SC4-2 Getting Started with the Fundamentals

SC4-3 Diagnosis, Improvement Ramp, Aims, FlowchartsCase

SC4-4 Change Concepts, PDSA-SDSA

SC4-5 Smart Change Ideas & Pulmonary Case

SC4-6 Nutrition Case

SC4-7 Engaging Patients and Families

SC4-8 Discussion and Wrap Up

SC5 Implementation of CF Newborn Screening & Current Approaches to Evaluation & Treatment

SC5-0 Introduction

SC5-1 Experience with the Rubric in Washington State

SC5-2 Experience with the Rubric: California

SC5-3 Experience with the Rubric: Challenges in Utah

SC5-4 Completing the communications circle for QA

SC5-5 Family focus after the initial diagnosis

SC5-6 Malnutrition: Prevention and Treatment

SC5-7 Lung Disease: Prevention and Treatment

SC5-8 SC5.8 TBD

SC6 Challenges & Goals for Clinical Research Practice Management

SC6-0 Introduction

SC6-1 The Why, What, and When of SOPs

SC6-2 Best Practices for Study Implementation

SC6-3 Tools for Efficiency

SC6-4 Leadership/Management Balance

SC7 Pulmonary Pathophysiology & Pharmacology for Advanced Practice Nurses

SC7-1 Cystic Fibrosis Lung Disease: pathophysiology, disease complications, and treatment

SC7-2 Pulmonary Function Testing in CF: Infant to Adult

SC7-3 Pulmonary Pharmacology in Cystic Fibrosis

SC7-4 Use & Interpretation of Different Radiology Techniques in Assessing Pulmonary Status in Patients with Cystic Fibrosis

SC7-5 Beyond the Physical - a Holistic Approach to Care

SC7-6 Questions

SC8 Basic ACT for the CF Team

SC8-0 Introduction

SC8-1 Active Cycle of Breathing

SC8-2 Autogenic Drainage

SC8-3 Positive Expiratory Pressure

SC8-4 Oscillating PEP

SC8-5 High Frequency Chest Wall Oscillation

SC8-6 Other Modalities

SC8-7 Review of Literature

SC8-8 Patient Selection

SC8-9 Conclusion

SC9 CF Nutrition 101

SC9-0 Nutrition for Cystic Fibrosis 101

SC9-1 Why is nutrition for CF different than nutrition for the general population?

SC9-2 Vitamins and Minerals

SC9-3 Nutrition in Bone Health in CF

SC9-4 Pancreatic Enzyme Replacement Therapy

SC9-5 Newly Diagnosed CF Patient

SC9-6 Nutrition Assessment - Tube Feedings

SC9-7 Nutrition Management of Cystic Fibrosis Related Diabetes (CFRD)

SC9-8 Nutrition and Port CF

SC9-9 Benchmarking Themes