Curriculm Vitae Frank J. Accurso, M.D

Curriculm Vitae – Frank J. Accurso, M.D.

CURRICULUM VITAE

1. PERSONAL HISTORY Name: Frank J. Accurso, M.D. Date and place of birth: March 9, 1947; Mount Vernon, New York, USA Present position: Professor Department of Pediatrics University of Colorado Denver School of Medicine Present address: The Children's Hospital 13123 East 16th Avenue (B395) Aurora, CO 80045 Phone: 720-777-2522 Fax: 720-777-7284 E-mail: [email protected] Citizenship: USA

2. EDUCATIONAL HISTORY City College of New York, New York City, B.S. 1964-1968 University of Colorado, Boulder, Colorado, M.S. 1968-1971 Albert Einstein College of Medicine, New York City, M.D. 1971-1974 University of Colorado Health Sciences Center, Denver, Colorado 1974-1977 Residency (Pediatrics) University of Colorado Health Sciences Center, Denver, Colorado 1978-1980 Fellowship (Pediatric Pulmonology)

3. LICENSURE Colorado, #21169 Montana, #7309

4. BOARD CERTIFICATIONS National Board of Medical Examiners #145748 July 1, 1975 - present Pediatrics #024091 May 5, 1980 - present Pediatric Pulmonology #0009 July 18, 1986 - present Pediatric Critical Care Medicine #0009 July 10, 1987 – 2002

5. PROFESSIONAL POSITIONS A. Academic appointments: University of Colorado School of Medicine, Denver, Colorado Instructor of Pediatrics 1979 Assistant Professor of Pediatrics 1980 Associate Professor of Pediatrics 1987 Professor of Pediatrics 1995-present Section Head, Pediatric Pulmonary Medicine 2003-present Professor of Preventive Medicine and Biometrics 2003-present Graduate Faculty, UCDHSC Clinical Science Program 2005-present Professor of Physiology and Biophysics 2006-present Graduate Faculty, Medical Scientist Training Program (MD/PhD) 2005- present

B. Medical Staff Appointments: The Children’s Hospital, Denver, Colorado. University Hospital, Denver, Colorado.

Memorial Hospital, Colorado Springs, Colorado. . St. Vincent’s Hospital, Billings, Montana.

1 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

6. AWARDS, HONORS, AND SPECIAL RECOGNITION A. Awards: Special Recognition Teaching Award - Medical Student Council, 1988 Service Awards – Denver Chapter of the Cystic Fibrosis Foundation, 1991, 1992 Honorary Co-Chairman, Cystic Fibrosis Foundation North American Meeting, 1999, 2006 John E. Strain Advocacy for Children Award - Denver Children’s Hospital Medical Staff, 2000 Kit Taylor Memorial; Twenty-first Annual Lecturer – University of Rochester, 2001 Minnie C.T. Love Outstanding Physician Award – Denver Children’s Hospital Foundation, 2002 Doris Tulcin Award for Research in Cystic Fibrosis – University of Alabama, 2002 Outstanding Service Award – Clinical Science Program – University of Colorado, 2003 Paul Di Sant’Agnese Award for Scientific Achievement in Cystic Fibrosis, Cystic Fibrosis Foundation, 2003. Outstanding Affiliated/Clinical Faculty Award - Department of Biometrics and Preventive Medicine, University of Colorado, 2003 Outstanding Research Mentor Award- Clinical Science Program, University of Colorado, 2004 Career Teaching Scholar, Department of Pediatrics, University of Colorado Health Sciences Center, 2006. Breath of Life Award, Denver Chapter of the Cystic Fibrosis Foundation, 2008. Physician of the Year, Colorado Rocky Mountain Chapter of the Pediatric Nurse Practitioners Association of North America, 2009.

B. Honors: Phi Beta Kappa, Magna Cum Laude, City College of New York, 1968 Parker B. Francis Foundation Pediatric Pulmonary Fellow, 1978 Best Doctors in America, 1994 - present Best Doctors in the Midwest, 1997 Best Doctors in Denver (5280 Magazine) 1997 - present Best Pediatricians in America, 2002 Castle Connolly Guide to America’s Top Doctors, 2002 - present Who's Who in America, 2009

7. MEMBERSHIPS IN PROFESSIONAL SOCIETIES AND OTHER AFFILIATIONS A. Professional Societies: American Thoracic Society Western Society for Pediatric Research American Association for the Advancement of Science Society for Pediatric Research American Pediatric Society

B. Affiliations: Adjunct Scientist, NIH, Bethesda, Maryland, Laboratory for Developmental Biology, NIDR, 1991-1992. Center for Computational Biology, University of Colorado Denver, 2001-2007 C. Consortia: Cystic Fibrosis Genotype Consortium Cystic Fibrosis Phenotype-Genotype Consortium D. Consultant: Colorado Department of Public Health, Newborn Screening Committee.

8. INSTITUTIONAL COMMITTEES AND RESPONSIBILITIES A. Service Committees: Pharmacy and Therapeutics Committee, 1980 - 1984. Co-Director, Pediatric Intensive Care Unit, 1980 - 1987. Associate Director, Cystic Fibrosis Center, 1980 - 1987. Pediatric Intensive Care Unit Remodeling Committee, 1981. Pediatric Clinical Research Center Advisory Committee, 1984 - 1986. Pediatric Residency Program Director, 1987 - 1988. Director, Cystic Fibrosis Center, 1987 to present. 2 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

Director, Pediatric Respiratory Therapy, 1987 - 1990. Director, Pediatric Inpatient Service, 1988 - 1989. Pediatric Quality Assurance Committee, 1988 - 1989. Pediatric Clinical Research Center Computer Advisory Committee, 1990-1991. Lung Transplant Committee, 1990. Director Pediatric Pulmonary Fellowship Training Program, 1992 - 1997 National Jewish Hospital/The Children's Hospital/University Hospital/CU School of Medicine- Feasibility of Program Relocation Task Force, Inpatient and Outpatient Task Force, 1994. Pediatric Residency Selection Committee, 1994 - 1996. School of Medicine Resesarch Retreat, Bioinformatics Committee, 2001. Department of Pediatrics, Fitzsimmons Relocation Planning Committee, Subcommittee on Clinical Research, 2002. Department of Pediatrics, Bioinformatics Planning Committee, 2002. Department of Pediatrics, Grants and Contracts Committee, 2003 Head, Department of Pulmonary Medicine, Children’s Hospital, 2003-2009 Section Head, Pediatric Pulmonary Medicine, University of Colorado School of Medicine, 2003- present. Dean’s Research Advisory Committee, School of Medicine, 2005-2006 Junior Faculty Research Retreat, Chairman, Planning Committee, 2005 Colorado Pulmonary Bioscience Day, planning committee, University of Colorado Denver School of Medicine, 2009

B. Clinical Sciences Graduate Program: Admission Committee, 2001 - 2007 Curriculum Committee, 2001 - 2003 Instructor “Introduction to Laboratory Medicine for Clinical Investigators” CLSC 7651, 2001- 2006. Instructor “Guided Research Tutorial” CLSC 6655, 2006- present

C. Dissertation Committees: Student Degree Department Year Institution Kathy Hirst Ph.D. Biometrics 1990 Univ. of Colorado HSC Nikki Carroll M.S. Biometrics 1994 Univ. of Colorado HSC Marci Sontag M.S. Biometrics 1996 Univ. of Colorado HSC Bristol Harris M.S. Pediatrics 1999 Univ. of Colorado HSC Judy Maselli M.S. Biometrics 2001 Univ. of Colorado HSC Kathe Bjork Ph.D. Biometrics 2003 Univ. of Colorado HSC Marci Sontag Ph.D. Biometrics 2004 Univ. of Colorado HSC Sonya Heltshe M.S. Biometrics 2005 Univ. of Colorado HSC. Neil Goldenberg* Ph.D. Clinical Science 2006 Univ. of Colorado HSC Scott Sagel* Ph.D. Clinical Science 2006 Univ. of Colorado HSC Terri Laguna M.S. Clinical Science 2006 Univ. of Colorado HSC Edith Zemanick M.S. Clinical Science 2006 Univ. of Colorado HSC J. Tod Olin MS Clinical Science 2011 Univ. of Colorado HSC *Chair

D. Fellow Scholarly Oversight Committee Amy Gottlieb MD Obstetrics 2007 Univ of Colorado HSC Casey Burg MD Pediatrics 2009 Univ of Colorado Denver Jonathon Popler MD Pediatrics 2009 Univ. of Colorado Denver Stacey Martiniano MD Pediatrics 2010 Univ. of Colorado Denver

9. PATENTS: None

10. NATIONAL AND INTERNATIONAL COMMITTEE, REVIEW AND CONSULTANT WORK A. Journals – Guest Referee:

Pediatric Research, Journal of Reproductive Medicine, Western Journal of Medicine , Biology of the Neonate, American Review of Respiratory Disease, American Journal of Physiology, Journal of Applied Physiology, Pediatric Pulmonology, Pediatrics, Journal of Pediatrics, American Journal of Human Genetics, Critical Care Medicine, American Journal of Respiratory and Critical Care Medicine, Acta Paediatrica, Journal of Allergy and Clinical Immunology, Journal of Clinical Investigation, Thorax, New England Journal of Medicine, Journal of the American Medical Association, Nature Medicine, Proceedings of the National Academy of Sciences, Public Library of Science – One, Experimental Biology and Medicine, American Journal of Respiratory Cell and Molecular Biology, Journal of Cystic Fibrosis, Chest, PLOS one, Science Translational Medicine.

B. Editorial Boards Advances in Pediatrics, 2003-2005 American Journal of Respiratory and Critical Care Medicine, 2004-2007 Deputy and Associate Editor

C. Review and Committee Work - National: American Lung Association - Ad hoc grant reviewer, 1984. American Thoracic Society - Nominating Committee, 1996. Pediatric Pulmonology Training Program Directors Association - Treasurer, 1996-1998. American Board of Pediatrics - Contributor first and second certifying examinations in Pediatric Pulmonology, 1985, 1988. American Academy of Pediatrics - Newborn Screening Guidelines Committee, 2001-2006

D. Review and Committee Work – International: Canadian Cystic Fibrosis Foundation, 1998, 2002, 2005, 2006, 2008 Prevention Programme of the Dutch Health Research and Development Council, 2000. Association Francaise de la Lutte contre la Mucoviscidose, France, 2000, 2001. World Health Organization – Workshop on ICD 11 Codes for Cystic Fibrosis and Related Disorders, Stockholm, Sweden, June 2000. Italian Cystic Fibrosis Research Foundation, 2003 Australian National Health and Medical Research Council, 2005 Bayer Healthcare Pharmaceuticals, Partnership Fund, 2007

E. NIH Activities: NCRR GCRC Site Visit Study Section Member, 1989, 1995, 1999 NHLBI Division of Lung Diseases. Invited Workshop Participant: Effects of Growth and Development on Lung Function: Models for Study of Childhood Asthma, 1996 NHLBI Ad Hoc Review Committee, Cystic Fibrosis Gene Therapy Centers, 1998. NICHD Invited Workshop Participant: Surrogate Markers in Pediatrics, 1998. NIDDK Special Study Section for Cystic Fibrosis Centers, 2000, 2008 NIDDK Ad hoc Reviewer for SBI grant program, 2000 NICHD Ad hoc Reviewer for Maternal and Child Health Research Subcommittee, 2002. NHLBI Ad hoc Reviewer for SCOR Program in Cystic Fibrosis, 2003 NIDDK Ad hoc Reviewer for Gene Therapy Cores, 2003 NHLBI Ad hoc Reviewer for Program Projects in Gene Therapy, 2003 NHLBI Special Emphasis Panel (K Awards), 2004 – present. NHLBI, NIDDK Joint Ireland-US Conference on Cystic Fibrosis, 2006. NHLBI Program Project Review Committee, 2007 NHLBI Pediatric Respiratory Disease Planning Meeting, 2008 NIDDK Special Emphasis Panel, 2008 NHLBI Program Project Review Committee, 2008 NHLBI Program Project Review Committee, 2009 NHLBI Special Emphasis Panel, Center Grants, 2009. NHLBI Special Emphasis Panel, T32 Institutional Training Grants, 2010

F. Cystic Fibrosis Foundation Activities. Professional Education Committee, 1987 - 2007

Ad hoc Reviewer for the Research/Research Training Committee, 1988, 1991, 1994, 1995, 1996. North American Meeting Program Committee, 1994 - present. Clinical Research Committee, 1997-1999. Co-chairman, Workshop on Infant Lung Function in CF, 1998. Clinical Research Committee, Chairman, 1999 - 2003. Therapeutics Development Network, Medical Director, Inflammatory Mediator Core Laboratory, 1999-present. Therapeutics Development Network, Protocol Review Committee, 1998 - 2004 Therapeutics Development Network, Long Range Planning Committee, 2001-2002. Therapeutics Development Network, Chairman of Task Force Committee on Personnel, 2001-02. Medical Advisory Council, 1999-present, Chairman, 2003-present. Board of Directors, Cystic Fibrosis Foundation Therapeutics Inc., 2000 – present. Scientific Advisory Board, Macrolide Antibiotic Trial, 2001-2002. Williamsburg Scientific Meeting Planning Committee, 2001-2002. Scientific Advisory Board, Early Pseudomonas Intervention Trial, Chairman, 2003-2004 Board of Trustees, 2003-present. Co-chairman, Workshop on Implementation of Newborn Screening for CF, 2004 Research and Development Program Review Committee, 2007 Participant, Workshop on Diagnosis of CF in the Era of Newborn Screening, 2007 Co-chairman, Workshop on Infant Care in CF, 2007 Clinical Research Executive Committee, 2007 - present External Advisory Panel, Cystic Fibrosis Research and Development Program, University of Washington, Seattle, Washington. 2006 - present External Advisory Panel, Cystic Fibrosis Research and Development Program, Cincinnati Childrens Hospital and Medical Research Center, 2008 – 2010. PTC/CFFT Clinical Development Advisory Committee 2008-present Sweat Rate Consortium Advisory Committee 2008-present

G. Industry - Consultation and/or Advisory Boards: Genentech Advanced Respiratory Pathogenesis/Chiron BioStar Targeted Genetics Genzyme Cortech Cortecs, Ltd. Bioproducts General Magainin NexStar Dupont Genzyme Vertex Centocor Hollis-Eden Novartis Digestive Care Vertex Syngenta AirPharma Kalobios Ampex N30 PTC

11. GRANTS AND CONTRACTS A. Clinical: Cystic Fibrosis Care Center Grant. Center Director - F. Accurso, M.D. Cystic Fibrosis Foundation #C017. 2010-2011 $188,740 2009-2010 $186,020 2008-2009 $216,300 2007-2008 $211,605 2006-2007 $203,307 2005-2006 $196,626 2004-2005 $229,490 2003-2004 $221,254 2002-2003 $200,958 2001-2002 $179,751 2000-2001 $173,550 1999-2000 $162,299 1998-1999 $142,184 1997-1998 $120,403 1996-1997 $112,538 1995-1996 $ 97,208 5 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

1994-1995 $ 74,771 1993-1994 $ 76,375 1992-1993 $ 61,497 1991-1992 $ 54,923 1990-1991 $ 49,161 1989-1990 $ 42,824 1988-1989 $ 39,192 1987-1988 $ 36,399

B. Training: 1. Cystic Fibrosis Foundation Clinical Fellowships in Pediatric Pulmonology. Sponsor: F. Accurso, M.D. Trainee Fellowship Years Award Tod Olin, MD 2nd 2009-2010 $49,750 Tod Olin, MD 1st 2008-2009 $48,000 Livia Veress, MD 2nd 2008-2009 $49,750 Edith Zemanick, MD 4th 2007-2008 $62,500 Livia Veress, MD 1st 2007-2008 $43,750 Theresa Laguna, MD 4th 2006-2007 $62,500 Jerri Lysinger, MD 2nd 2006-2007 $43,750 Jerri Lysinger, MD 1st 2005-2006 $42,000 Edith Zemanick, MD 1st 2004-2005 $42,500 Theresa Laguna, MD 1st 2003-2004 $42,000 Scott Sagel, MD 4th 2002-2003 $62,600 Greg Montgomery, MD 1st 2002-2003 $42,000 Keith Cavanaugh, MD 2nd 2002-2003 $45,000 Jamie Wooldridge, MD 4th 2001-2002 $62,600 Keith Cavanaugh, MD 1st 2001-2002 $42,000 Monica Jones, MD 1st 2000-2001 $36,000 Jamie Wooldridge, MD 3rd 2000-2001 $55,000 Jamie Wooldridge, MD 2nd 1999-2000 $40,000 Scott Sagel, MD 1st 1999-2000 $36,000 Jamie Wooldridge, MD 1st 1998-1999 $36,000 Theresa Guilbert, MD 1st 1996-1997 $30,000 Ronald Morton, MD 1st 1995-1996 $30,400 Gwendolyn Kerby, MD 1st 1994-1995 $30,400 Andrew Gelfand, MD 2nd 1994-1995 $32,400 Steven Copenhaver, MD 3rd 1993-1994 $45,000 Andrew Gelfand, MD 1st 1993-1994 $30,400 Steven Copenhaver, MD 2nd 1993-1994 $32,400 Giuseppe Colasurdo, MD 2nd 1992-1993 $32,400 Steven Copenhaver, MD 1st 1992-1993 $30,400 Giuseppe Colasurdo, MD 1st 1991-1992 $30,400 Lee Rusakow, MD 2nd 1990-1991 $32,400 Robin Deterding, MD 2nd 1990-1991 $30,400 Lee Rusakow, MD 1st 1989-1990 $30,400 Amanda Dove, MD 2nd 1988-1989 $20,400 Dennis Shellhase, MD 3rd 1988-1989 $30,000 Amanda Dove, MD 1st 1987-1988 $20,400

2. Cystic Fibrosis Foundation Harry S. Schwachman Clinical Investigator Award Sponsor: F. Accurso, M.D. Gwendolyn Kerby, M.D. 1999-2001 $120,000 per yr

Title: Infant Lung Function in Cystic Fibrosis

Edith Zemanick, M.D. 2008-2011 $255,000 ($78,704 per year) CFF # ZEMANI08A0 Title: Polymicrobial and Staphylococcal Infection in Early Cystic Fibrosis. Sponsor: F. Accurso, M.D. 3. National Center for Research Resources (NCRR) K23 Physician Scientist Award Sponsor: F. Accurso, M.D. #1 K23 RR018611-01 Scott Sagel, M.D. 8/1/03 – 7/31/08 $653,400 Title: Noninvasive Biomarkers of Proteolytic Activity in Cystic Fibrosis

C. Research - Investigator Initiated: NIH Awards

Clinical Proteomics Center in Lung Disease 1U01HL081335-01 P.I. Frank J. Accurso, M.D. 25% effort Duration of entire project: 8/15/05 - 6/30/09 (No-cost extension to 6/30/2010) Funding for entire project: $4,216,147

Determinants of Early Pancreatic Injury in Cystic Fibrosis 1RO1 DK61886-01 P.I. Frank J. Accurso, M.D. 20% effort Duration of entire project: 9/30/01 – 8/31/06 Funding for entire project: $2,287, 423

Colorado Clinical Translational Sciences Institute NIH/NCRR 1 UL 1RR025780-01 Principal investigator: Ronald J. Sokol, MD Role: Pediatric Core Laboratory Director 5/19/08 - 4/30/13 $11,957,923 (Annual Direct Costs) Role: Pediatric Core Laboratory Director. 5% effort

Pediatric General Clinical Research Center NIH/NCRR 2 MO1 RR00069-39 P.I.: Richard D. Krugman, M.D. Program Director: Ronald J. Sokol, M.D. Project period: 6/1/01 - 2/28/11 Budget period: 6/1/01 - 2/28/11 Total direct costs Yr. 39: $2,928,998 Role: Core Laboratory Director, 15% effort

Cystic Fibrosis Foundation Therapeutics, Inc. Awards

Therapeutics Development Center – Sweat Analysis Core Laboratory CFF #ACCURS08Y2 P.I. Frank J. Accurso, M.D. 5% Effort Project period: 1/1/09 – 12/31/10 Budget period: 1/1/10 – 12/31/10 Total direct costs: $31,743 (Current Year)

Therapeutics Development Center – Biochemical Biomarker Laboratory CFF #ACCURS03Y2 (PI Accurso) P.I. Frank J. Accurso, M.D. 5% Effort Project Period: 11/1/03 – 12/31/10 Budget Period: 11/1/10 – 12/31/10 Total direct costs: $103,542 (Current Year) 7 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

Translational Therapeutics Development Center CFF # ACCURS09Y0 P.I. Frank Accurso, M.D. (10% effort); Jerry Nick, M.D. (Co P.I., National Jewish Adult Center) Project Period: 1/1/10– 12/31/10 Budget Period: 1/1/09 – 12/31/09 Total direct costs: $160,000 (Split 50% with National Jewish Research & Medical Center)

Electronic Data Capture Equipment for NPD Studies CFF # ACCURS08A0-EQP P.I. Frank J. Accurso, M.D. Project Period: 11/1/08 – 4/30/09 Budget Period: 11/1/08 – 4/30/09 Total direct costs: $7,765

CFTR Function in Human Red Blood Cells CFF # ACCURS08XX0 P.I. Frank J. Accurso, M.D. 5% Effort Project Period: 1/1/09 – 12/31/09 (No cost extension to 12/31/10) Budget Period: 1/1/09 – 12/31/09 Total direct costs: $50,000

Biomarkers of Airway Injury in Cystic Fibrosis Clinical Trials CFF # ACCURS09A0 P.I. Frank J. Accurso, M.D. Project Period: 1/1/09 – 12/31/09 (No cost extension to 12/31/2010) Budget Period: 1/1/09 – 12/31/09 Total direct costs: $18,000 Role:

Colorado Cystic Fibrosis Therapeutics Development Center CFF # ACCURS03Y0 P.I. Frank J. Accurso, M.D. 6% Effort Project Period: 11/1/06 – 12/31/08 Budget Period: 11/1/07 – 12/31/08 Total direct costs: $116,667

Cystic Fibrosis Foundation

CFF #DUNCAN0100 (P.I. Duncan) Cystic Fibrosis Foundation Protein Expression Analysis in Cystic Fibrosis. Project Period: 3/1/02- 2/28/06 Role: Dr. Accurso is a co-investigator. Total direct costs $953,000 The goal of this project is to perform proteomic analysis of clinical specimens in CF in order to identify relevant biomarkers of lung disease.

Effects of P. aeruginosa on Inflammatory Gene Expression. Cystic Fibrosis Foundation PI: David Rodman, M.D. Project I – Airway Epithelial Cell Genomics and Pseudomonas aeruginosa Infection in CF Project Period: 3/01/01-2/28/06 Total direct costs: $1,500,000 Role: Principal Investigator for Project I: F. Accurso, M.D. Funding for Project I: $375,000

1RO1 DK57755-027 8 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

Inhaled Tobramycin in Young Cystic Fibrosis Patients P.I. Bonnie Ramsey, M.D. Site P.I. Jeffrey Wagener, M.D. Site Co.I. F. Accurso, M.D. Site funding: $74,952 direct costs

Sputum Induction in Children with Cystic Fibrosis P.I.: F. Accurso, M.D. Cystic Fibrosis Foundation, Account #ACCURS00A0 Total Award: $126,942, 4/01/00-3/31/03

Safety, Tolerability and Pharmacokinetics of Oral Glutathione in Cystic Fibrosis. PI. F. Accurso, M.D. Cystic Fibrosis Foundation Account #ACCURS04A0 Project Period: 2/1/05-1/31/06 Total Award: $64,800

Ion Transport in Cystic Fibrosis. PI. F. Accurso, M.D. Cystic Fibrosis Foundation Account #ACCURS05A0 Project Period: 1/1/06 - 12/31/06 Total Award: $129,000

Airway Clearance in Cystic Fibrosis P.I.: F. Accurso, M.D. Cystic Fibrosis Foundation Account #ACCURS98A0 Project Period: 1/1/99 – 12/31/04 Total Award: $876,805

Cystic Fibrosis Therapeutic Development Center P.I. F. Accurso, M.D. Cystic Fibrosis Foundation Account #ACCURS98Y0 Project Period: 4/1/98 – 3/31/03 Total Award: $809,770

Early Pathogenesis and Treatment of Cystic Fibrosis. P.I. F. Accurso, M.D. Cystic Fibrosis Foundation, Account #ACCURS97R0 Project Period: 11/1/97 – 10/31/99 Total Award: $400,000

Effect of Postal Transport on Sputum Inflammatory Mediators in Cystic Fibrosis. CFF ACCURS00A1 PI: F. Accurso, M.D. 3% effort Project Period: 11/15/00 - 6/30/02. Total Costs: $34,420

A Prospective, Longitudinal Determination of Computed Tomography of the Chest and Systemic Markers of Inflammation in Children with Cystic Fibrosis Aged 6-10 Years with Pulmonary Abnormalities. P.I. F. Accurso, M.D. Cystic Fibrosis Foundation Account # ACCURS97A0. Total Award: $306,025, 7/1/97-6/30/01. Early Inflammation in the Pathogenesis of Cystic Fibrosis. P.I. F. Accurso, M.D. Cystic Fibrosis Foundation Account # ACCURS96P0. Total Award: $150,000. 12/1/96-11/30/98.

Early Inhaled Corticosteroid Treatment in Cystic Fibrosis. 9 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

P.I. F. Accurso, M.D. Cystic Fibrosis Foundation Account # ACCURS95A0. Total Award: $145,000, 7/1/96-6/30/98.

Early Pulmonary Inflammation in Cystic Fibrosis. P.I. F. Accurso, M.D. Cystic Fibrosis Foundation Account # A745. Total Award: $256,153, 7/1/93-6/30/96.

Colorado Heart Association Chronic Occlusion of the Ductus Arteriosus in the Ovine Fetus. P.I. F. Accurso, M.D. Colorado Heart Association. Total Award: $10,000, 7/1/86-6/30/87.

American Lung Association Adaptation of Fetal Pulmonary Blood Flow to Vasodilating Stimuli. P.I. F. Accurso, M.D. American Lung Association. Total Award: $35,000, 7/1/84-6/30/86.

D. Industry

A Multicenter, Double-Blind, Placebo Controlled, Phase II Study of Aerosolized tgAAVCF for the Treatment of Cystic Fibrosis. P.I. F. Accurso, M.D. Children’s Hospital & Regional Medical Center (Cystic Fibrosis Foundation Therapeutics, Inc.) Estimated Total Award: $54,680 Date of Project: 6/1/04-12/1/05

A Phase I/II Study of Inteferon Gamma-Ib by Inhalation for the Treatment of Patients with Cystic Fibrosis Intermmune Pharaceuticals P.I.: J. Wagener, M.D. Co.I.: F. Accurso, M.D. Expected amount: $126,000 Date of Project: 1/01/01-1/01/02

A Phase Ib12a ascending dose cohort study to assessing INS37217and placebo. CF Therapeutics Development Network, Inspire Pharmaceuticals. P.I.: Robin Deterding, M.D. Co. I.: F. Accurso, M.D. Expected amount: $73,508 Date of Project: 4/01/01-1/31/02.

Evaluation of the Efficacy, Safety, Tolerance and Pharmacokinetics of Meropenem for the Treatment of Acute Pulmonary Exacerbations in Patients with Cystic Fibrosis. 1999-September 2001.

A Randomized, Double-blinded, Placebo-controlled, Multicenter Study of the Safety and Activity of TENOVIL (rHuIL-10) in Clinically Stable Cystic Fibrosis P.I. F. Accurso, M.D. Cystic Fibrosis Foundation, Account #ACCURS00Y3 Total award expected: $12,000. 3/1/00-10/31/01

A Randomized, Double-Blind, Multi-Center Evaluation of Escalating, Single Doses of INS365 in Adult and Pediatric Cystic Fibrosis Patients P.I. F. Accurso, M.D. Inspire Pharmaceuticals, Inc. 10 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

Total Award: $58,000, 1998-1999.

A Multicenter, International, Randomized, Placebo-Controlled, Double-Blind Study to Determine ® the Effect of Pulmozyme on Pulmonary Function Over 96 Weeks in Subjects with Cystic Fibrosis Aged 6–10 Years. Site PI: F. Accurso, M.D. Genentech, Inc. Total Award: $70,000. 1997.

A Phase IIA Dose-Escalation Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of Multiple Doses of DMP777 in Adult Patients 18 Years of Age and Older with Cystic Fibrosis. Site PI: F. Accurso, M.D. DuPont Merck Pharmaceutical Co. Total Award: $63,950. 1997.

A Double Blind, Multicenter Crossover Study to Compare the Safety and Efficacy of Ultrase® MT12 to Placebo in the Treatment of Steatorrhea in Patients with Cystic Fibrosis. Site PI: F. Accurso, M.D. Scandipharm, Inc. Total Award: $93,044. 1997

A Comparison of the Efficacy and Desirability of Chest Wall Oscillatory Vibration with the Flutter VRP1 in Cystic Fibrosis. Site PI: F. Accurso, M.D. American Biosystems, Inc. Total Award: $26,000. 1996.

A Comparison of the Efficacy and Desirability of the Flutter VRP1 with Conventional Chest Physiotherapy in Cystic Fibrosis. PI: F. Accurso, M.D. American Biosystems, Inc. Total Award: $20,000. 1995.

A Randomized, Multi-Center, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy of a Mucoid Exopolysaccharide Pseudomonas aeruginosa Immune Globulin, Intravenous (human) (MEP IVIG) in Reducing the Frequency of Acute Pulmonary Exacerbation in Patients with Cystic Fibrosis. Site PI: F. Accurso, M.D. UNIVAX Biologics, Inc. UNX-0402. Total Award: $80,000 (1995-1996)

A Phase IV, Multicenter, Randomized Trial in Patients with Mild Cystic Fibrosis to Determine the  Relative Efficacy of Pulmozyme (dornase alfa) Delivered by Two Different Nebulizer Systems. Site PI: F. Accurso, M.D. Genentech, Inc. (GNE 0655g). Total Award: $25,000. 1995.

A Two Stage Pilot Study to Determine Safety and Deposition of a Single Daily Dose of Aerosolized Recombinant Human DNase I (dornase alfa, Pulmozyme) in Young Cystic Fibrosis Patients. Site PI: K. Kirchner Co-Investigator: F. Accurso, M.D. Genentech, Inc. (GNE Z0644g). Total Award: $100,000. 1995.

 A Phase IV, Multicenter, Study to Monitor the Long-term Safety and Efficacy of Pulmozyme (dornase alfa) in Cystic Fibrosis Patients Who Participated in a Phase III Study. Site PI: K. Kirchner. Co-Investigator: F. Accurso, M.D. Genentech, Inc. (GNE Z0620n). Total Award: $10,000/year. 1994-1995.

A Multicenter, Longitudinal Follow-up Study of Patients with Cystic Fibrosis to Evaluate Breathing Function, the Rate of Respiratory Tract Infection and the Safety of Long Term . Treatment With Pulmozyme Site PI: F. Accurso, M.D. Genentech, Inc. (GNE Z0525n). Total Award: $50,000 per year, 1994-1997.

A Multicenter, Epidemiologic Protocol to Evaluate Pulmonary Function and the Rate of Respiratory Infection in Patients with Cystic Fibrosis. Site PI: K. Kirchner Co-Investigator: F. Accurso, M.D. Genentech, Inc. (GNE Z0525n). Total Award: $6,000 per year, 1993-1994.

A Multicenter, Open-Label Treatment Protocol to Provide Aerosolized rhDNase Therapy to Patients with Cystic Fibrosis. Site PI: K. Kirchner Co-investigator: F. Accurso, M.D. Genentech, Inc., (GNE Z0507g-A2). Total Award: $10,000 per year. 1993-1994.

A Randomized Multicenter Study to Evaluate the Safety and Efficacy of Aerosolized Recombinant Human DNase I (rhDNase) Administered with Three Different Aerosol Delivery systems in Patients with Cystic Fibrosis. Site PI: K. Kirchner Co-investigator: F. Accurso, M.D. Genentech, Inc., (GNE Z05555g). Total Award: $20,000 per year. 1993-1994.

A Multicenter Double-Blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of Aerosolized rhDNase Therapy in Patients with Cystic Fibrosis Who Have Advanced Lung Disease PI: K. Kirchner Co-investigator: F. Accurso, M.D. Genentech, Inc., (GNE Z0554g). Total Award: $20,000 per year. 1993-1994.

A Phase II Study to Evaluate the Safety and Efficacy of Inhaled Secretory Leukoprotease Inhibitor in Patients with Cystic Fibrosis. PI: J. Wagener, M.D. Co-investigator: F. Accurso, M.D. Synergen, Inc. Total Award: $100,000. 1992-1994.

A Phase III Multicenter, Double-Blind, Placebo Controlled, Parallel Study to Evaluate the Safety and Efficacy of Aerosolized Recombinant Human DNase I (DNase) in Patients with Cystic Fibrosis. PI: K. Kirchner, Co-investigator: F. Accurso, M.D. Genentech, Inc. Total Award: $50,000 per year. 1993-1994.

A Randomized Parallel Group, Double-blind Clinical Trial Comparing Amiloride Hydrochloride with Placebo in Adolescent and Adult Patients with Cystic Fibrosis. Site PI: D. Rodman, Co-investigator: F. Accurso, M.D. Glaxo, (AML-303). Total Award: $50,000 per year, 1991-1994.

12. MAJOR SCIENTIFIC INTEREST: Cystic Fibrosis.

13. BIBLIOGRAPHY of Frank J. Accurso, M.D.

A. Original Research Published in Refereed Journals 1. Ratner I, Hernandez J, Accurso FJ. Low peak inspiratory pressure for ventilation of infants with hyaline membrane disease. J Pediatr 100:802-804, 1982. 2. Cogbill TH, Moore FA, Accurso FJ. Primary tracheomalacia. Ann Thoracic Surg 35:538-541, 1983. 3. Reardon MC, Hammond KB, Accurso FJ, Fisher CD, McCabe ER, Cotton EK, Bowman CM. Nutritional deficits exist before two months of age in some infants with cystic fibrosis identified by screening test. J Pediatr 105:27l-274, 1984. 4. Abman SH, Accurso FJ, Koops BL. Experience with home oxygen in the management of infants with bronchopulmonary dysplasia. Clin Pediatr 23:47l-476, 1984. 5. Abman SH, Accurso FJ, Bowman CM. Unsuspected cardiopulmonary abnormalities complicating bronchopulmonary dysplasia. Arch Dis Child 59:966-970, 1984. 6. Abman SH, Wolfe RR, Accurso FJ, Koops BL, Bowman CM, Wiggins JW. Pulmonary vascular response to oxygen in infants with severe bronchopulmonary dysplasia. Pediatrics 75:80-84, 1985. 7. Updike PA, Accurso FJ, Jones RH. Physiologic circadian rhythmicity in preterm infants. Nurs Res 34:160-163, 1985. 8. Abman SH, Reardon MC, Accurso FJ, Hammond KB, Sokol R. Hypoalbuminemia at diagnosis as a marker for severe respiratory course in infants with Cystic Fibrosis identified by newborn screening. J Pediatr 107:933-935, 1985. 9. Abman SH, Accurso FJ, Bowman CM. Persistent morbidity and mortality of protein calorie malnutrition in young infants with CF. Pediatr Gastroenterol and Nutr 5:393-396, 1986. 10. Accurso FJ, Alpert B, Peterson RJ, Wilkening RB, Meschia G. Time dependent response of fetal pulmonary blood flow to an increase in fetal oxygen tension. Respir Physiol 63:43-52, 1986. 11. Abman SH, Wilkening RB, Ward RM, Accurso FJ. Adaptation of fetal pulmonary blood flow to local infusion of tolazoline. Pediatr Res 20:1131-1135, 1986. 12. Abman SH, Accurso FJ, Wilkening RB, Meschia G. Persistent fetal pulmonary hypoperfusion after acute hypoxia. Am J Physiol 22:H941-H948, 1987. 13. Accurso FJ, Wilkening RB. Temporal response of the fetal pulmonary circulation to pharmacologic vasodilators. Proc Soc Exp Biol Med 187:89-98, 1988. 14. Accurso FJ, Wilkening RB. Fetal pulmonary vasodilation and vasoreactivity during metabolic acidemia. J Dev Physiol 10:411-421, 1988. 15. Abman SH, Ogle JW, Butler-Simon N, Rumack CM, Accurso FJ. Role of respiratory syncytial virus in early hospitalizations for respiratory distress in young infants with cystic fibrosis. J Pediatr 113:826-830, 1988. 16. Abman SH, Shanley PF, Accurso FJ. Failure of postnatal adaptation of the pulmonary circulation after chronic intrauterine pulmonary hypertension in fetal lambs. J Clin Invest 83:1849-1858, 1989.

17. Sokol RJ, Reardon MC, Accurso FJ, Stall C, Narkewicz M, Abman SH, Hammond KB. Fat-soluble- vitamin status during the first year of life in infants with cystic fibrosis identified by screening of newborns. Am J Clin Nutr 50:1064-1071, 1989. 18. Abman SH, Accurso FJ. Acute effects of partial compression of the ductus arteriosus on fetal pulmonary circulation. Am J Physiol 257:H626-H634, 1989. 19. Truog RD, Accurso FJ, Wilkening RB. Fetal pulmonary vasodilation by histamine: Response to H1 and H2 stimulation. Dev Pharmacol Ther 14:180-186, 1990. 20. Accurso FJ, Abman SH, Wilkening RB, Worthen S, Henson P. Fetal pulmonary vasodilation after exogenous platelet activating factor. J Appl Physiol 70:778-787, 1991. 21. Abman SH, Accurso FJ. Sustained fetal pulmonary vasodilation with prolonged atrial natriuretic factor and GMP infusions. Am J Physiol 260:H183-H192, 1991. 22. Hammond KB, Abman SH, Sokol RJ, Accurso FJ. Efficacy of statewide neonatal screening for Cystic Fibrosis by assay of trypsinogen concentrations. N Eng J Med 325:769-774, 1991. 23. Abman SH, Ogle JW, Harbeck RJ, Butler-Simon N, Hammond K, Accurso FJ. Early bacteriologic, immunologic and clinical courses of young infants with cystic fibrosis identified by newborn screening. J Pediatr 119:211-217, 1991. 24. Seltzer WK, Accurso FJ, Fall MZ, VanRiper AJ, Descartes M, Huang Y, McCabe ERB. Screening for Cystic Fibrosis: Feasibility of molecular genetic analysis of dried blood specimens. Biochem Med Metab Biol 46:105-109, 1991. 25. Bronstein MN, Sokol RJ, Abman SH, Chatfield BA, Hammond KB, Hambidge KM, Stall RD, Accurso FJ. Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 120: 533-540, 1992. 26. Dove AM, Szefler SJ, Hill MR, Jusko WJ, Larsen GL, Accurso FJ. Altered prednisolone pharmacokinetics in patients with cystic fibrosis. J Pediatr 120: 789-794, 1992. 27. Mohon RT, Wagener JS, Abman SH, Seltzer WS, Accurso FJ. Relationship of genotype to pulmonary function in infants with cystic fibrosis identified through neonatal screening. J Pediatr 122:550-555, 1993. 28. Rusakow LS, Abman SH, Sokol RJ, Seltzer W, Hammond K, Accurso FJ. Immunoreactive trypsinogen levels in infants with cystic fibrosis complicated by meconium ileus. Screening 2:13-17, 1993. 29. Grabe T, Seltzer W, DeMarchi J, Silva D, Doane WW, Gozal D, Richter SF, Bowman CM, Norman RA, Rhodes S, Harnrie L, Murphy S, Harwood I, Accurso FJ, Jain D. Genetic Analysis of Hispanic Individuals with Cystic Fibrosis. Am J Hum Gen 54:443-446, 1994. 30. Bronstein MN, Davies PSW, Hambidge KM, Accurso FJ. Normal energy Expenditure in the infant with presymptomatic Cystic Fibrosis. J Pediatr 126:28-33, 1995. 31. Khan TK, Wagener JS, Boat T, Marinez J. Accurso FJ, Riches DWH. Early Pulmonary Inflammation in Infants with Cystic Fibrosis. Amer J Resp Crit Care Med 151:1075-82, 1995. 32. Giles DR, Wagener JS, Accurso FJ, Butler-Simon N. Short-term effects of postural drainage with clapping vs. autogenic drainage on oxygen saturation and sputum recovery in patients with Cystic Fibrosis. Chest 108:952-54 1995. 33. Kirchner KK, Wagener JS, Khan TZ, Accurso FJ. Increased DNA levels in bronchoalveolar lavage fluid obtained from infants with cystic fibrosis. Am J Respir Crit Care Med 1996;154:1426-1429. 34. Erskine JM, Lingard CD, Sontag MK, Accurso FJ. Enteral nutrition for patients with cystic fibrosis: comparison of a semi-elemental and nonelemental formula. J Pediatr 132(2):265-269, 1998. 35. Easley D, Krebs N, Jefferson M, Miller L, Erskine J, Accurso FJ, Hambidge KM. Effect of pancreatic enzymes on zinc absorption in cystic fibrosis. J Pediatr Gastroenterol Nutr 26(2):136-139, 1998. 36. Krebs N, Sontag M, Accurso FJ, Hamidge K. Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 133(6):761-4, 1998. 14 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

37. Feranchak AP, Sontag MK, Wagener JS, Hammond KB, Accurso FJ, Sokol RJ. Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. J Pediatr 1999 Nov;135(5):601-10. 38. Rosenfeld M, Emerson J, Accurso FJ, Armstrong D, Castile R, Grimwood K, Hiatt P, McCoy K, McNamara S, Ramsey B, Wagener J. Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. Pediatr Pulmonol 1999 Nov;28(5):321-8. 39. Dobyns EL, Griebel J, Kinsella JP, Abman SH, Accurso FJ. Infant lung function after inhaled nitric oxide therapy for persistent pulmonary hypertension of the newborn. Pediatr Pulmonol 28 (1):24-30, 1999. 40. Krebs NF, Westcott JE, Arnold TD, Kluger BM, Accurso FJ, Miller LV, Hambidge KM. Abnormalities in zinc homeostasis in young infants with cystic fibrosis. Pediatr Res 48 (2): 256-261, 2000. 41. Noone PG, Hamblett N, Accurso FJ, Aitken ML, Boyle M, Dovey M, Gibson R, Johnson C, Kellerman D, Konstan MW, Milgram L, Mundahl J, Retsch-Bogort J, Rodman D, Williams-Warren J, Wilmott RW, Zeitlin P, Ramsey B. Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: Results of a phase I multi-center study. Pediatr Pulmonol 32 (2): 122-8, 2001. 42. Bals R, Weiner DJ, Meegalla RL, Accurso FJ, Wilson JM. Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid. Am J Respir Cell Mol Biol 25 (1): 21-5, 2001. 43. Wojtczak HA, Kerby GS, Wagener J, Copenhaver S, Gotlin W, Riches DWH, Accurso FJ. Beclomethasone Diproprionate Reduced Airway Inflammation without adrenal suppression in Young Children with Cystic Fibrosis: A Pilot Study. Pediatr Pulmonol 32:293-301, 2001. 44. Oermann CM, Sockrider MM, Giles D, Sontag MK, Accurso FJ, Castile RG. A Pilot Study Comparing High-Frequency Chest Wall Oscillation and Oscillating PEP in the Home Management of Cystic Fibrosis Pediatr Pulmonol 32:293-301, 2001. 45. Bennett M, Galan H, Owens G, Dewey R, Banks R, Hobbins J, Accurso FJ, Schaack J. In Utero Gene Delivery by Intra-Amniotic Injection of a Retroviral Vector Producer Cell Line in a Non-Human Primate Model. Hum Gene Ther 12:1857-65, 2001. 46. Sagel SD, Kapsner R, Osberg I, Sontag MK, Accurso FJ. Airway inflammation in children with Cystic Fibrosis and healthy children assesed by sputum induction. Am J Respir Crit Care Med 2001 Oct 15; 164(8 Pt 1): 1425-31. 47. Vandivier RW, Fadok VA, Hoffmann PR, Bratton DL, Penvari C, Brown KK, Brain JD, Accurso FJ, Henson PM. Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis. J Clin Invest 2002 Mar; 109(5): 661-70 48. Kerby GS, Cottin V, Accurso FJ, Hoffmann F, Chan ED, Fadok VA, Riches DW. Impairment of macrophage survival by NaCl: implications for early pulmonary inflammation in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 2002 Jul; 283(1):L188-97. 49. Galan HL, Bennett ML, Tyson RW, Owens G, Regnault TR, Accurso FJ, Hobbins JC, Schaack J. Inefficient transduction of sheep in utero after intra-amniotic injection of retroviral producer cells. Am J Obstet Gynecol 2002 Aug; 187(2):469-474. 50. Davies PS, Erskine JM, Hambidge KM, Accurso FJ. Longitudinal investigation of energy expenditure in infants with cystic fibrosis. Eur J Clin Nutr 2002 Oct;56(10):940-6. 51. Sagel SD, Sontag MK, Wagener JS, Kapsner RK, Osberg I, Accurso FJ. Induced sputum inflammatory measures correlate with lung function in children with cystic fibrosis. J Pediatr 2002 Dec;141(6):811-7. 52. Maselli JH, Sontag MK, Norris JM, MacKenzie T, Wagener JS, Accurso FJ. Risk Factors for Initial Acquisition of Pseudomonas aeruginosa in Children with Cystic Fibrosis identified by Newborn Screening. Pediatr Pulmonol 2003:35:257-62. 53. Gibson RL, Emerson J, McNamara S, Burns JL, Rosenfeld M, Yunker A, Hamblett N, Accurso FJ, Dovey M, Hiatt P, Konstan MW, Moss R, Retsch-Bogart G, Wagener J, Ramsey B. Significant Microbiologic Effects of Inhaled Tobramycin in Young Children with Cystic Fibrosis. Am J Respir Crit Care Med 2003 Mar 15;167(6):841-9. 15 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

54. Maciel P, Yan J, Feng J, Accurso FJ, Sommer S. Improved single-tube method for determination of F508del genotype in the CFTR gene using Bidirectional PCR Amplification of Specific Alleles (Bi-PASA). Biotechniques 2003 Mar;34(3):460-2. 55. Ordonez CL, Henig NR, Mayer-Hamblett N, Accurso FJ, Burns JL, Chmiel JF, Daines CL, Gibson RL, McNamara S, Retsch-Bogart GZ, Zeitlin PL, Aitken ML. Inflammatory and Microbiologic Markers in Induced Sputum following intravenous Antibiotics in Cystic Fibrosis. Am J Respir Crit Care Med 2003 Dec 15;168(12):1471-5. 56. Saiman L. Marshall BC. Mayer-Hamblett N. Burns JL. Quittner AL. Cibene DA. Coquillette S. Fieberg AY. Accurso FJ. Campbell PW 3rd. Macrolide Study Group. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 290(13):1749-56, 2003. 57. Wooldridge JL, Deutsch GH, Sontag MK, Osberg I, Chase DR, Silkoff PE, Wagener JS, Abman SH, Accurso FJ. NO pathway in CF and non-CF children. Pediatr Pulmonol Apr;37(4):338-50, 2004. 58. Rubio F, Cooley J, Accurso FJ, Remold-O”Donnell E. Linkage of neutrophil serine proteases and decreased surfactant protein-A (SP-A) levels in inflammatory lung disease. Thorax Apr;59(4):318-323, 2004. 59. Rancourt RC, Tai S, King M, Heltshe SL, Penvari C, Accurso FJ, White C. Thioredoxin Mucolyzes sputum. A Redox based strategy for CF. Am J Physiol Lung Cell Mol Physiol May;286(5):L931-8. 2004. 60. Karp CL, Flick L, Park KW, Softic S, Greer TM, Keledjian R, Yang R, Uddin J, Guggino WB, Atabani SF, Belkaid Y, Xu Y, Whitsett JA, Accurso FJ, Wills-Karp M, Petasis NA. Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway. Nat Immunol Apr;5(4):388-92, 2004. 61. Rodman DM, Polis JM, Heltshe SL, Sontag MK, Chacon C, Rodman RV, Brayshaw SJ, Huitt GA, Iseman MD, Saavedra MT, Taussig LM, Wagener JS, Accurso FJ, Nick JA. Late diagnosis defines a unique population of long-term survivors of cystic fibrosis. Am J Respir Crit Care Med 2005 Mar 15;171(6):621-6. 62. Condino AA, Hoffenberg EJ, Accurso FJ, Penvari C, Anthony M, Gralla J, O'Connor JA. Frequency of ASCA seropositivity in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2005 Jul;41(1):23-6. 63. Sontag MK, Hammond KB, Zielenski J, Wagener JS, Accurso FJ . Two-tiered immunoreactive trypsinogen- based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes. J Pediatr 2005 Sep;147(3 Suppl):S83-8. 64. Accurso FJ, Sontag MK, Wagener JS. Complications associated with symptomatic diagnosis in infants with cystic fibrosis. J Pediatr 2005 Sep;147(3 Suppl):S37-41. 65. Lee RL, Rancourt RC, del Val G, Pack K, Pardee C, Accurso FJ, White CW. Thioredoxin and dihydrolipoic acid inhibit elastase activity in cystic fibrosis sputum. Am J Physiol Lung Cell Mol Physiol 2005 Nov;289(5):L875-82. 66. Sontag MK, Corey M, Hokanson JE, Marshall JA, Sommer SS, Zerbe GO, Accurso FJ. Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screening. J Pediatr 2006 Nov;149(5):650-657. 67. Mayer-Hamblett N, Aitken ML, Accurso FJ, Kronmal RA, Konstan MW, Burns JL, Sagel SD, Ramsey BW. Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med 2007 Apr 15;175(8):822-8. 68. Rancourt RC, Lee RL, O'Neill H, Accurso FJ, White CW. Reduced thioredoxin increases proinflammatory cytokines and neutrophil influx in rat airways: modulation by airway mucus. Free Radic Biol Med 2007 May 1;42(9):1441-1453. 69. Gibson RL, Emerson J, Mayer-Hamblett N, Burns JL, McNamara S, Accurso FJ, Konstan MW, Chatfield BA, Retsch-Bogart G, Waltz DA, Acton J, Zeitlin P, Hiatt P, Moss R, Williams J, Ramsey BW. Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis. Pediatr Pulmonol 2007 Jul;42(7):610-23.

70. Papas KA, Sontag MK, Pardee C, Sokol RJ, Sagel SD, Accurso FJ, Wagener JS. A pilot study on the safety and efficacy of a novel antioxidant rich formulation in patients with cystic fibrosis. J Cyst Fibros 2007 Jun 12; 71. Moss RB, Milla C, Colombo J, Accurso FJ, Zeitlin PL, Clancy JP, Spencer LT, Pilewski J, Waltz DA, Dorkin HL, Ferkol T, Pian M, Ramsey B, Carter BJ, Martin DB, Heald AE. Repeated Aerosolized AAV-CFTR for Treatment of Cystic Fibrosis: A Randomized Placebo-Controlled Phase 2B Trial. Hum Gene Ther 2007 Aug;18(8):726-32. 72. Baker CD, Federico MJ, Accurso FJ. Case report: skin discoloration following administration of colloidal silver in cystic fibrosis. Curr Opin Pediatr 2007 Dec;19(6):733-5. 73. Harris JK, De Groote MA, Sagel SD, Zemanick ET, Kapsner R, Penvari C, Kaess H, Deterding RR, Accurso FJ, Pace NR. Molecular identification of bacteria in bronchoalveolar lavage fluid from children with cystic fibrosis. Proc Natl Acad Sci U S A 2007 Dec 18;104(51):20529-33. Epub 2007 Dec 11. 74. Cooley J, McDonald B, Accurso FJ, Crouch EC, Remold-O'Donnell E.Patterns of neutrophil serine protease-dependent cleavage of surfactant protein D in inflammatory lung disease. J Leukoc Biol 2008 Apr;83(4):946-55. Epub 2008 Jan 22. 75. Laguna TA, Sagel SD, Sontag MK, Accurso FJ. The clinical course of a Mexican female with cystic fibrosis and the novel genotype S531P/S531P. J Cyst Fibros 2008 Sep;7(5):454-6. 76. Laguna TA, Sontag MK, Osberg I, Wagener JS, Accurso FJ, Sokol RJ. Decreased Total Serum Coenzyme Q(10) Concentrations:A Longitudinal Study in Children with Cystic Fibrosis. J Pediatr 2008 Sep;153(3):402-7. 77. Saavedra MT, Hughes GJ, Sanders LA, Carr M, Rodman DM, Coldren CD, Geraci MW, Sagel SD, Accurso FJ, West J, Nick JA. Circulating RNA Transcripts Identify Therapeutic Response in Cystic Fibrosis Lung Disease. Am J Respir Crit Care Med 2008 Nov 1;178(9):929-38. 78. Bernus A, Wagner BD, Accurso FJ, Doran A, Kaess H, Ivy DD. Brain Natriuretic Peptide Levels in Managing Pediatric Patients with Pulmonary Arterial Hypertension.. Chest 2009 Mar;135(3):745-51. 79. Sagel SD, Sontag MK, Accurso FJ. Relationship between antimicrobial proteins and airway inflammation and infection in cystic fibrosis. Pediatr Pulmonol 2009 Apr;44(4):402-9. 80. Sontag MK, Wright D, Beebe J, Accurso FJ, Sagel SD. A New Cystic Fibrosis Newborn Screening Algorithm: IRT/IRT1 /DNA. J Pediatr 2009 Nov;155(5):618-22. Epub 2009 Jun 21.PMID: 19540513 81. Laguna TA, Wagner BD, Luckey HK, Mann SA, Sagel SD, Regelmann W, Accurso FJ. Sputum Desmosine During Hospital Admission for Pulmonary Exacerbation in Cystic Fibrosis. Chest. 2009 Dec;136(6):1561-8. Epub 2009 Jun 30.PMID: 19567495 82. Kalka-Moll WM, Lipuma JJ, Accurso FJ, Plum G, van Koningsbruggen S, Vandamme P. Airway infection with a novel Cupriavidus species in persons with cystic fibrosis. J Clin Microbiol 2009 Sep;47(9):3026- 8.PMID: 19605576 83. Dorfman R, Li W, Sun L, Lin F, Wang Y, Sandford A, Pare PD, McKay K, Kayserova H, Piskackova T, Macek M, Czerska K, Sands D, Tiddens H, Margarit S, Repetto G, Sontag MK, Accurso FJ, Blackman S, Cutting GR, Tsui LC, Corey M, Durie P, Zielenski J, Strug LJ. Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping results. Hum Genet 2009 Aug 7. [Epub ahead of print] PMID:19662435 84. Sontag MK, Quittner AL, Modi AC, Koenig JM, Giles D, Oermann CM, Konstan MW, Castile R, Accurso FJ; Investigators and Coordinators of the Airway Secretion Clearance Trial. Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis. Pediatr Pulmonol. 2010 Mar;45(3):291-300.PMID: 20146387

85. Wagner BD, Accurso FJ, Laguna TA. The applicability of urinary creatinine as a method of specimen normalization in the cystic fibrosis population. J Cyst Fibros 2010 Mar 12. [Epub ahead of print] PMID:20227353

86. Modi AC, Cassedy AE, Quittner AL, Accurso FJ, Sontag MK, Koenig JM, Ittenbach RF. Trajectories of Adherence to Airway Clearance Therapy for Patients with Cystic Fibrosis. J Pediatr Psychol 2010 Mar 18. [Epub ahead of print] PMID: 20304772 87. Nick JA, Chacon CS, Brayshaw SJ, Jones MC, Barboa CM, St Clair CG, Young RL, Nichols DP, Janssen JS, Huitt GA, Iseman MD, Daley CL, Taylor-Cousar JL, Accurso FJ, Saavedra MT, Sontag MK. Effects of Gender and Age of Diagnosis on Disease Progression in Long-Term Cystic Fibrosis Survivors. Am J Respir Crit Care Med 2010 May 6. [Epub ahead of print] PMID: 20448091 88. Solomon GM, Konstan MW, Wilschanski M, Billings J, Sermet I, Accurso FJ, Vermeulen F, Levin E, Hathorne H, Reeves G, Sabbatini G, Hill A, Mayer-Hamblett N, Ashlock M, Clancy JP, Rowe SM. An International Randomized Multicenter Comparison of Nasal Potential Difference Techniques. Chest 2010 May 14. [Epub ahead of print] PMID: 20472865 89. Zemanick ET, Wagner BD, Harris JK, Wagener JS, Accurso FJ, Sagel SD. Pulmonary exacerbations in cystic fibrosis with negative bacterial cultures. Pediatr Pulmonol. 2010 Jun;45(6):569-77.PMID: 20503282 [PubMed - in process]

90. Wagner BD, Accurso FJ, Laguna TA. The applicability of urinary creatinine as a method of specimen normalization in the cystic fibrosis population. J Cyst Fibros. 2010 May;9(3):212-6. Epub 2010 Mar 15.PMID: 20227353 [PubMed - in process]

91. Accurso FJ, Rowe SM, ClancyJP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, , Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson EO, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW. VX-770 in Persons with Cystic Fibrosis and the G551D CFTR Mutation. New Engl J Med. Accepted for Publication 2010.

92. Cooley J, Sontag MK, Accurso FJ, Remold-O’Donnell E. SerpinB1 in CF airway fluids: quantity, molecular formand mechanism of elastase inhibition. Eur Respir J. 2010 Sept 3 [Epub ahead of print] PMID: 20817705

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer- Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010 Nov 18;363(21):1991- 2003. PMID: 21083385 [PubMed - indexed for MEDLINE]

93. 94. Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimens. 95. Zemanick ET, Wagner BD, Sagel SD, Stevens MJ, Accurso FJ, Harris JK. Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimens. 96. 97. PLoS One. 2010 Nov 30;5(11):e15101. 98. PMID: 99. 21152087 100. [PubMed - in process]

101.Denufosol Tetrasodium in Patients with Cystic Fibrosis and Normal to Mildly Impaired Lung Function. 102.Accurso FJ, Moss RB, Wilmott RW, Anbar RD, Schaberg AE, Durham TA, Ramsey BW; the TIGER-1 Investigator Study Group. 18 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

103.Am J Respir Crit Care Med. 2010 Dec 17. [Epub ahead of print] 104. PMID: 105. 21169471 106. [PubMed - as supplied by publisher] 107.

B. Review articles, book chapters, editorials, letters, and other publications 1. Accurso FJ. Relation of fluid intake to bronchopulmonary dysplasia (letter). J Pediatr 94:681-682, 1979. 2. Koops BL, Abman SH, Accurso FJ. Outpatient management and follow-up of bronchopulmonary dysplasia. Clin in Perinatol 11:101-122, 1984. 3. Hammond K, Reardon MC, Accurso FJ, Abman SH, Cotton EK, Pinney M, Fisher C, Sokol RJ, Ask CG, Watts DC, Bonner AM. Early detection and follow-up of cystic fibrosis in newborns: The Colorado experience. Proceedings of the XVI Annual Birth Defects Symposium Management. Genetic Disease: Screening and Management, Alan R. Liss, Inc., pp 81-101, 1986. 4. Accurso FJ, Reardon M, Abman S, Pinney M, Cotton E, Sokol R, Hammond K. Early abnormalities in infants with cystic fibrosis diagnosed through newborn screening. Insights into Paediatrics 1:29-33, 1987. 5. Accurso FJ, Abman SH, Reardon MC, Hammond KB. Early abnormalities in infants with cystic fibrosis diagnosed through newborn screening. Pediatr Pulmonol Suppl 1: 83-85, 1987. 6. Accurso FJ, Helton J, Hammond K, Abman S. Newborn screening for cystic fibrosis - Pro. Pediatr Pulmonol Suppl 2: 51-53, 1988. 7. Hammond KB, Ask CA, Watts DC, Accurso FJ. Issues of follow-up sweat tests and their interpretation. In, Travert G, editor. Muscoviscidose: Depistage Neonatal et Prise en Charge Precoce. Proceedings of Conference International Mucoviscidose; 1988 Oct 5-6; (Caen, France): Universite de Caen, 1988:81-89. 8. Hammond KB, Reardon MC, Accurso FJ. Neonatal screening and early diagnosis of cystic fibrosis. Bethesda (MD): Maternal and Child Health and Crippled Children's Services: 1988 Aug. Report No: MCJ-080508- 03. 9. Abman SH, Accurso FJ. Acute and chronic fetal pulmonary hypertension alter pulmonary vasoreactivity. Chest 93: Suppl 2:117S-119S, 1988. 10. Sokol RJ, Accurso FJ, Abman SH and Hammond KH. Do CF patients need fat-soluble vitamins? Pediatr Pulmonol, Suppl 4:63-65, 1989. 11. Stenmark KR, Abman SH, Accurso FJ. Etiologic mechanisms in the pathogenesis of persistent pulmonary hypertension of the newborn. In, Pulmonary Vascular Physiology and Pathophysiology. Weir EK and Reeves JT (eds.). New York: Marcel Dekker, 1989, pp. 355-402. 12. Sokol RJ, Butler-Simon N, Heugi JE, Iannaccone ST, McClung HJ, Accurso FJ, Hammond K, Heyman M, Sinatra F, Riely C, Perrault J, Levy J, Silverman A. Vitamin E deficiency in children with fat malabsorption: studies in cystic fibrosis and cholestasis. Ann N Y Acad Sci 570:156-169, 1989. 13. Abman SH, Accurso FJ, Sokol RJ. Hypoalbuminemia in young infants with cystic fibrosis (letter). J Pediatr 116:840-841, 1990. 14. Sokol RJ, Accurso FJ, Abman SH, Hammond K, Stall C. Nutritional issues in cystic fibrosis. In Pediatric Gastroenterology and Nutrition. Aspen Seminars on Pediatric Disease, Balisteri NF and Vanderhoff J (eds.) New York: Chapman and Hall Medical. IV:273-295, 1990. 15. Accurso FJ, Sokol RJ, Hammond KB, Abman SH. Early respiratory course in infants with cystic fibrosis: relevance to newborn screening. Pediatr Pulmonol Suppl 7:42-45, 1991.

16. Helton JL, Harmon RJ, Robinson N, Accurso FJ. Parental attitude toward newborn screening for cystic fibrosis. Pediatr Pulmonol Suppl 7:23-28, 1991. 17. Sokol RJ, Reardon MC, Accurso FJ, Stall C, Narkewicz MR, Abman SH, Hammond KB. Fat-Soluble vitamins in infants identified by cystic fibrosis newborn screening. Pediatric Pulmonol Suppl 7:52-55, 1991. 18. Krebs NF, Accurso FJ, Hambidge KM. Zinc Status in Cystic Fibrosis. Pediatric Pulmonol Suppl 9:163-164, 1993. 19. Accurso FJ, Eigen H, Loughlin GM. History and Physical Examination. In Respiratory Disease in Children, Loughlin GM and Eigen H (eds). Baltimore: Williams and Wilkins, 1994, pp 67-76. 20. Accurso FJ. Assessment of Lung Disease in Infants with Cystic Fibrosis. New Insights into Cystic Fibrosis. Gardiner-Caldwell, Cheshire, UK, 1994. 21. Accurso FJ. Aerosolized Dornase Alfa in cystic Fibrosis Patients with Clinically Mild Lung Disease. Dornase Alfa Clinical Series Vol 1, No. 5, 1994. 22. Accurso FJ. Does Inflammation Precede Infection in Early Airways Disease? Pediatr Pulmonol Suppl 12:71- 72, 1995. 23. Wagener JS, Copenhaver SC, Kirchner KK, Accurso FJ. Early Pulmonary Treatment in Cystic Fibrosis. Pediatr Pulmonol Suppl 12:84-85, 1995. 24. Wagener JS, Erskine JE, Krebs NF, Sokol RJ, Accurso FJ. Airway Inflammation and Nutrition in Young Children with Cystic Fibrosis. Pediatr Pulmonol Suppl 1996; 13:161-162, 1996. 25. Calvi AM, Anthony M, Helton JL, Accurso FJ. Psychosocial Issues in Families of Infants with Cystic Fibrosis Identified Through Newborn Screening: The Early Years. Pediatr Pulmonol Suppl 13:148-149, 1996. 26. Accurso FJ, Sontag MK; Newborn Screening for Cystic Fibrosis: A Paradigm for Public Health Genetics Policy Development. Clinical Course of Infants with Cystic Fibrosis: Opportunities for Early Intervention; Proceedings of a 1997 Workshop. MMWR 46 (RR-16), 1997 27. Accurso FJ, Sontag MS, Wagener JS. Newborn screening for cystic fibrosis: The need from a center director’s standpoint. Pediatr Pulmonol Suppl 14:188-189, 1997. 28. Accurso FJ, The rationale for early intervention in cystic fibrosis: Introduction. Pediatr Pulmonol Suppl 24(2):132-133, 1997. 29. Wagener JS, Accurso FJ. Early Lung Disease in Cystic Fibrosis. In Cystic Fibrosis: Pathogenesis and New Therapies Slide Module. Volume 2. Gardiner-Caldwell SynerMed, Califon, NJ. 1997. 30. The Role of Prophylactic Therapies in the Management of CF Patients with Mild, Asymptomatic Disease. Roundtable discussion on tape. Gardiner-Caldwell SynerMed, Califon, NJ. 1997. 31. Eid NS, Accurso FJ, Stenzler A. Early asymptomatic pulmonay changes in infants with cyctic fibrosis. Pediatr Pulmonol Suppl 16:276-277, 1997. 32. Wagener JS, Khan TZ, Copenhaver SC, Accurso FJ. Early inflammation and the development of pulmonary disease in cystic fibrosis. Pediatr Pulmonol Suppl 16:267-268, 1997. 33. Accurso FJ. Early pulmonary disease in cystic fibrosis. Curr Opin Pulm Med 3(6): 400-403, 1997. 34. Accurso FJ. Treatment of airway inflammation and hyprrectivity in cystic fibrosis. In, Les medicaments de la muscoviscidose chez l’enfant. Pons G, Lenoir G, Navarro J (editors). Paris: Springer, 1997:107-117. 35. Accurso FJ, Wagener JS, Sontag MK. The role of early diagnosis and treatment in the development of the CF Phenotype. Pediatr Pulmonol Suppl 17:98-99, 1998. 36. Sontag MK, Hammond K, Wagener JS, Accurso FJ. Maturation of sweat chloride and sodium in young children with early diagnosis of cystic fibrosis in Colorado. In: Neonatal Screening for Cystic Fibrosis, Caen Cedex, France: Presses Universitaires de Caen, 1999, p. 91-95.

37. Accurso FJ, Wagener JS, Sontag MK, Hammond KB. Strategies for effective early clinical intervention in cystic fibrosis. In: Neonatal Screening for Cystic Fibrosis, Caen Cedex, France: Presses Universitaires de Caen, 1999, p. 199-207. 38. Wagener JS, Sontag MK, and Accurso FJ. Prevalence of pseudomonas aeruginosa in children with cystic fibrosis detected by neonatal screening. In: Neonatal Screening for Cystic Fibrosis, Caen Cedex, France: Presses Universitaires de Caen, 1999, p. 199-207. 39. Wojtczak HA, Sontag MK, Wagener JS, Accurso FJ. Inhaled corticosteroid treatment in infants and young children with cystic fibrosis. Pediatr Pulmonol Suppl 19:105-6, 1999. 40. Larsen GL, Accurso FJ, Deterding RD, Halbower AC, Kerby, GS, White CW. Respiratory tract and mediastinum. Current Pediatric Diagnosis and Treatment 17:428-75, 2000. 41. Krawiec ME, Wenzel SE, Accurso FJ. Lipid Abnormalities and Cystic Fibrosis Lung Disease. Pediatr Pulmonol Suppl 20:159-60, 2000. 42. Welsh MJ, Ramsey BW, Accurso FJ, Cutting GR. Cystic Fibrosis in the Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill, New York, 3: 521-88, 2001. 43. Sagel SD, Dowell EB, Accurso FJ. Bacterial colonization and infection in the CF lung. Methods Mol Med.;70:433-46, 2002. 44. Vandivier RW, Fadok VA, Ogden CA, Hoffmann PR, Brain JD, Accurso FJ, Fisher JH, Greene KE, Henson PM. Impaired clearance of apoptotic cells from cystic fibrosis airways. Chest Mar;121(3 Suppl):89S-90S, 2002. 45. Chan ED, Choi HS, Cool C, Accurso FJ, Fantuzzi G. Interleukin-18 expression in cystic fibrosis lungs. Chest Mar; 121(3 Suppl): 85S-6S, 2002. 46. Brumbaugh DE, Accurso FJ. Persistent silent aspiration in a child with Trisomy 21. Curr Opin Pediatr. Apr;14(2):231-3, 2002. 47. Sagel SD, Accurso FJ. Monitoring inflammation in CF. Cytokines. Clin Rev Allergy Immunol. Aug;23(1):41- 57, 2002. 48. Accurso FJ, Sagel SD, Sontag MK, Wagener JS. How We Do It: Care of the Newly Diagnosed Infant with Cystic fibrosis identified through Newborn Screening- The Initial Visit. Pediatric Pulmonol Suppl 24:171, 2002. 49. Accurso FJ, Sontag MK. Seeking modifier genes in cystic fibrosis. Am J Respir Crit Care Med. 2003 Feb 1; 167(3): 289-90. 50. Wagener JS, Sontag MK, Accurso FJ, 2003. Newborn screening for cystic fibrosis. Curr Opin Pediatr. 2003 Jun;15(3):309-15. 51. Accurso FJ, Sagel SD, Sontag MK, Wagener, JS. Anti-Inflammatory Endpoints in Cystic Fibrosis (CF): Lessons from Clinical Trials. Pediatric Pulmol 25: 104-105, 2003. 52. Kerby GS, Larsen GL, Accurso FJ, Deterding RR, White CW, Sagel SD, Balasubramaniam V: Respiratory tract and mediastinum. In: Current Pediatric Diagnosis and Treatment (17th edition). Ed: WW Hay, Jr., RR Deterding, MJ Levin, JM Sondheimer. McGraw-Hill, New York, 2004. 53. Sontag MK, Accurso FJ. Gene Modifiers in Pediatrics: Application to Cystic Fibrosis. Adv Pediatr 51:5-36, 2004. 54. Accurso FJ. Curcumin and Cystic Fibrosis. J Pediatr Gastroenterol Nutr. 2004 Sep;39(3):235. 55. Accurso FJ, Sontag MK, Sagel SD, Wagener JS. Medical Consequences of Delayed Diagnosis. Pediatric Pulmonol Suppl 27: 180-181, 2004. 56. Kurgan L, Cios KJ, Sontag M, Accurso FJ. Mining the Cystic Fibrosis Data. Next Generation of Data-Mining Applications, IEEE Press, .415-444, 2005.

57. Bush A, Accurso FJ, Macnee W, Lazarus SC, Abraham E. Cystic fibrosis, pediatrics, control of breathing, pulmonary physiology and anatomy, and surfactant biology in AJRCCM in 2004. Am J Respir Crit Care Med. 2005 Mar 15;171(6):545-53. 58. Accurso FJ. Update in cystic fibrosis 2005. Am J Respir Crit Care Med. 2006 May 1;173(9):944-7. 59. Swiercz, W.; Cios, K.; Staley, K.; Kurgan, L.; Accurso, F. Sagel, S. A New Synaptic Plasticity Rule for Networks of Spiking Neurons. IEEE Press 94-105, 2006. 60. Kaye CI; Committee on Genetics; Accurso FJ, La Franchi S, Lane PA, Northrup H, Pang S, Schaefer GB. Introduction to the newborn screening fact sheets. Pediatrics. 2006 Sep;118(3):1304-12. 61. Kaye CI; Committee on Genetics; Accurso FJ, La Franchi S, Lane PA, Hope N, Sonya P, G Bradley S, Michele A LP. Newborn screening fact sheets. Pediatrics. 2006 Sep;118(3):e934-63. 62. Comeau AM, Accurso FJ, White TB, Campbell PW 3rd, Hoffman G, Parad RB, Wilfond BS, Rosenfeld M, Sontag MK, Massie J, Farrell PM, O'Sullivan BP; Cystic Fibrosis Foundation. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics, 2007 Feb; 119(2); e495-518. 63. Accurso FJ. Update in cystic fibrosis 2006. .Am J Respir Crit Care Med. 2007 Apr 15;175(8):754-7. 64. Rowe SM, Accurso F J, Clancy JP. Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proc Am Thorac Soc. 2007 Aug 1;4(4):387-98. 65. Hunsucker SW, Accurso FJ, Duncan MW. Proteomics in Pediatric Research and Practice, Adv Pediatr. 2007;54:9-28. 66. Gerszten RE, Accurso FJ, Bernard GR, Caprioli RM, Klee EW, Klee GG, Kullo IJ, Laguna TA, Roth FP, Sabatine M, Srinivas P, Wang TJ, Ware LB. Challenges in Translating Plasma Proteomics from Bench to Bedside: Update from the NHLBI Clinical Proteomics Programs. Am J Physiol Lung Cell Mol Physiol. 2008 May 2. 67. Accurso FJ, Sontag MK.Gene modifiers in cystic fibrosis. J Clin Invest. 2008 Mar;118(3):839-4. 68. Accurso FJ. Update in cystic fibrosis 2007. Am J Respir Crit Care Med. 2008 May 15;177(10):1058-61. 69. Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW 3rd; Cystic Fibrosis Foundation. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008 Aug;153(2):S4-S14. 70. Castro M, Ramirez MI, Gern JE, Cutting G, Redding G, Hagood JS, Whitsett J, Abman S, Raj JU, Barst R, Kato GJ, Gozal D, Haddad GG, Prabhakar NR, Gauda E, Martinez FD, Tepper R, Wood RE, Accurso FJ, Teague WG, Venegas J, Cole FS, Wright RJ. Strategic plan for pediatric respiratory diseases research: an NHLBI working group report. Proc Am Thorac Soc. 2009 Jan 15;6(1):1-10. 71. Abman S, Jobe A, Chernick V, Blaisdell C, Castro M, Ramirez MI, Gern JE, Cutting G, Redding G, Hagood JS, Whitsett J, Abman S, Raj JU, Barst R, Kato GJ, Gozal D, Haddad GG, Prabhakar NR, Gauda E, Martinez FD, Tepper R, Wood RE, Accurso FJ, Teague WG, Venegas J, Cole FS, Wright RJ, Gail D, Hamvas A, Kercsmar C, Kiley J, Weinmann G; NHLBI working group report. Strategic plan for pediatric respiratory diseases research: an NHLBI working group report. Pediatr Pulmonol. 2009 Jan;44(1):2-13. 72. Siparksy G, Accurso FJ, Pediatric Laboratory Medicine & Reference Ranges. In: Current Pediatric Diagnosis and Treatment (19th edition). Ed: WW Hay, Jr., MJ Levin, JM Sondheimer, RR Deterding, McGraw-Hill, New York, 2009. 73. Zemanick ET, Harris JK, Conway S, Konstan MW, Marshall B, Quittner AL, Retsch-Bogart G, Saiman L, Accurso FJ. Measuring and improving respiratory outcomes in cystic fibrosis lung disease: opportunities and challenges to therapy. J Cyst Fibros. 2009 Oct 13. [Epub ahead of print] PMID: 19833563 74. Borowitz, D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, Michel SH, Parad RB, White TB, Farrell PM, Marshall BC, AccursoFJ. Cystic Fibrosis Foundation Evidence-Based Guidelines for Management of Infants with Cystic Fibrosis J Pediatr 2009;155:S73-93. 22 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

75. Borowitz D, Parad RB, Sharp JK, Sabadosa, MPH, Karen A. Robinson KA, PhD, Rock MJ, Farrell PM, Sontag MK, Rosenfeld M, Davis SD, Marshall BC, Accurso FJ. Cystic Fibrosis Foundation Practice Guidelines for the Management of Infants with Cystic Fibrosis Transmembrane Conductance Regulator- Related Metabolic Syndrome during the First Two Years of Life and Beyond J Pediatr 2009;155:S106-16.

C. Publications Resulting From Multi-Center Trials and Committee Work 1. Correlation between genotype and phenotype in cystic fibrosis: Analysis of seven common mutations. The Cystic Fibrosis Genotype-Phenotype Consortium. NEJM 329:1308-1313, 1993. 2. Ramsey BW, Boat TF, Consensus Group. Outcome measures for clinical trials in cystic fibrosis. J Pediatr 124(2):177-192, 1994. 3. Quan JM, Tiddens HAWM, Sy JP, McKenzie SG, Montgomery MD, Robinson PJ, Wohl MEB, Konstan MW. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr 139:813-820, 2001. 4. Bartlett JR, Friedman KJ, Ling SC, Pace RG, Bell SC, Bourke B, Castaldo G, Castellani C, Cipolli M, Colombo C, Colombo JL, Debray D, Fernandez A, Lacaille F, Macek M Jr, Rowland M, Salvatore F, Taylor CJ, Wainwright C, Wilschanski M, Zemkova D, Hannah WB, Phillips MJ, Corey M, Zielenski J, Dorfman R, Wang Y, Zou F, Silverman LM, Drumm ML, Wright FA, Lange EM, Durie PR, Knowles MR; Gene Modifier Study Group. Genetic modifiers of liver disease in cystic fibrosis. JAMA 302(10):1076-83, 2009. PMID: 19738092 5. Treggiari MM, Rosenfeld M, Mayer-Hamblett N, Retsch-Bogart G, Gibson RL, Williams J, Emerson J, Kronmal RA, Ramsey BW; EPIC Study Group Early anti-pseudomonal acquisition in young patients with cystic fibrosis: rationale and design of the EPIC clinical trial and observational study.. Contemp Clin Trials. 2009 May;30(3):256-68. Epub 2009 Jan 15. Schechter MS, McColley SA, Silva S, Haselkorn T, Konstan MW, Wagener JS; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis; North American Scientific Advisory Group for ESCF. Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis. J Pediatr 155(5):634-9, 2009. [Epub 2009 Jul 16] PMID:19608199Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort. Rosenfeld M, Emerson J, McNamara S, Joubran K, Retsch-Bogart G, Graff GR, Gutierrez HH, Kanga JF, Lahiri T, Noyes B, Ramsey B, Ren CL, Schechter M, Morgan W, Gibson RL; EPIC Study Group Participating Clinical Sites. Pediatr Pulmonol. 2010 Sep;45(9):934-44. PMID: 20597081 [PubMed - indexed for MEDLINE] 6. 7.

D. Abstracts (*Selected for Presentation) 1. * Accurso FJ, Moore R, Cotton E, Orr W, Grunstein M, Brooks J. Comparison of magnetometry and body plethysmography for determination of respiratory timing and amplitude in infants. Am Rev Respir Dis 121:283A, 1980. 2. * Accurso FJ, Bailey D, Cotton E. Effect of syringe heparinization technique on arterial blood gas determination. Pediatr Res 14:588A, 1980. 3. * Ratner I, Hernandez J, Accurso FJ. Low peak inspiratory pressures (PIP) for ventilation of infants with hyaline membrane disease (HMD). Pediatr Res 15:676A, 1981.

4. * Koops BL, Abman SH, Accurso FJ. A 4-year clinical experience with follow-up of neonates with bronchopulmonary dysplasia on home oxygen. Published in Jugoslavenska Pedijatrija, Vol 24, Suppl I, 1981. 5. * Abman S, Accurso FJ, Koops B. Relationship of growth to improvement in oxygenation in infants with bronchopulmonary dysplasia. Clin Res 30:139A, 1982. 6. * Koops B, Abman S, Accurso FJ. Follow-up of premature infants with bronchopulmonary dysplasia. Pediatr Res 16:353A, 1982. 7. * Updike PA, Accurso FJ. Physiologic circadian rhythmicity in the human preterm infant. Am Rev Respir Dis 125:137A, 1982. 8. * Nichols WD, Accurso FJ, Morris HG, Grunstein MM. Effect of naloxone on respiratory parameters and beta endorphin levels in preterm infants. Clin Res 31:142A, 1982. 9. * Alpert BE, Accurso FJ, Gordon J, Taylor D, Cotton EK. Transcutaneous PO2 improves in patients with cystic fibrosis hospitalized for treatment of acute pulmonary exacerbations. Cystic Fibrosis Club Abstracts, 1982. 10. * Abman SH, Wolfe RR, Accurso FJ, Koops BL, Wiggins JW. Pulmonary vascular reactivity in infants with severe bronchopulmonary dysplasia. Clin Res 31:102A, 1983. 11. * Alpert B, Accurso FJ, Meschia G. Adaptation of fetal pulmonary blood flow to change in fetal arterial oxygen tension. Pediatric Research 17:370, 1983. 12. * Accurso FJ, Wilkening RB, Truog R, Meschia G. Adaptation of fetal pulmonary blood flow to acetylcholine and histamine. Clin Res 32(1):130A, 1984. 13. * Reardon MC, Hammond K, Accurso FJ, McCabe ERB, Cotton EK, Bowman CM. Nutritional and pulmonary abnormalities at the time of diagnosis of CF in infants identified by neonatal screening. Pediatr Res 18:402A, 1984. 14. * Truog R, Accurso FJ, Wilkening RB, Meschia G. Fetal pulmonary vasodilation with histamine: Mediation by H1 and H2 receptors. Pediatr Res 18:161A, 1984. 15. * Accurso FJ, Truog R, Wilkening RB, Meschia G. Adaptation of fetal pulmonary blood flow to pharmacologic vasodilators. Pediatr Res 18:385A, 1984. 16. * Abman SH, Accurso FJ, Bowman CM. Morbidity and mortality of protein-calorie malnutrition in infants with cystic fibrosis. Cystic Fibrosis Club Abstracts, 1984. 17. * Reardon MC, Hammond K, Cotton EK, Accurso FJ, McCabe ERB, Pinney M, Fisher C, Rumack C, Bowman CM. Nutritional and pulmonary abnormalities appearing in the first six months of life in newborns with cystic fibrosis identified by screening. Cystic Fibrosis Club Abstracts, 1984. 18. * Parker P, Hammond K, Reardon MC, Pinney M, Bowman CM, Cotton EK, Drenker J, Accurso FJ. Family psychosocial responses to the diagnosis of cystic fibrosis through newborn screening. Cystic Fibrosis Club Abstracts, 1984. 19. * Abman SH, Accurso FJ, Wilkening RB. Direct effect of tolazoline on fetal pulmonary blood flow. Clin Res 33:146A, 1985. 20. * Abman SH, Accurso FJ, Ward RM, Wilkening RB. Adaptation of fetal pulmonary blood flow to local infusion of tolazoline. Pediatr Res 19:148A, 1985. 21. * Accurso FJ, Wilkening RB. Adaptation of the fetal pulmonary circulation to an increase in fetal PO2 following fetal acidemia and maternal oxygen administration. Am Rev Respir Dis 131:A254, 1985. 22. * Abman SH, Wilkening RB, Accurso FJ. Temporal response of the fetal pulmonary circulation to local infusion of arachidonic acid and prostaglandins. Am Rev Respir Dis 131:A255, 1985. 23. * Abman SH, Accurso FJ, Wilkening RB, Meschia G. Persistent fetal pulmonary vasoconstriction following acute hypoxia. Clin Res 34:151A, 1986 and Pediatr Res 20:470A, 1986.

24. * Accurso FJ, Abman S, Wilkening RB, Worthen S, Henson PM. Exogenous platelet activating factor produces pulmonary vasodilation in the ovine fetus. Am Rev Respir Dis 133:A11, 1986. 25. * Stall C, Reardon MC, Hammond K, Abman SH, Sokol R, Accurso FJ. Fat malabsorption in infants with cystic fibrosis less than 3 months of age diagnosed through newborn screening. CF Club Abstracts, 1986. 26. * Abman SH, Harbeck RJ, Butler-Simon NA, Reardon MC, Ogle JW, Accurso FJ. Early Pseudomonas aeruginosa (PA) colonization in young infants with cystic fibrosis (CF) identified by newborn screening. Pediatr Res 21:499A, 1987. 27. * Accurso FJ, Abman SH, Wilkening RB. Desensitization of the fetal pulmonary circulation to vasodilatory stimuli. Chest 93:184S, 1988. 28. * Abman SH, Reardon MC, Harbeck RJ, Ogle JW, Butler-Simon N, Hammond KB, Accurso FJ. Early bacteriologic, immunologic and clinical courses of young infants with cystic fibrosis (CF) identified by newborn screening. Pediatr Pulmonol Suppl 1:124, 1987. 29. * Pinney MA, Abman SH, Accurso FJ. Reproductive decision-making in families of infants with cystic fibrosis (CF) diagnosed through newborn screening. Pediatr Pulmonol Suppl 1:142, 1987. 30. * Accurso FJ, Abman SH, Reardon MC, Stall CD, Pinney MA, Hammond KB, Sokol, R.J. Prospective longitudinal evaluation of growth in young infants with cystic fibrosis diagnosed through newborn screening. Pediatr Pulmonol Supp 1:138, 1987. 31. * Abman SH, Accurso FJ. Acute effects of partial compression of the ductus arteriosus on pulmonary blood flow in the fetal lamb. Pediatr Res 23:560A, 1988. 32. * Accurso FJ, Abman SH. Fetal pulmonary vascular responses to cyclic nucleotides, atrial natriuretic peptide (ANP), and sodium nitroprusside (SNP). Clin Res 36:239A, 1988 and Pediatr Res 23:561A, 1988. 33. * Chatfield B, Abman S, Stall C, Sokol R, Accurso FJ. Growth, fat malabsorption and nitrogen balance in young infants with cystic fibrosis. Clin Res 36:223A, 1988. 34. * Abman SH, Shanley PF, Accurso FJ. Chronic intrauterine pulmonary hypertension alters perinatal pulmonary vasoreactivity and structure. Pediatr Res 23:561A, 1988. 35. * Chatfield B, Abman SH, Hammond KB, Stall CD, Sokol RJ, Accurso FJ. Poor growth and steatorrhea in infants with cystic fibrosis (CF) under 3 months of age. Pediatr Res 23:300A, 1988. 36. * Helton J, Hammond K, Accurso FJ. Parental attitude toward newborn screening for cystic fibrosis. In, Proceedings of the Conference Internationale Mucoviscidose: Depistage Neonatal et Prise en Charge Precoce Ed: Travert G. Caen, France, 1988. 37. * Accurso FJ, Hammond K, Helton J, Abman S. Respiratory morbidity in neonates and infants with CF identified through newborn screening. Pediatr Pulmonol, Suppl 4:139-140, 1989. 38. * Helton J, Hammond K, Robinson N, Accurso FJ. Parental attitude toward newborn screening for CF. Pediatr Pulmonol Suppl 4:155, 1989. 39. *Abman SH, Accurso FJ. Time-dependent effects of vasodilator stimuli on the pressure-flow relationship of the fetal pulmonary circulation. Pediatr Res 25:363A, 1989. 40. *Accurso FJ, Abman SH, Sokol RJ, Hammond KB. Newborn screening for cystic fibrosis (CF): laboratory diagnosis and early clinical course. Pediatr Res 25:363A, 1989. 41. *Accurso FJ, Abman S, Hammond K. Newborn screening for cystic fibrosis: Laboratory diagnosis and early clinical course. Am Rev Respir Dis 139:A69, 1989. 42. * Dove AM, Szefler SI, Hill MR, Larsen GL, Accurso FJ. Altered prednisolone pharmacokinetics in patients with cystic fibrosis. Am Rev Respir Dis 141:87A, 1990. 43. * Helton J, Accurso FJ, Robinson N, Harmon R. Reproductive decision making in newborn screen families. Pediatr Pulmonol Suppl 5:282A, 1990.

44. * Krebs N, Hambidge KM, Accurso FJ, Bronstein MN. Zinc (ZN) status of infants with cystic fibrosis (CF) prior to therapy. Pediatric Pulmonol Suppl 5:262A, 1990. 45. * Accurso FJ, Fall L, Hammond K, Seltzer W. Incidence of Delta 508 deletion in infants with cystic fibrosis (CF) identified through immunoreactive trypsin (IRT) based newborn screening. Pediatric Pulmonol Suppl 5:199A, 1990. 46. * Rusakow L, Abman S, Hammond K, Sokol R, Accurso FJ. Immunoreactive trypsin (IRT) in infants with cystic fibrosis (CF) complicated by meconium ileus (MI). Pediatr Pulmonol Suppl 5:289A, 1990. 47. * Bronstein MN, Accurso FJ, Hambidge KM, Sokol RJ, Hammond KB. Assessment of Nutritional status in cystic fibrosis infants identified through newborn screening. FASEB Journal 5:A1238, 1991. 48. * Bronstein B, Accurso FJ, Davies PSW, Hambidge KM. Assessment of total energy expenditure in cystic fibrosis infants identified through newborn screening. Pediatr Res, 29:101A, 1991. 49. * Mohon RT, Abman SH, Wagener JS, Accurso FJ. Pulmonary function and bronchodilator responsiveness in asymptomatic infants with cystic fibrosis (CF) diagnosed through newborn screening. Am Rev Respir Dis 143:A296, 1991. 50. * Bronstein MN, Accurso FJ, Hambidge KM, Chatfield BA, Sokol RJ, Hammond K. Assessment of early nutritional status in cystic fibrosis infants identified through newborn screening. Pediatr Pulmonol Suppl 6:237A, 1991. 51. * Mohon RT, Wagener JS, Accurso FJ, Abman SH. Early pulmonary function and bronchodilator responsiveness in infants with cystic fibrosis (CF) diagnosed through newborn screening. Pediatr Pulmonol Suppl 6:283A, 1991. 52. * Mohon RT, Wagener JS, Accurso FJ. Relationship of genotype to pulmonary function in infants with cystic fibrosis identified through neonatal screening. Pediatr Pulmonol Suppl 8:302, 1992. 53. * Khan T, Wagener JS, Riches DWH, Accurso FJ. Increased interleukin-8 levels and gene expression by pulmonary macrophages in bronchoalveolar lavage fluid from infants with cystic fibrosis. Clin Res 41:2A, 1993 and Amer Rev Resp Dis 147:463A, 1993. 54. * Krebs NF, Miller LV, Fennessey PV, Accurso FJ, Hambidge KM. Excessive fecal losses of endogenous zinc in infants with cystic fibrosis. Pediatr Res 33:103A, 1993. 55. * Sokol RJ, Erskine J, Abman S, Wagener J, Hammond K, Accurso FJ. Prospective study of fat-soluble vitamin status in 101 infants with cystic fibrosis identified by newborn screening. Pediatr Pulmonol Suppl 9:279, 1993. 56. * McCool PL, Wagener JS, Accurso FJ. Gender differences during progressive incremental exercise in cystic fibrosis: Assessment of oxygen uptake. Pediatr Pulmonol Suppl 9:254, 1993. 57. * Giles DR, Accurso FJ, Wagener JS. Acute effects of postural drainage and clapping vs. autogenic drainage in oxygen saturation and sputum recovery in cystic fibrosis. Pediatr Pulmonol Suppl 9:252, 1993. 58. * Mohon RT, Wagener JS, Accurso FJ. Prospective longitudinal pulmonary function in infants with cystic fibrosis identified through newborn screening. Pediatr Pulmonol Suppl 9:241, 1993. 59. * Kirchner KK, Khan TZ, Wagener JS, Accurso FJ. Increased DNA levels in bronchoalveolar lavage fluid obtained from infants with cystic fibrosis. Pediatr Pulmonol Suppl 9:316, 1993. 60. * Sokol RJ, Krebs NF, Bronstein MN, Stall CD, Abman SH, Hammond KB, Accurso FJ. Randomized trial of predigested formula vs. standard infant formula in infants with cystic fibrosis (CF) identified by newborn screening. Pediatric Research 35(4):136A, 1994. 61. * Khan TZ, Wagener JS, Riches DWH, Accurso FJ. Increased monocyte chemoattractant protein-1 gene expression by pulmonary macrophages in infants with cystic fibrosis. Am J Resp Crit Care Med 149(4):667A, 1994.

62. * Sokol RJ, Narkewicz MR, Carroll NM, Wagener JS, Accurso FJ. Prospective evaluation of liver blood tests during the first 8 years of life in children with cystic fibrosis identified by newborn screening. Pediatr Pulmonol Suppl 10:345, 1994. 63. * Copenhaver SC, Khan TZ, Wagener JS, Johnson Z, Vasil A, Accurso FJ, Vasil M. Airway inflammation in the absence of detectable Pseudomonas aeruginosa by culture and PCR in infants with cystic fibrosis. Pediatr Pulmonol Suppl 10:372, 1994. 64. * Narkewicz MR, Sokol RJ, Lear JL, Wagener JS, Accurso FJ. Effect of ursodeoxycholic acid (UDCA) therapy for CF liver disease on indirect tests of hepatic function. Pediatr Pulmonol Suppl 10:342, 1994. 65. * Accurso FJ, Wagener JS, Abman SH, Sokol RJ, Carroll NM, Hammond KB. Growth and pulmonary function outcome in children with cystic fibrosis identified through newborn screening. Pediatr Pulmonol Suppl 10:419, 1994. 66. * Khan TZ, Copenhaver SC, Kirchner KK, Wagener JS, Carroll NM, Accurso FJ. Serial assessment of airway inflammation in infants with cystic fibrosis identified through newborn screening. Pediatr Pulmonol Suppl 10:373, 1994. 67. * Copenhaver SC, Vasil AI, Johnson Z, Wagener JS, Accurso FJ, Vasil ML. Constant strain-specific identification of Pseudomonas aeruginosa in an individual cystic fibrosis patient by PCR and southern hybridization over a ten year period in childhood. Pediatr Pulmonol Suppl 10:264, 1994. 68. *MacEntee MV, Khan TZ, Stenmark KS, Riches DWH, Accurso FJ. Airway inflammation and bacterial colonization in stable pediatric patients with tracheostomies. Submitted Am J Resp Crit Care Med, 1994. 69. * Khan TZ, Gillespie MMK, Parsons PE, Wagener JS, Riches DWH, Accurso FJ. Airway inflammation in infants with Cystic Fibrosis can occur in the absence of detectable endotoxin in bronchoalveolar lavage fluid (BAL). Submitted Am J Resp Crit Care Med, 1994. 70. * Erskine JM, Lingard C, Accurso FJ, Krebs NF. Enteral nutrition for patients with Cystic Fibrosis: Comparison of a semi-elemental and non-elemental formula. Pediatr Pulmonol Suppl 12:295, 1995. 71. * Erskine JM, Accurso FJ, Davies PSW. Longitudinal measurement of energy expenditure in infants with Cystic Fibrosis identified by newborn screen. Pediatr Pulmonol Suppl 12:296, 1995. 72. * Butler-Simon N, McCool P, Giles D, Pritts J, Accurso FJ, Wagener J. Efficacy and desirability of autogenic draining vs. conventional postural drainage and percussion. Pediatr Pulmonol Suppl 12:265, 1995. 73. * Copenhaver SC, Sage AE, Cohen JJ, Vasil AI, Accurso FJ, Vasil ML. Purified phospholipase from pseudomonas aeruginosa in cellular necrosis and apoptosis in human monocytes and rat thymocytes. Pediatr Pulmonol Suppl 12:225, 1995. 74. * Accurso FJ, Hammond KB, Copenhaver SC, Khan TZ, Wagener JS. Elevated mucin associated carbohydrate antigen 19-9 in bronchoalveolar lavage fluid obtained from young children with Cystic Fibrosis. Pediatr Pulmonol Suppl 12:320, 1995.

75. * Wagener JS, Copenhaver SC, Khan TZ, Accurso FJ. Correlation of circulating and airway elastase/a1 antiproteinase complex in infants with Cystic Fibrosis. Pediatr Pulmonol Suppl 12:321, 1995. 76. * Accurso FJ, Ahrens R, Birrer P, Eigen H, Johnson C, Kirchner K, McCubbin M, Nickerson B, Palazzo R, Rock M, Wagener J. Deposition of Aerosolized rhDNase in Infants and Children with Cystic Fibrosis. Israel Journal of Medical Science Suppl 32:189, 1996. 77. * Wojtczak HA, Wagener JS, Accurso FJ. Inhaled Steroid Effects on Airway Inflammation in Children with Cystic Fibrosis. Israel Journal of Medical Statistics Suppl 32:189, 1996. 78. * Copenhaver S, Sage A, Vasil A, Johnson Z, Sontag M, Accurso FJ. BALF PCR and ELISA Identification of Non-Culturable Pseudomonas Aeruginosa in Infants and Toddlers with Cystic Fibrosis. Pediatr Pulmonol Suppl 13: 296, 1996. 79. * Wojtczak HA, Wagener JS, Kerby G, Gotlin G, Accurso FJ. Effect of Inhaled Beclomethasone Diproprionate on Airway Inflammation in Children with Cystic Fibrosis. Pediatr Pulmonol Suppl 13: 414, 1996.

80. * Kerby G, Wojtczak HA, Accurso FJ, Riches D. Regulation of Interleukin - 8 mRNA Levels in Airway Macrophages in Cystic Fibrosis in Response to inhaled steroids. Pediatr Pulmonol Suppl 13: 413, 1996. 81. * Eichner J, Hay T, Sontag M, Accurso FJ. Comparison of the Brasfield and Wisconsin Chest Radiograph Scoring Systems for Detecting Lung Disease Progression in Infants and Children with Cystic Fibrosis Identified through Newborn Screening. 79. Pediatr Pulmonol Suppl 13: 334, 1996. 82. * Harris BE, Sontag M, Wagener J, Accurso FJ. Longitudinal determination of the Decline in Lung Function in School Age Children with Cystic Fibrosis Identified through Newborn Screening. Pediatr Pulmonol Suppl 13: 488, 1996. 83. * Giles D, Sontag M, Wagener J, Accurso FJ. Effect of One Month of Treatment with Flutter Valve or Postural Drainage and Clapping on Pulmonary Function and Sputum Recovery in Cystic Fibrosis. Pediatr Pulmonol Suppl 13: 354, 1996. 84. * Sontag M, Mohon R, Zerbe G, Accurso FJ. Longitudinal Mixed Effects Modeling of Lung Function in Infants with Cystic Fibrosis Identified through Newborn Screening. Pediatr Pulmonol Suppl 13: 327, 1996. 85. * Vendegna T, Ballard R, Miller Y, Accurso FJ. Airway Substance P and Neutral Endopeptidase in Adults with Cystic Fibrosis. Pediatr Pulmonol Suppl 13: 449, 1996. 86. * Eid N, Accurso FJ, Stenzler A. Distribution of Ventilation in Infants And Young Children With Cystic Fibrosis. Pediatr Pulmonol Suppl 13: 322, 1996. 87. * Rock C, Kirchner K, McCubbin M, Accurso FJ, Ahrens R, Birrer P, Eigen H, Johnson C, Nickerson B, Palazzo R, Wagener J. Aerosol Delivery and Safety of rhDNase in Young Children with Cystic Fibrosis: A Bronchoscopic Study. Pediatr Pulmonol Suppl 13: 268, 1996. 88. * McCubbin M, Kirchner K, Ahrens R, Rock M, Accurso FJ, Nickerson B, Palazzo R, Birrer P, Eigen H, Wagener J. Bronchoscopy with BAL Detects Early Inflammation and Infection in Young CF Patients: A Multicenter Study. Pediatr Pulmonol Suppl 13: 324, 1996.

89. * Sontag MK, Hammond KB, Accurso FJ. Incidence of F508 mutation in infants with cystic fibrosis who are false negative for immunoreactive trypsinogen based newborn screening. Pediatr Pulmonol Suppl 14: 152, 1997. 90. * Lingard CD, Sontag MK, Hammond KB, Accurso FJ. Development of pancreatic insufficiency in infants with cystic fibrosis identified through newborn screen: longitudinal relationship to serum immunoreactive trypsinogen. Pediatr Pulmonol Suppl 14: 381, 1997. 91. * Sontag MK , Drescher AA, Accurso FJ, Krebs NF. Longitudinal characterization of growth and growth tracking in infants and toddlers with cystic fibrosis identified through newborn screen. Pediatr Pulmonol Suppl 14: 357, 1997. 92. * Wagener JS, Sontag MK, Accurso FJ. Long term stability of airway inflammation in infants and young children with cystic fibrosis. Pediatr Pulmonol Suppl 14: 420, 1997. 93. * Krebs NF, Sontag MK, Accurso FJ, Hambidge M. Plasma zinc and albumin in cystic fibrosis infants identified through newborn screening: relationship to enzyme therapy. Pediatr Pulmonol Suppl 14: 377, 1997. 94. * Rosenfeld M, Accurso FJ, Armstrong D, Castile R, Emerson J, Grimwood K, Hiatt P, McCoy K, Ramsey B, Wagener J. The diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. Pediatr Pulmonol Suppl 14: 325, 1997. 95. * Greene KE, Ye SH, Mason RJ, Accurso FJ. BAL surfactant protein-A levels are decreased in infants with CF. Pediatr Pulmonol Suppl 14: 289, 1997. 96. * Accurso FJ, Sontag MK, Wagener JS, Hammond KB. Cystic fibrosis in infants with persistent hypertrypsinogenemia and “borderline” sweat tests. Pediatr Pulmonol Suppl 17: 292, 1998. 97. * Sontag MK, Hammond KB, Wagener JS, Accurso FJ. Maturation of sweat chloride and sodium in young children with early diagnosis of cystic fibrosis. Pediatr Pulmonol Suppl 17: 252, 1998.

98. * Wojtczak HA, Wagener JS, Sontag MK, Accurso FJ. Bronchoalveolar lavage assessment of airway delivery of fluticasone proprionate via pressurized metered dose inhaler in infants and young children with cystic fibrosis. Pediatr Pulmonol Suppl 17: 341, 1998. 99. *Accurso FJ, Sontag, MK, Wagener, JS. Relationship of bronchoalveolar lavage levels of antimicrobial proteins to airway inflammation in cystic fibrosis. Pediatr Pulmonol Suppl 17: 388, 1998. 100. * Wagener JS, Sontag MK, Accurso FJ. Prevalence of Pseudomonas aeruginosa (PA) in children with cystic fibrosis (CF) detected by neonatal screening. To be presented at the Fifth International Conference on Neonatal Screening for Cystic Fibrosis Conference. Caen, France. September 1998. 101. * Thatayatikom A, Accurso FJ, Gelfand EW. Bronchiolitis obliterans organizing pneumonia (BOOP) associated with lymphopholiferative disorder in common variable immunodeficiency. American College of Allergy, Asthma, & Immunology Abstract, 1999. 102. * Noone PG, Accurso FJ, Aitken ML, Dovey M, Gibson RL, Konstan M, Rodman DM, Wilmott RW, Zeitlin P, Gorden J, Shaffer C. Safety of aerosolized INS365 in patients with mild to moderate CF: results of a multicenter therapeutic development network study. Pediatr Pulmonol Suppl 19:244-245, 1999. 103.* Rubio F, Cooley J, Accurso FJ, Remold-O’Donnell E. Role of neutrophil proteases and monocyte/neutrophil elastase inhibitor (MNEI) in cleavage of pulmonary surfactant protein-A (SP-A). Pediatr Pulmonol Suppl 20:255, 2000. 104. * Sagel SD, Osberg I, Kapsner R, Accurso FJ. Assessment of airway inflammation and infection in non- expectorating children with cystic fibrosis through sputum induction. Pediatr Pulmonol Suppl 20:297, 2000. 105. * Chatfield BA, Jackson WD, Henshaw C, Accurso FJ. New Diagnoses of Cystic Fibrosis. Can We Justify Not Screening All Newborns? Pediatr Pulmonol Suppl 20:313, 2000. 106. * Vandivier RW, Fadok VA, Brain JD, Accurso FJ, Henson PM. Neutrophil elastase cleaves receptors for ingestion of apoptotic cells; Implications for airway accumulation of apoptotic cells in cystic fibrosis. Am J Resp Crit Care Med 163 (5): A489, 2001. 107.* Wooldridge JL, West J, Gao B, Geraci M, Accurso FJ. Microarray Determination of Gene Expression in Cystic Fibrosis (CF) and Normal Whole Lung. Am J Resp Crit Care Med 163 (5): A563, 2001. 108. * Saavedra M, West J, Vasil M, Geraci M, Accurso FJ, Rodman D. P. Aeruginosa (PA) Human Airway Epithelial (HAE) Cell Interaction: Analysis by DNA Microarray. Am J Resp Crit Care Med 163 (5): 564, 2001. 109. * Wallick K, Wooldridge J, Geraci M, Accurso FJ, Rodman D. Microarray Analysis of Airway Epithelial RNA from Bronchial Brushings in Cystic Fibrosis. Am J Resp Crit Care Med 163 (5): 566, 2001. 110. * Modi AC, Davis MA, Quittner AL, Accurso FJ, Koenig, J. The Relationship Between Demographic and Health Status Variables and Health-Related Quality of Life in Individuals with Cystic Fibrosis, submitted 2001. 111. * Cooley J, Rubio F, Sontag M, Osberg I, Accurso FJ, Remold-O’Donnell E. MNEI (Monocyte/Neutrophil Elastase Inhibitor) is Found at Increased Levels in Cystic Fibrosis Bronchoaveolar Lavage Fluid. Pediatr Pulmonol Suppl 22:271, 2001. 112.* Wallick K, Geraci M, Accurso FJ, Rodman D. Microarray Analysis of Airway RNA from Nasal Scrapings in Cystic Fibrosis, submitted Pediatr Pulmonol, 2001. 113. * Sagel SD, Kapsner R, Osberg I, Sontag MK, Accurso FJ. Indices of Inflammation in Saliva from Children with Cystic Fibrosis and Healthy Children. Pediatr Pulmonol Suppl 22:274, 2001. 114. * Wooldridge JL, Osberg I, Sontag MK, Wagener JS, Abman SH, Accurso FJ. Bronchoscopic Determination of Airway Nitric Oxide and Metabolites in Children with Cystic Fibrosis. Pediatr Pulmonol Suppl 22:277, 2001. 115. * Wooldridge JL, Sontag MK, West J, Gao B, Geraci M, Accurso FJ. Microarray Determination of Gene Expression in Cystic Fibrosis (CF) and Control Whole Lung Tissue. Pediatr Pulmonol Suppl 22:278, 2001. 116.* Wallick KA, Jones M, Pickard C, Brayshaw S, Accurso FJ, Rodman DM, Nick JA. Outpatient monitoring of pulmonary function in adults with cystic fibrosis. Pediatr Pulmonol Suppl 22:299, 2001.

117.* Brody AS, Long F, Castile R, Crotty E, Bean J, Quan J, Accurso FJ. High-resolution CT demonstrates progressive bronchiectasis in young CF patients with mild lung disease. Pediatr Pulmonol Suppl 22:300, 2001. 118.* Modi AC, Davis MA, Quittner AL, Accurso FJ, Koenig J. The Relation Between Demographic and Health Status Variables and Health-Related Quality of Life in Individuals with Cystic Fibrosis. Pediatr Pulmonol Suppl 22:349, 2001. 119.* Modi A, Davis M, Quittner A, Buu A, Koenig J, Accurso FJ. Validation of a Disease Specific Quality of Life Measure for Cystic Fibrosis Across Two Samples. Journal of Cystic Fibrosis, 328, 2001. 120.* Davis M, Modi A, Quittner A, Buu A, Koenig J, Accurso FJ. Psychometric Analysis of the Cystic Fibrosis Questionaire for Parents of School-Age Children, Journal of Cystic Fibrosis, 329, 2001. 121.* Nick JA, Saaverda M, Poch K, West J, Malcolm K, Avdi NJ, Accurso FJ, Rodman DM, Vasil ML, Worthen GS. A Coordinated Functional and Genomic Analysis of the Response of Human Neutrophils to Pseudomonas aeruginosa: Contribution of the Iron-Regulated PA01 Virulence to the Inflammatory Response. Pediatr Pulmol Suppl 24:269, 2002. 122.* Wooldridge JL, Deutsch GH, Sontag MK, Osberg I, Chase DR, Wagener JS, Silkoff PE, Accurso FJ. The Nitric Oxide Pathway in Infants and Young Children with Cystic Fibrosis. Pediatr Pulmonol Suppl 24:273, 2002. 123.* Ordonez CL, Gibson RL, Julia E, McNamara S, Accurso FJ, Burns JL, Zeitlin PL, Daines C, Retsch-Bogart GZ, Chmiel JF, Henig NR, Moira AL. Validation of Sputum Induction as an Outcome Measure for Lower Airway Sampling in Patients with Cystic Fibrosis (CF). Pediatr Pulmonol Suppl 24:299, 2002. 124.* Gibson RL, Emerson J, McNamara S, Burns JL, Rosenfeld M, Yunker A, Hamblett N, Accurso FJ, Dovey M, Hiatt P, Moss R, Konstan M, Retsch-Bogart G, Waltz D, Wilmott R, Wagener J, Zeitlin P, Ramsey B. A Randomized, Controlled Trial of Inhaled Tobramycin in Young Children with Cystci Fibrosis: Eradication of Pseudomonas From the Lower Airway. Pediatr Pulmonol Suppl 24:300, 2002. 125.* Wallick K, Sontag M, Jones M, Brayshaw S, Accurso FJ, Rodman D, Nick J. Clinical Characteristics of Adult Patients Over Age 40 with Cystic Fibrosis: Significance of Early and Delayed Diagnosis. Pediatr Pulmonol Suppl 24:319, 2002. 126.* Narkewicz MR, Sontag M, Accurso FJ, Sokol RJ, Wagener J. Prevalence and Persistence of Abnormal Aminotransferases in Children with Cystic Fibrosis Diagnosed by Newborn Screen. Pediatr Pulmonol Suppl 24:335, 2002. 127.* Sontag MK, Zerbe GO, Hokanson JE, Anthony MM, Corey M, Marshall JA , Rock M, Splaingard M, Kawasoe J, Li Z, Tsui, L-C, Zielenski J, Accurso FJ. Cfm1 Locus As a Potential Genetic Modifier of Early Pancreatic Disease in Newborn Screened Children with Cystic Fibrosis. Pediatric Pulmonol 25:216, 2003. 128.* Sokol R, Osberg I, Sontag MK, Wagener JS, Accurso FJ. Reduced Serum Coenzyme Q-10 Concentrations In Children With Cystic Fibrosis. Pediatric Pulmonol 25:338, 2003. 129.* Nick JA, Saavedra M, Poch K, Walker TS, Malcolm K, Avdi NJ, Accurso FJ, Higgins LS, Vasil ML, Worthen GS. Selective Modifications of the Response of Human Neutrophils to Pseudomonas aeruginosa by inhibition of p38 Map Kinase. Pediatric Pulmonol 25:269, 2003. 130.* Saavedra MT, Nick J, West J, Potter B, Accurso FJ, Randell S, Vasil M, Rodman D. A Novel Zinc Finger Protein Inhibits Activation of NF-kB in Airway Epithelial Cells Infected with Pseudomonas aeruginosa Strain PAO1. Pediatric Pulmonlol 25:273, 2003. 131.* Morton RL, Hess DR, Gelfand AS, Julius SM, Konstan MW, Accurso FJ. The National Airway Clearance Registry (NACR) for Patients with Respiratory Neurological and Other Conditions Associated with Airway Clearance Complications. Am J Resp Crit Care Med 169 (7) A380 2004. 132.* Lee RL, Rancourt RC, del Val G, Accurso FJ, White CW. Inhibition of Cystic Fibrosis Sputum Elastase Activity by E. coli and Human Thioredoxins. Am J Resp Crit Care Med 169 (7) A387 2004. 133.* Lee RL, Rancourt RC, Pack KM, del Val G, Penvari C, Accurso FJ, White CW. Liquefaction of CF Sputum by Dihydrolipoic Acid. Pediatric Pulmonol Suppl 27:255, 2004.

134.* Moss RB, Milla C, Colombo J, Clancy JP, Zeitlin P, Spencer T, Accurso FJ, Pilewski J, Waltz DA, Dorkin HL, Ferkol T, Pian M, Ramsey B, Martin D, Anklesaria P, Heald AE. A Multicenter Double-Blind Placebo- Controlled. Phase IIB study of Aerosolized TGAAVCF for the Treatment of Cystic Fibrosis. Pediatric Pulmonol Suppl 27: 257, 2004. 135.* Saavedra MT, Accurso FJ, Rodman D, Nick J. Serious Pleural Space Infections in Cystic Fibrosis. Pediatric Pulmonol Suppl 27:302, 2004. 136.* Saavedra MT, Fierro B, West J, Nick J, Randell S, Vasil M, Accurso FJ, Rodman D. LKLF Inhibits NF-kB Activity and Mediates its Nuclear Binding in Human Airway Cells. Pediatric Pulmonol Suppl 27: 269, 2004. 137.* Accurso FJ, Sontag MK, Koenig JM, Quittner AL. Multi-Center Airway Secretion Clearance Study in Cystic Fibrosis. Pediatric Pulmonol Suppl 27:317, 2004. 138.* Quittner AL, Modi AC, Accurso FJ, Koenig JM, Sontag MK, Oermann C, Sockrider M. Treatment Satisfaction, Health Related Quality of Life and Airway Clearance Therapies in Patients with Cystic Fibrosis. Pediatric Pulmonol Suppl 27:314, 2004. 139.* Dolan SA, Dowell E, John LJ, Valdez S, Heltzel D, Chan K, Accurso FJ, Wagener J, James JF. An Outbreak of Burkholderia Cepacia Complex Associated with an Intrinsically Contaminated Nasal Decongestant Spray. Pediatric Pulmonol Suppl 27: 320, 2004. 140.* Saavedra MT, Patterson A, Randell S, Accurso FJ, Vasil M, Rodman D, Nick J. Activated Human Neutrophils Inhibit Expression of Anti Inflammatory Transcription Factor LKLF in Human Airway Cells. Pediatric Pulmonol Suppl 28:231, 2005. 141.* Day BJ, Belsor LW, Accurso FJ. Alterations in Broncoaveolar Lavage Fluid Lactate Levels in Cystic Fibrosis and CFTR KO Mice. Pediatric Pulmonol Suppl 28:236, 2005. 142.* Papas K, Pardee C, Sokol, R, Accurso FJ, Wagener J. Treatment of Cystic Fibrosis Patients with Antioxidant Solution: A Pilot Study. Pediatric Pulmonol Suppl 28:263, 2005. 143.* Harris JK, De Groote M, Sagel S, Kapsner R, Penvari C, Kaess H, Helsche S, Accurso FJ, Pace N. Ribosomal RNA Sequence-Based Indentification of Unusual Bacteria in the Airway of Children with Cystic Fibrosis. Pediatric Pulmonol Suppl 28:287, 2005. 144. * De Groote M, Harris K, St.Amand A, Kapsner R, Kaess H, Pace N, Accurso FJ. In Situ Hybridizaiton VS PCR Detection of M. Abscessus in Airway Specimens from Children with Cystic Fibrosis. Pediatric Pulmonol Suppl 28:292, 2005. 145. * Hunsucker SW, Baltz JL, Accurso FJ, Duncan MW. Proteomic Discovery of Uniary Biomarkers in Cystic Fibrosis. Pediatric Pulmonol Suppl 28:309, 2005. 146. * Sagel SD, Heltshe S, Accurso FJ. Prediction of Long Term Lung Function Decline Through use of Sputum Biomarkers of Inflammation. Pediatric Pulmonol Suppl 28:314, 2005. 147. *Deterding RR, Laguna T, Emmett R, Sontag M, Accurso FJ. Cytokine Profiles in Bronchoalveolar Lavage fluid from Children with Interstitial Lung Disease. Am J Resp Crit Care Med. Vol 3. Abstracts Issue. 164A. 2006. 148.* Laguna TA, Heltshe S, Sontag MK, Osberg I, Cooper A, Pardee C, Accurso FJ. Effect of different sample processing techniques and overnight transport on sputum inflammatory mediators in Cystic Fibrosis. Pediatric Pulmonol Suppl 29:163, 2006. 149.* Searing DA, Sagel SD, Heltshe SL, Kaess H, Sontag MK, Accurso FJ. Sweat chloride as a biomarker for treatment effect of molecules with cystic fibrosis transmembrane conductance regulatory (CFTR) activity). Pediatric Pulmonol Suppl 29:259, 2006. 150.* Sagel SD, Sontag M, Emmett P, Zhang L, Kaess H, Wagener J, Accurso FJ. Value of systemic biomarkers of inflammation in children with CF: data from the Pulmozyme early intervention trial. Pediatric Pulmonol Suppl 29:386, 2006. 151.* Wagener J, Sontag M, Pardee C, Sokol RJ, Accurso FJ. Impact of an antioxidant solution on airway inflammation in patients with cystic fibrosis. Pediatric Pulmonol Suppl 29:508, 2006.

152. * Remold-O'Donnell, Cooley J, McDonald B, Accurso FJ, Crouch EC. Cleavage of Surfactant Protein-D: Implications for Cystic Fibrosis. Am J Resp Crit Care Med. 2007: 175 A320. 153. * Chmiel JF, Konstan MW, Lymp J, Mayer-Hamblett N, Hilliard KA, Accurso FJ, Ramsey B. Assessment of Induced Sputum as a Tool to Evaluate Anti-Inflammatory Agents in CF. Pediatric Pulmonol Suppl 30:228, 2007. 154. * LaVange L, Engels J, Accurso FJ. Analyzing Percent Change in Cystic Fibrosis Clinical Trials. Pediatric Pulmonol Suppl 30:290, 2007. 155. * Harris JK, Zemanick ET, Sagel SD, Accurso FJ, Pace NR. Molecular Identification of Bacteria in the CF Airway. Pediatric Pulmonol Suppl 30:312, 2007. 156.* Laguna TA, Sontag MK, Sagel SD, Accurso FJ. Urinary and Sputum Desmosine Levels During Pulmonary Exacerbation in Pediatric Cystic Fibrosis. E-PAS2008:3450.1 and Am J Resp Crit Care Med 2008 A461. 157. * Zemanick ET, Harris JK, Sagel SD, Accurso FJ. Microbia Comunities in Children with Cystic Fibrosis and Clincially Stable Lung Disease. E-PAS2008:3450.3 and Am J Resp Crit Care Med 2008 A461. 158. * Sontag MK, Wagener JS, Accurso FJ, Sagel SD. Consistent Incidence of Cystic Fibrosis in a Long-Term Newborn Screen Population. Pediatric Pulmonol Suppl 31:272, 2008. 159. * Accurso FJ, Rowe SM, Durie PR, Konstan MW, Dunitz J, Hornick DB, Sagel SD, Boyle MP, Uluer AZ, Upadhyay D, Ramsey BW, Freedman SD, Dong Q, Ahmad AM, Stone AJ, Olson ER, Ordonez CL, Clancy JP, Campbell PW, Ashlock MA. Interim Results of Phase 2A Study of VX-770 to Evaluate Safety, Pharmacokinetics, and Biomarkers of CFTR Activity in Cystic Fibrosis Subjects with G551D. Pediatric Pulmonol Suppl 31:295, 2008. 160. * Van Goor F, Hadida S, Negulescu P, Clancy JP, Accurso FJ, Ashlock MA, Ordonez C, Dong Q, Olson E, Grootenhuis P. Relationship of VX-770 Activity on CFTR Function in a Cell Culture System to the Activity Observed in a Clinical Study of VX-770. Pediatric Pulmonol Suppl 31:314, 2008. 161. *Saavedra MR, HughesG, Sanders L, Carr M, Rodman D, Coldren C, Geraci M, Sagel SD, Accurso FJ, West J, Nick JA. Novel Markers For Clinical Trials; Circulating RNA Transcripts Identify Therapeutic Response in Cystic Fibrosis Lung Disease. Pediatric Pulmonol Suppl 31:316, 2008. 162. *Harris JK, Zemanick ET, Wagner BD, Sagel SD, Accurso FJ. Large Scale Multiplex Sequencing For Ribosomal RNA Metagenomics from Cystic Fibrosis Airway Samples. Pediatric Pulmonol Suppl 31:323, 2008. 163. * Milla C, Chmiel J, McCoy KS, Accurso FJ, Billings J, Boyle MP, Atkinson J, Liou T, Clancy JP, Pilewski J, Acton J, Pearce T. A Phase I, II Randomized, Double-Blind, Placebo-Controlled, Single-Dose, Dose Escalation Study of KB001 in Cystic Fibrosis Patients Infected with Pseudomonas aeruginosa. Pediatric Pulmonol Suppl 31:341, 2008. 164. * Laguna TA, Wagner B, Kaess H, Mann S, Accurso FJ. Urinary and Sputum Desmosine Levels During Pulmonary Exacerbation in Cystic Fibrosis. Pediatric Pulmonol Suppl 31:378, 2008. 165. * Laguna TA, Wagner BD, Accurso FJ. The Applicability of Urinary Creatinine as a Method of Specimen Nomalization in the Cystic Fibrosis Population. Pediatric Pulmonol Suppl 31:378, 2008. 166. * Sontag MK, Hughes-Austin J, Accurso FJ. Relationship of CFTR Mutation to Longitunial Clinical Outcome in Cystic Fibrosis. Pediatric Pulmonol Suppl 31:400, 2008. 167. * Nick JA, Chacon C, Brayshaw S, Jones M, Barboa C, Young RL, Janssen JF, Gleeksman C, Huitt GA, Iseman MD, Daley CL, Accurso FJ, Saavedra MT, Sontag MK. Diagnosis, Patterns of Care, and Long-Term Survival in Nonclassic Cystic Fibrosis. Pediatric Pulmonol Suppl 31:402, 2008. 168. * Konstan MW, Strausbaugh SD, Ahrens RC, Accurso FJ, Graff GR, Nasr SZ. A Randomized, Double-Blind, Placebo-Controlled, Multicenter, Cross-Over Study to Evaluate the Effectiveness and Safety of a Novel Pancrelipase (Pacrecarb MS-16) In Reducing Steatorrhea in Children and Adults with Cystic Fibrosis. Pediatric Pulmonol Suppl 31:425, 2008. 169. * Lingard C, Sontag MK, Fordyce J, Wagener JS, Accurso FJ, Sokol R. Children With CF and Meconium Ileus Are At Higher Risk For Vitamin D Deficiency Compared To Those Diagnosed By Newborn Screening. Pediatric Pulmonol Suppl 31:429, 2008. 170. * Milla C, Accurso FJ, Chmiel J, McCoy K, Billings J, Atkinson J, Clancy J, Liou T, Acton J, Lynch S, Burns J, Mayer-Hamblett N, Harris K, Patel R, Tremblay T, Parli T. A Phase I/II Study of the Anti-PcrV Antibody KB001 in Cystic Fibrosis Patients with Pseudomonas aeruginosa, [Publication Page: A1190].

171. * Moss RB, Anbar RD, Wilmott RW, Barnes M, Schaberg AE, Durham TA, Accurso FJ. Phase 3 Study of Denufosol Tetrasodium for the Treatment of Cystic Fibrosis, [Publication Page: A1189]. 172. * Kerby GS, Kopecky C, Wilcox SL, Wagner B, Hay T, Popler J, Accurso FJ, Deterding RR. Infant Pulmonary Function Testing in Children with Neuroendocrine Cell Hyperplasia with and without Lung Biopsy, [Publication Page: A3671]. 173. * Zemanick ET, Harris JK, Wagner BD, Accurso FJ, Sagel SD. Characteristics of Cystic Fibrosis Patients with Pulmonary Exacerbation and No Detectable CF Airway Pathogens, [Publication Page: A1787]. 174. * Accurso FJ, Rowe SM, Durie PR, Konstan MW, Dunitz J, Hornick DB, Sagel SD, Boyle MP, Uluer AZ, Moss RB, Ramsey BW, Freedman SD, Dong Q, Zha J, Stone AJ, Olson ER, Ordonez CL, Clancy JP, Campbell PW, Ashlock MA. Final results of a 14- and 28-day study of VX-770 in subjects with CF authors: Journal of Cystic Fibrosis: Volume 8 Supplement 2: p. S25, June 2009. 175. * Mospan AR, Durham TA, Schaberg AE, Accurso FJ. Effects of denufosol on sinusitis-related complaints in a phase 3 trial in cystic fibrosis patients. Journal of Cystic Fibrosis:8 Supplement 2 p. S26. 176. * Accurso FJ, Durham TA, Schaberg AE. Relationship between pulmonary exacerbations and lung function decline in a six month trial of denufosol. Journal of Cystic Fibrosis:8 Supplement 2 p. S26. 177.* Solomon GM, Konstan MW, Wilschanski M, Billings J, Sermet I, Accurso FJ, Vermeleun F,Young H, Reeves G, Sabbatini G, Mayer-Jamblett N, Ashlock M, Clancy JP, Rowe SM. Universal protocol for nasal potential differences studies: Results of an international multi-center clinical trial. Pediatric Pulmonol Suppl 32:215, 2009. 178.* Boyle M, Clancy JP, Rowe SM, Durie P, Dunitz J, Konstan MW, Hornick D, Sagel SD, Ramsey B, Dong Q, Ordonez C, Campbell P, Ashlock M, Accurso FJ. Effect of VX-770, a CFTR potentiator, on spirometry and QOL assessment in subjects with CF and the G551D-CFTR mutation. Pediatric Pulmonol Suppl 32:217, 2009. 179.* Clancy JP, Rowe SM, Durie P, Freedman S, Dong Q, Ordonez C, Ashlock M, Accurso FJ. NPD evaluation of ion transport in G551D CF patients treated with a CFTR potentiator. Pediatric Pulmonol Suppl 32:222, 2009. 180.* Accurso FJ, Rowe SM, Durie PR, Konstan MW, Dunitz J, Hornick D, Sagel SD, Boyle MP, Uluer AZ, Moss RB, Donaldson S, Pilewski J, Rubenstein RC, Aitken ML, Ramsey B, Freedman SD, Dong Q, Zha J, Stone A, Olson ER, Ordonez C, Campbell P, Ashlock M, Clancy JP. Improvement in sweat chloride concentration by the CFTR potentiator VX-770 in subjects with cystic fibrosis and the G551D-CFTR mutation. Pediatric Pulmonol Suppl 32:240, 2009. 181.* Harris JK, Wagner BD, Zemanick E, Accurso FJ, Sagel SD. Prevalence of anaerobic bacteria in CF respiratory samples from routine clinical practice. Pediatric Pulmonol Suppl 32:290, 2009. 182.* Zemanick E, Harris JK, Wagner BD, Accurso FJ, Sagel SD. Reliability and validity of quantitative real-time PCR microbial detection from CF airway specimens. Pediatric Pulmonol Suppl 32:362, 2009. 183.* Milla C, Chmiel JF, Accurso FJ, McCoy KS, Billings JL, Atkinson JJ, Clancy JP, Liou TG, Acton JD, Lynch SV, Slusher N, Burns JL, Hamblett NM, Harris JK, Patel R, Tremblay TM, Parli TJ. Anti-inflammatory effect of KB001, an anti-PCRV antibody fragment, in CF patients chronically infected with Pseudomonas aeruginosa. Pediatric Pulmonol Suppl 32:369, 2009. 184.* Navratil T, Schaberg A, Mathews D, Deans C, Durham T, Accurso FJ. Pharmacological treatment of CF: Lessons learned from a phase 3 clinical trial. Pediatric Pulmonol Suppl 32:416, 2009. 185. * Durham T, Navratil T, Schaberg A, Deans C, Smiley L, Herje N, Retsch-Bogart G, Accurso FJ. Concomitant medication use in patients with mild cystic fibrosis lung disease assigned to placebo in Phase 3 clnical trial of Denufosol (Study 08-108/TIGER-1). Am J Respir Crit Care Med 181;2010:A1842 186. * LagunaTA, Wagner BD, Accurso FJ. Specific Gravity as a Novel Normalization Method for Urinary Biomarker Measurement in a Cystic Fibrosis Population during Pulmonary Exacerbation. Am J Respir Crit Care Med 181;2010:A1838.

187.* Popler J, Wagner BD, Accurso FJ, Deterding RR. Airway Cytokine Profiles in Children’s Interstitial Lung Diseases. Am J Respir Crit Care Med 181;2010:A3316.

188. * Moss RB, Schaberg A, Deans C, Tian W, Smiley L, Herje N, Durham T, Accurso FJ. Denufosol improves lung function in adolescent patients. J Cyst Fibros 2010; Vol. 9, Supplement 1, page S20.

189. *Treggiari M, Retsch-Bogart G, Mayer-Hamblett N, Khan U, Kronmal R, Ramsey B, Accurso FJ. Comparative efficacy and safety of four randomized regimens to treat earlyPseudomonas aeruginosa infection in children with cystic fibrosis. J Cyst Fibros 2010; Vol. 9, Supplement 1, page S56. 190.* Ratjen F, Saiman L, Mayer-Hamblett N, Lands LC, Kloster M, Emmett P, Goss CH, Rose L, Burns JL, Marshall B, Accurso FJ, Anstead M. The Effect of Azithromcyin on inflammatory markers in CF children and adolescents uninfected with pseudomonas aeruginosa. Pediatric Pulmonol Suppl 33:271, 2010. 191.* Clancy JP, Rowe SM, Accurso FJ, Ballmann M, Boyle MP, DeBoeck C, Konstan MW, Spencer-Green G. A Phase II, Randomized, Placebo-Controlled, Clinical trial of four doses of VX-809 in CF patients homozygous for the F508DEL CFTR mutation. Pediatric Pulmonol Suppl 33:298, 2010. 192.* Accurso FJ, Tian W, Schaberg A, Navratil T, Howenstine M, Liou T, Ratjen F. Denufosol efficacy in patients w ith minimally impaired baseline lung function and on minimal background therapy demonstrates its potential as early intervention for CF lung disease. Pediatric Pulmonol Suppl 33:299, 2010. 193.* Harris JK, Wolfson A, Walker J, Wagner B, Deterding R, Sagel SD, Accurso FJ. High-dimensional biomarker discovery in BAL fluid from pediatric CF patients using somamer technology. Pediatric Pulmonol Suppl 33:300, 2010. 194.* Clancy JP, Rowe SM, Durie PR, Konstan MW, Dunitz J, Hornick DB, Sagel SD, Boyle MP, Uluer AZ, Moss RB, Freedman S, Dong Q, Zha J, Stone A, Olson E, Ordonez C, Campbell P, Ashlock M, Accurso FJ. Comparison of NPD parameters in a Phase IIA study to optimize detection of CFTR modulator bioactivity in clinical trials. Pediatric Pulmonol Suppl 33:301, 2010. 195.* Moss RB, Schaberg A, Tian W, Xue X, Ramsey B, Accurso FJ. Denufosol improved lung function and was well tolerated in adolescents with cystic fibrosis. Pediatric Pulmonol Suppl 33:310, 2010. 196.* Kerem E, Wilschanski M, Melotti P, Sermet-Gaudelus I, DeBoeck K, Rowe SM, Konstan MW, Accurso FJ, Pimentel S, Constantine S, Miller N, Barth J, Miller L, Ajayi T. Phase 3 study of ataluren (PTC124®) in nonsense mutation cystic fibrosis (NMCF): demographic and other baseline data. Pediatric Pulmonol Suppl 33:314, 2010. 197.*Ratjen F, Navratil T, Evans C, Schaberg A, Durham T, Ren C, Moss R, Accurso FJ. Denufosol aerosol properties, pharmacokinetics and efficacy and safety profiles demonstrate its potential as early intervention for cystic fibrosis lung disease. Pediatric Pulmonol Suppl 33:317, 2010. 198.*Rowe SM, van Goor F, Clancy JP, Durie PR, Konstan MW, Dunitz J, Hornick DB, Sagel SD, Boyle MP, Uluer AZ, Moss RB, Ramsey B, Freedman S, Dong Q, Zha J, Stone A, Olson E, Ordonez C, Campbell P, Ashlock M, Accurso FJ. Pediatric Pulmonol Suppl 33:319, 2010. 199.*Zemanick ET, Wagner B, Accurso FJ, Sagel SD, Harris JK. Oropharyngeal bacterial microbiome in young children with cystic fibrosis and healthy controls. Pediatric Pulmonol Suppl 33:324, 2010. 200.*Federico MJ, Miller MR, Accurso FJ, Sontag MK. Hispanic children with cystic fibrosis are at higher risk for early first positive pseudomonas aeruginosa culture than non-hispanic whites. Pediatric Pulmonol Suppl 33:359, 2010. 201.*Sontag MK, Sagel SD, Wagener J, Accurso FJ. Missed cases in the Colorado newborn screening: A systematic review of 25 years of IRT/IRT screening. Pediatric Pulmonol Suppl 33:396, 2010. 202.*Zemanick ET, Towler E, Accurso FJ, DeVoogd R, Wagener J, Sagel SD. Success in eradicating pseudomonas aeruginosa at a large CF center using a quality improvement initative. Pediatric Pulmonol Suppl 33:409, 2010. 203.*Rowe SM, Clancy JP, Boyle M, Van Goor F, Ordonez C, Dong Q, Campbell P, Ashlock M, Accurso FJ. Parallel effects of VX-770 on transepithelial potential difference in vitro and in vivo. J Cyst Fibros 2010; Vol. 9, Supplement 1:S20(74). 204.*Moss RB, Schaberg A, Deans C, Tian W, Smiley L, Herje N, Durham T, Accurso FJ. Denufosol improves lung function in adolescent CF patients. J Cyst Fibros 2010; Vol. 9, Supplement 1:S20(76).

205.*Accurso FJ, Tian W, Schaberg A, Navratil T, Howenstine MS, Liou TG. Potential of denufosol as an early intervention in CF lung disease: efficacy in patients with minimal pharmacotherapy in a US phase 3 clinical trial. J Cyst Fibros 2010; Vol. 9, Supplement 1:S21(77). 206.*Navratil T, Evans C, Schaberg A, Johnson F, Durham T, Ren CL, Ratjen F, Moss RB, Accurso FJ. Aerosol and pharmacokinetic properties of denufosol support its use for early intervention in CF lung disease. J Cyst Fibros 2010; Vol. 9, Supplement 1:S21(78). 207.*Treggiari M, Retsch-Bogart G, Mayer-Hamblett N, Khan U, Kronmal R, Ramsey B, Accurso FJ. Comparative efficacy and safety of four randomized regimens to treat earlyPseudomonas aeruginosa infection in children with cystic fibrosis. J Cyst Fibros 2010; Vol. 9, Supplement 1:S54(209). 208.*Accurso FJ, Durham T, Navratil T, Deans C, Herje N, Smiley L, Schaberg A. Analysis of polypharmacy in patients with mild CF lung disease assigned to placebo in phase 3 clinical trail of denufosol. J Cyst Fibros 2010; Vol. 9, Supplement 1:S56(215). 209.*Durham T, Navratil T, Schaberg A, Deans C, Smiley L, Herje N, Retsch-Bogart GZ, Accurso FJ. Concomitant Medication Use In Patietns With Mild Cystic Fibrosis Lung Disease Assigned To Placebo In Phase 3Clinical Trial Of Denufosol (Study 08-108/TIGER-1). American Thoracic Society, New Orleans, LA: May, 2010. 210.*Navratil T, Evans C, Schaberg A, Johnson F, Durham T, Smiley L, Herje N, Burke B, Ren CL, Ratjen F, Moss RB, Accurso FJ. Denufosol Tetrasodium – An Investigational Inhaled Therapy With Aersol And Pharacokinetic Properties Suitable For Early Intervention In CF Lung Disease. American Thoracic Society, New Orleans, LA: May, 2010. 211.*Deterding RR, Wolfson A, Harris JK, Walker JJ, Accurso FJ. Aptamer Proteomic Analysis Of Bronchoalveolar Lavage Fluid Yields Different Protein Signatures From Children With Children’s Interstitial Lung Disease, Cystic Fibrosis And Disease Controls. American Thoracic Society, New Orleans, LA: May, 2010.

E. Invited Presentations and Visiting Professorships (Regional, National or International) 1. Early abnormalities in infants with cystic fibrosis diagnosed through newborn screening. Presented at the Conference on Clinical Implications of Screening for Cystic Fibrosis, Peterborough, UK, July 1986. 2. Early abnormalities in infants with cystic fibrosis diagnosed through newborn screening. Presented at the North American Cystic Fibrosis Conference, Toronto, Canada, October 1987. 3. Newborn screening for cystic fibrosis - Pro. Presented at the North American Cystic Fibrosis Conference, Orlando, Florida, October 1988. 4. Early respiratory abnormalities in infants with CF diagnosed through newborn screening. Presented at Conference Internationale Mucoviscidose: Depistage Neonatal et Prise en Charge Precoce, Caen, France, October 1988. 5. Newborn screening for cystic fibrosis. Presented at the American Lung Association Science Writers Forum, Annapolis, Maryland, October 1989. 6. Pulmonary vasoreactivity and growth in the developing pulmonary circulation. Poster symposium chaired at the Society for Pediatric Research Meeting, Washington, DC, May 1990. 7. Viruses and infants with cystic fibrosis. Presented at the North American Cystic Fibrosis Conference, Arlington, Virginia, October 1990. 8. Early respiratory abnormalities in screened infants. Presented at the Fourth International Conference on Newborn Screening for Cystic Fibrosis, Estes Park, Colorado, September 1990. 9. Cystic Fibrosis. Presented at the Third International Conference on Pulmonary Rehabilitation, Denver, Colorado, March 1991.

10. Approaches to treatment of cystic fibrosis. Presented at the International Conference on Cystic Fibrosis, Lipari, Italy, October 1991. 11. Newborn screening for cystic fibrosis. Presented during visiting professorship. University of Messina, Messina, Italy, October 1991. 12. Early course in cystic fibrosis. Presented during visiting professorship. University of Pennsylvania, Philadelphia, Pennsylvania, March 1992. 13. Infant pulmonary function testing. Presented at the Consensus Conference: Clinical Outcomes for Evaluation of New CF Therapies, Rockville, Maryland, December 1992. 14. Pediatric clinical fellows session. Clinical educational conference chaired at the North American Cystic Fibrosis Conference, Dallas, Texas, October 1993. 15. “Improving Survival in Cystic Fibrosis.” Presented at the American Academy of Pediatrics Meeting, Denver, Colorado, April 1994. 16. The Adolescent with Cystic Fibrosis. Presented at the American Academy of Pediatrics Meeting, Denver, Colorado, April 1994. 17. Respiratory disease in children with Cystic Fibrosis. Presented during visiting professorship. Clinca Pediatrica Istituto per Infanzia, Trieste, Italy, June 1994. 18. Assessment of lung disease in infants with Cystic Fibrosis. Presented at the North American Cystic Fibrosis Conference, Orlando, Florida, October 1994. 19. Infection and Inflammation in Infants with CF: Evaluation by Bronchoalveolar lavage. Roundtable chaired at the North American Cystic Fibrosis Conference, Orlando, Florida, October 1994. 20. Inflammatory disease in the younger patient with Cystic Fibrosis. Presented at the Epidemiologic Study of Cystic Fibrosis Meeting, Orlando, Florida, October 1994. 21. Why treat the mild patient with cystic fibrosis? Presented at the Genentech Investigators Meeting on Comparison of Nebulization systems, San Francisco, California, February 1995. 22. Early lung disease in Cystic Fibrosis. Presented at the Eleventh Annual Meeting for CF Centers, Austin, Texas, April 1995. 23. Airway Inflammation in Cystic Fibrosis. Symposium Chaired at the American Thoracic Society Meeting, Seattle, Washington, May 1995. 24. Lung Disease in Infants. Presented at the Cystic Fibrosis Foundation Williamsburg Conference, Williamsburg, Virginia, June 1995. 25. Start of Lung Disease in Cystic Fibrosis: Therapeutic Implications. Presented at the European Cystic Fibrosis Conference, Brussels, Belgium, June 1995. 26. Assessment of Lung Disease in Infants with Cystic Fibrosis. Presented to the Fifth Annual Meeting of the Cystic Fibrosis Research Institute, Palo Alto, California, July 1995. 27. Does Inflammation Precede Infection in Early Airways Disease? Presented at the North American Cystic Fibrosis Conference, Dallas, Texas, October 1995. 28. Early Markers of Inflammation. Roundtable chaired at the North American Cystic Fibrosis Conference, Dallas, Texas, October 1995. 29. Treatment of mild, moderate, and severe lung disease in CF. Cystic Fibrosis: Advances in Treatment of the Pulmonary Manifestations, Sao Palo, Brazil, October 1995. 30. Neonatal screening for cystic fibrosis. Presented during visiting professorship. University of Texas at San Antonio, San Antonio, Texas, November 1995. 31. Pathogenesis of lung disease in Cystic Fibrosis. Genentech Investigator meeting. San Francisco, California, March 1996.

32. Early lung disease in Cystic Fibrosis. Presented during visiting professorship. University of Wisconsin, Madison, Wisconsin, May 1996. 33. Early manifestations of cystic fibrosis. Presented at meeting of CF Families. University of California, San Diego, California, April 1996. 34. The Rationale for Early Interventions in Cystic Fibrosis. Symposium chaired at the XIIth International Cystic Fibrosis Congress, Jerusalem, Israel, July 1996. 35. Neonatal Screening for Cystic Fibrosis. Presented during visiting professorship, University of Kentucky, Louisville, Kentucky, August 1996. 36. Insights in Airway Injury from Studies of Cystic Fibrosis. Presented at NHLBI: Division of Lung Diseases, Effects of Growth and Development in Lung Function: Models for study of childhood asthma, Rockville, Maryland, September 1996. 37. Pathogenesis of CF Airway Disease: Early Disease - Studies in Infants. Presented at the Conference on Antimicrobial Peptides and CF Airway Infections, Chicago, Illinois, October 1996. 38. Infection and Inflammation at Mucosal Surfaces. Symposium co-chaired at the 10th Annual Cystic Fibrosis Foundation North American Conference, Orlando, Florida, October 1996. 39. Markers of Early Inflammation. Roundtable discussion chaired at the 10th Annual Cystic Fibrosis Foundation North American Conference, Orlando, Florida, October 1996. 40. Clinical course of infants with cystic fibrosis: Opportunities for early intervention. Presented at Newborn Screening for Cystic Fibrosis: A paradigm for public health genetics policy development. Centers for Disease Control and Prevention, Atlanta, Georgia, January 1997. 41. Inflammatory Response as Surrogate Marker for Outcomes in Young Children. Presented to the joint Food and Drug Administration/Cystic Fibrosis Foundation Workshop on Surrogate Markers for Cystic Fibrosis, Bethesda, Maryland, April 1997. 42. Bronchoalveolar lavage in Infants with Cystic Fibrosis. Presented at the Cystic Fibrosis Foundation Williamsburg Conference. Williamsburg, Virginia, June 1997. 43. Early Lung Disease in Cystic Fibrosis. Presented during visiting professorship at the University of Minnesota, Minneapolis, Minnesota, August 1997. 44. Lung Defense 101. Course Co-chaired at the Cystic Fibrosis North American Conference, Nashville, Tennessee, October 1997. 45. Newborn Screening for Cystic Fibrosis: Need, Benefit, Risk. Symposium chaired at the Cystic Fibrosis North American Conference, Nashville, Tennessee, October 1997. 46. Newborn Screening for Cystic Fibrosis: The Need from a Center Director’s Viewpoint. Presented at the Cystic Fibrosis North American Conference, Nashville, Tennessee, October 1997. 47. Treatment of airway inflammation and hyperreactivity in cystic fibrosis. Presented at Medicaments de la mucoviscidose chez l’enfant, Paris, France, November 1997. 48. Inhaled Corticosteroid Treatment in Young Children with Cystic Fibrosis. Presented at Case Western Reserve University, Cleveland, Ohio, March 1998. 49. Early inflammatory response and anti-inflammatory therapy with aerosolized steroids. Presented at the Canadian Cystic Fibrosis Foundation Tenth Broken Arrow Conference, Ontario, Canada, March 1998. 50. Surveillance in cystic fibrosis: early detection of pulmonary involvement. Presented at The Fourth Annual Child Health Research Symposium, Calgary, Canada, April 1998. 51. Cystic Fibrosis Screening programs should be instituted. Debate chaired at The Fourth Annual Child Health Research Symposium, Calgary, Canada, April 1998. 52. Newborn screening for Cystic Fibrosis. Presented at University of New Mexico, Albuquerque, New Mexico, May 1998. 37 2/15/11 FJA Curriculm Vitae – Frank J. Accurso, M.D.

53. Early airway inflammation in cystic fibrosis. Plenary session presented at the 22nd European Cystic Fibrosis Conference, Berlin, Germany, June 1998. 54. Anti-inflammatory Drugs. Symposium co-chaired at the 22nd Annual European Cystic Fibrosis Conference, Berlin, Germany, June 1998. 55. Infection and Inflammation in infants. Presented at the Cystic Fibrosis Foundation Williamsburg Conference. Williamsburg, Virginia, June 1998. 56. Modifying Inflammation and Early Infection: Inhaled Steroids. Presented at the Cystic Fibrosis Foundation Williamsburg Conference. Williamsburg, Virginia, June 1998. 57. Strategies for effective early clinical intervention. Presented at the Fifth International Conference on Neonatal Screening for Cystic Fibrosis, Caen, France, September 1998. 58. Can Systemic or Local Indices of Inflammations Serve as Surrogate Outcome Measures for Airway Disease in Children? Presented at the NICHD Conference on Surrogate Outcome Measures in Pediatrics. Bethesda, Maryland, September 1998. 59. Lung Defense 201. Course chaired at the Cystic Fibrosis North American Conference, Montreal, Canada, October 1998. 60. The Role of Early Diagnosis and Treatment in the Development of the CF Phenotype. Presented at the Cystic Fibrosis North American Conference, Montreal, October 1998. 61. Newborn Screening for Cystic Fibrosis. Plenary Address presented to the Cystic Fibrosis Foundation North American Conference, Montreal, Canada, October 1998. 62. Cystic Fibrosis. Presented to the University of Texas at San Antonio during visiting professorship, January 1999. 63. Early Inflammation in Cystic Fibrosis. Presented to the Missouri Cystic Fibrosis Consortium, Kansas City, Missouri, March 1999. 64. Newborn Screening for Cystic Fibrosis. Presented to the pediatric update course at the University of Louisiana at Shreveport, May 1999. 65. Early Diagnosis and Airway Inflammation in Cystic Fibrosis. Presented to the Australian Cystic Fibrosis Meeting, Sydney, Australia, August 1999. 66. Issues in the Evaluation and Treatment of Infants. Workshop chaired at the Cystic Fibrosis Foundation North American Meeting, Seattle, Washington, October 1999. 67. Lung Defense 301. Course chaired at the Cystic Fibrosis North American Conference, Seattle, Washington, October 1999. 68. Newborn Screening for Cystic Fibrosis. Visiting Professorship at the University of Indiana, Indianapolis, Indiana, December 1999. 69. Clinical Research in Cystic Fibrosis. Presented at the American College of Chest Physician Review Course in Pediatric Pulmonology, San Francisco, California, January 2000. 70. Overview of Inflammation in Cystic Fibrosis. Presented to the Cystic Fibrosis Foundation workshop on Airway Inflammation in Cystic Fibrosis. Virginia, February 2000. 71. Update on Cystic Fibrosis Research. Presented to the Atlanta Cystic Fibrosis Foundation Family Night, Atlanta, Georgia, March 2000. 72. Therapy for Airway Inflammation in Cystic Fibrosis. Workshop chaired at the American Thoracic Society Meeting in Toronto, Canada, May 2000. 73. Eicosanoids in Cystic Fibrosis Lung Disease. Workshop chaired at the Williamsburg Conference of the Cystic Fibrosis Foundation, Williamsburg, Virginia, May 2000.

74. Lung Disease in Cystic Fibrosis. Aspen Allergy Course sponsored by National Jewish Center for Medicine and Research, Aspen, Colorado, July 2000. 75. Newborn Screening for Cystic Fibrosis, Presented to the University of Washington Cystic Fibrosis Center annual meeting, Seattle, Washington, July 2000. 76. Newborn Screening for Cystic Fibrosis and Collaborative Study of Tobi in Infants. Presented to the Ohio Cystic Fibrosis Consortium, Columbus, Ohio, August 2000 77. Cystic Fibrosis Clinical Trial Review. Presented to the Amereican College of Chest Physicians Fellows Conference, San Francisco, California, October 2000. 78. Solving the Puzzle: CF Clinical Research 2000. Plenary session address at the Fourteenth Annual North American Cystic Fibrosis Meeting, Baltimore, Maryland, November 2000. 79. Clinical Research in Cystic Fibrosis. Presented to the NIH GCRC Core Laboratory Directors at the GCRC National Meeting, Crystal City, Virginia, March 2001. 80. Clinical Sampling and Outcome Measures in Cystic Fibrosis: How Can Proteomics Help? Presented to the Cystic Fibrosis Foundation Meeting on Proteomics, Bethesda, Maryland, March 2001. 81. Newborn Screening for Cystic Fibrosis and Clinical Research in Cystic Fibrosis. Presented to the Spanish Cystic Fibrosis Center Directors Conference, Madrid, Spain, May 2001. 82. Current Perspectives in Cystic Fibrosis Clinical Research. Presented to the Italian Cystic Fibrosis Center Directors Conference “What’s Cooking in Cystic Fibrosis?” Portofino, Italy, May 2001. 83. Genetic Modifiers of Early Pancreatic Disease in Cystic Fibrosis. Presented to the Williamsburg Cystic Fibrosis Foundation Conference, June 2001. 84. Opportunities for Early Treatment of Cystic Fibrosis with Docosahexanoic Acid. Presented to the Cystic Fibrosis Foundation Workshop on Fatty Acids in CF, Bethesda, Maryland, July 2001. 85. Newborn Screening for Cystic Fibrosis: The Colorado Experience. Presented to the State of California Public Health Department Conference on Newborn Screening for Cystic Fibrosis, San Jose, California, August, 2001. 86. Clinical Care in Cystic Fibrosis. Presented to the Cystic Fibrosis Research Institute, San Jose, California, August 2001. 87. Early Events in Airways Disease. Workshop chaired at the Fifteenth Annual North American Cystic Fibrosis Meeting, Orlando, Florida, October 2001. 88. Update on Cystic Fibrosis. Presented at the University of Utah CF Clinic 2002 family meeting, Salt Lake City, Utah, April 2002. 89. Airway Inflammation in Cystic Fibrosis: Opportunities for Treatment. Symposium chaired at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, June 2002. 90. Epithelial Gene Expression in Individuals with CF. Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, June 2002. 91. Early Diagnosis and Treatment of Cystic Fibrosis. Grand Rounds during Visiting Professorship, University of Alabama, Birmingham, Alabama, July 2002. 92. How We Do It: Care of the Newly Diagnosed Patient with Cystic Fibrosis. Symposium chaired at the Sixteenth Annual North American meeting of the Cystic Fibrosis Foundation in New Orleans, Louisiana, October 2002. 93. Care of the Newly Diagnosed Infant with Cystic Fibrosis Identified through Newborn Screening. Presented at the Sixteenth Annual North American meeting of the Cystic Fibrosis Foundation in New Orleans, Louisiana, October 2002. 94. Microarray Analysis of Clinical Specimens. Presented at the Sixteenth Annual North American meeting of the Cystic Fibrosis Foundation in New Orleans, Louisiana, October 2002.

95. Assessment of Airway Inflammation in Cystic Fibrosis. Presented at the Annual meeting of the Cystic Fibrosis Foundation Therapeutics Development Network, Seattle, Washington, April 2003. 96. Newborn screening for cystic fibrosis is needed to treat early pancreatic insufficiency. Presented at the National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Center for Disease Control, Atlanta, Georgia, April 2003. 97. The Promise of Proteomics in Cystic Fibrosis. Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, June 2003. 98. Epithelial Inflammation in Early Cystic Fibrosis. Presented at the European Society for Cystic Fibrosis meeting, Belfast, Northern Ireland, June 2003. 99. Antiinflammatory Outcome Measures: Lessons from Clinical Trials. Presented at the Seventeenth Annual North American meeting of the Cystic Fibrosis Foundation, Anaheim, California, October 2003. 100. Zinc in Cystic Fibrosis. Presented at the Cystic Fibrosis Foundation Antioxidant Workshop, Baltimore, Maryland, November 2003. 101. Epidemiology and Natural History of Cystic Fibrosis. Presented at the conference “Newborn Screening for Cystic Fibrosis, 2003,” National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia, November 2003. 102. Role of CF Model Systems and Human Trials in Investigational Decisions. Presented at the Annual meeting of the Cystic Fibrosis Foundation Therapeutics Development Network, Seattle, Washington, April 2004. 103. Microbial Diversity in Cystic Fibrosis. Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, June 2004. 104. Drug Resistant Organisms in Cystic Fibrosis. Presented at the Sixth Annual Mountain States Cystic Fibrosis Consortium. Boulder, Colorado, June 2004. 105. Cystic Fibrosis Foundation Workshop on Implementation of Newborn Screening for Cystic Fibrosis. Workshop Cochaired, Arlington, Virginia, August 2004. 106. Clinical Characteristics of Infants with Cystic Fibrosis. Presented at the Cystic Fibrosis Foundation Workshop on Implementation of Newborn Screening for Cystic Fibrosis. Arlington, Virginia, August 2004. 107. Current Aproaches to Care of Infants with Cystic Fibrosis: Colorado Protocols. Presented at the Cystic Fibrosis Foundation Workshop on Implementation of Newborn Screening for Cystic Fibrosis. Arlington, Virginia, August 2004. 108. Newborn Screening for Cystic Fibrosis. Symposium co-chaired at the Eighteenth Annual North American meeting of the Cystic Fibrosis Foundation, St. Louis, Missouri, October 2004. 109. Medical Consequences of Delayed Diagnosis. Presented at the Eighteenth Annual North American meeting of the Cystic Fibrosis Foundation, St. Louis, Missouri, October 2004. 110. Elements of a Pediatric Cystic Fibrosis Center. Presented at the Eighteenth Annual North American meeting of the Cystic Fibrosis Foundation, St. Louis, Missouri, October 2004. 111. Update on Cystic Fibrosis. Presented at the Regional Meeting of the American Academy of Pediatrics, Denver, Colorado, May 2005. 112. Mechanisms of Childhood Lung Disease. Session chaired at the Pediatric Academic Societies meeting, Washington DC, May 2005. 113. Inflammation and Proteomics in CF. Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, June 2005. 114. Genetic Modifiers in CF. Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, June 2005.

115. Cystic Fibrosis in the 21st Century. Northwestern Pediatric Society Lecture Honoring Warren J. Warwick at University of Minnesota Medical School Course “Topics and Advances in Pediatrics.” Minneapolis, Minnesota, June 2005. 116. Care of Infants with CF Identified Through Newborn Screening. Presented at the nineteenth annual North American Cystic Fibrosis Conference, Baltimore, Maryland, October 2005. 117. Assessment of Airway Inflammation in CF. Roundtable Chaired at the nineteenth annual North American Cystic Fibrosis Conference, Baltimore, Maryland, October 2005. 118. Current Biomarker Knowledge and Clinical Practice in Cystic Fibrosis. Keynote address to the Proteomics and CF Biomarkers Workshop, sponsored by the Cystic Fibrosis Foundation, Bethesda, Maryland, November 2005. 119. Newborn Screening for Cystic Fibrosis. Presented to the New York City Greater Area Center Directors, New York City, NY, March 2006. 120. Newborn Screening for Cystic Fibrosis. Presented to the Northern New England CF consortium, Portland, Maine, March 2006. 121. Newborn Screening for Cystic Fibrosis. Pediatric Grand rounds given at the University of Arizona, Tucson, Arizona, April 2006. 122. Sweat Testing as an Outcome Measure in CF. Presented at the National Meeting of the Cystic Fibrosis Foundation Therapeutics Development Network, Seattle, Washington. April 2006. 123. Inflammation vs. Infection in CF. Presented at the Joint US-Ireland Workshop: Inflammation and Infection in Cystic Fibrosis and Therapeutic Implications. Dublin, Republic of Ireland, June 2006. 124. Opportunities for research in newborn screening for CF. Presented at the Joint US-Ireland Workshop: Inflammation and Infection in Cystic Fibrosis and Therapeutic Implications. Dublin, Republic of Ireland, June 2006. 125. Clinical Trials Networks in CF. Presented at the 6th Symposium: Research projects funded by the Mukoviszidose e.V. Dusseldorf, Germany, September 2006.

126. Discovery and Clinical Proteomics in Airway Inflammation in CF. Workshop co-chaired at the twentieth annual North American Cystic Fibrosis Conference, Denver, Colorado, November 2006. 127. Proteomic biomarkers of lower airway disease. Symposium talk Presented at the twentieth annual North American Cystic Fibrosis Conference, Denver, Colorado, November 2006. 128. Protein Panels for Lung disease, the Call for Validation. Presented at the American Heart Association Scientific Sessions 2006 in the symposium “Cardiovascular Proteomics: Ready for the Clinic?” Chicago, Illinois, November 2006. 129. Clinical Trial Update. Presented to the Cystic Fibrosis Foundation Therapeutics Development Network Meeting. Seattle, Washington. April 2007. 130. Characterization of Infants Diagnosed with Cystic Fibrosis Through Newborn Screening: the Spectrum of Disease. Presented at the Conference "Diagnostic Criteria for Cystic Fibrosis in the New Era of Early Recognition through Newborn Screening." Bethesda, Maryland, May, 2007. 131. Biomarkers in CF Research and Newborn Screening for CF. Talks presented during Visiting Professorship, University of Pennsylvania, Children's Hospital of Philadelphia. Philadelphia, Pennsylvania, May 2007. 132. Clinical Proteomics in Pediatric Lung Disease. Presented in session "Mining the proteome for biomarkers in lung and heart disease: Update from the NHLBI Clinical Proteomics Programs.” Session co-chaired at the American Thoracic Society meeting, San Francisco, California, May 2007. 133. Clinical Evidence for Excess/Early/Independent Inflammation in CF. Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation. Williamsburg, Virginia, June 2007.

134. CFTR Dysfunction. Session Chaired at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation. Williamsburg, Virginia, June 2007. 135. Assessment of Airway Inflammation in Children. Presented to the International Society of Aerosol Medicine. Tours, France, June 2007. 136. Care of Infants with Cystic Fibrosis Identified Through Newborn Screening. Cystic Fibrosis Foundation workshop co-chaired. Bethesda, Maryland, July 2007. 137. Cystic Fibrosis. Pediatric Grand Rounds. Memorial Children's Hospital, Colorado Springs, Colorado. August 2007. 138. Outcome Measures to Assess CF Interventions. Symposium co-chaired at the twenty first annual North American Cystic Fibrosis Conference, Anaheim, California, October 2007. 139. Biomarkers of Pulmonary Hypertension. Presented at the Biomarkers of Cardiovascular Risk: State of the Art conference. Mayo Laboratories, Rochester, Minnesota, November 2007 140. Discovery to Validation, the Long Path to Clinical Utility: In search of Protein Panels for Lung Disease. Presented to the American Heart Association Scientific Session 2006 in the Symposium "Bringing Proteomics to the Clinic: From Discovery to Validation" Orlando, Florida, November 2007. 141. Infection in Cystic Fibrosis (CF): A Clinician’s View. Presented at the Symposium "Mixed Infections in CF. Orlando Florida, November, 2007. 142. Update on Sweat Eletrolytes. Presented to the annual Therapeutics Development Network meeting of the Cystic Fibrosis Foundation, Seattle, Washington, April 2008. 143. Asthma and Cystic Fibrosis. Platform Session chaired at the Pediatric Academic Societies Meeting, Honululu, Hawaii, May 2008. 144. Clinical proteomics and Clinical Trials for Lung Disease in Childhood. Presented at the session "Proteomic Approaches to Lung and Heart Disease: Update from the NHLBI Clinical Proteomics Programs, American Thoracic Society Meeting, Toronto, Canada, May 2008. 145. Can Sweat Chloride Provide a Useful In Vivo Measure of CFTR Correction? Presented at the Williamsburg Scientific Meeting of the Cystic Fibrosis Foundation, Williamsburg, Virginia, 2008. 146. Inflammation. Presented at the 31st European Cystic Fibrosi Conference, Plenary Session entitled: Translating Laboratory Discoveries to Patient Benefit: What do we still need to know to stop CF lung damage. Prague, Czech Republic, June 2008. 147. Interim results of Phase 2a Study of VX-770 to Evaluate Safety, Pharmacokinetics, and Biomarkers of CFTR activityin Cystic Fibrosis Subjects with G551D. Presented at the 31st European Cystic Fibrosis Conference, Late Breaking Science Session, Prague, Czech Republic, June 2008. 148. The TIGER-1 Phase 3 Clinical Trial of Denufosol in Cystic Fibrosis. Presented at the 22nd Annual North American Cystic Fibrosis Conference, Orlando, Florida, October 2008. 149. Panel Member, Richard Talamo, MD, Memorial Lecuture, Massachusetts General Hospital, Boston, Massachusettes, November 2008. 150. CFTR outcomes: Sweat Testing. Presented to the annual Cystic Fibrosis Foundation Therapeutics Development Network Meeting. Denver, Colorado, March 2009. 151. Newborn Screening Experience. Presented to the Mountain West Cystic Fibrosis Consortiu, Santa Fe, New Mexico, April 2009. 152. Activation of Mutant Ion Channels; Sweating the Small Molecule. Presented at the American Thoracic Society Conference Scientific Symposium: Correcting the Basic Defect of Cystic Fibrosis: From Molecular Target to Therapeutic Testing. San Diego, California, May 2009. 153. Newborn Screening Provides a Clnical Advantage in Cystic Fibrosis: Pro. Presented at the American Thoracic society Symposium: Pro-Con Debate in Pediatric Pulmonology. San Diego, California, May 2009.

154. It’s Not All Mucus Clearance: Innate and Adaptive Host Defenses in CF. Symposium co-chaired at the 23rd Annual North American Cystic Fibrosis Conference, Minneapolis, Minnesota, October 2009. 155. Clinical and Laboratory Correlates of Imparied Host Defense in CF. Presented as part of the Symposium entitled “It’s Not All Mucus Clearance: Innate and Adaptive Host Defenses in CF” at the 23rd Annual North American Cystic Fibrosis Conference, Minneapolis, Minnesota, October 2009. 156. Offense and Defense in the CF Airway: Workshop co-chaired at the 23rd Annual North American Cystic Fibrosis Conference, Minneapolis, Minnesota, October 2009. 157. “Outcome in Cystic Fibrosis”. Richard C. Talamo Memorial Lecture, Massachusetts General Hospital, Boston, Massachusetts, November 2009. 158. “Cystic Fibrosis: Clinical Pediatric Studies” Facilitator for Thematic Poster Session, American Thoracic Society Conference, New Orleans, Louisiana, June 2010. 159. “Management of Infants Diagnosed Through Newborn Screening: a Brief Review of the US and European Guidelines.” Invited talk to the conference Progress and Challenges in Cystic Fibrosis Newborn Screening: Perspectives from the USA, Europe and Spain. Sponsored by Centro Superior de Investigacion en Salud Publica, Valencia, Spain, June 2010. 160.

43 2/15/11 FJA