Peadar Noone

Peadar Noone


MB, Bch BAO:
(1983) U. College Galway Medical School, Ireland

MD [Doctorate Degree]:
(1997) U. College Galway Medical School, Ireland

(1983-89) Univ. Hospital in Galway & Dublin, Ireland Tufts University, Boston, MA
University of North Carolina at Chapel Hill, Chapel Hill, NC

Academic & Professional Titles:

Professor of Medicine
Fax: 919-966-7013

Clinical Interests:

  1. Lung Transplant Medicine
  2. General internal medicine & General Pulmonary Medicine
  3. Lung diseases associated with genetic abnormalities – cystic fibrosis, primary ciliary dyskinesia, bronchiectasis.
  4. Infectious diseases of the lung – tuberculosis and non-tuberculous infections

Research Interests:

The correlations between genetic disease and disease expression at a cell biologic and organ level, particularly as pertains to airways disease and other related diseases.

  1. Airway host defence, disorders of ciliary structure/function.
  2. Drug delivery to the lung.
  3. Gene therapy, and other novel therapies for CF.
  4. Genetics of airways diseases.

Recent Publications:

1. JL Lobo, MA Zariwala and PG Noone.  Primary Ciliary Dyskinesia.  Quarterly Journal of Medicine, London UK.  (12 pages, 25 references).  QJM. 2014 Mar 19. [Epub ahead of print] PMID: 24652656 

2.  Michael R Knowles, Lawrence E Ostrowski, Margaret W Leigh, Patrick R Sears, Stephanie D Davis, Whitney E Wolf, Milan J Hazucha, Johnny L Carson, Kenneth N Olivier, Scott D Sagel, Margaret Rosenfeld, Thomas W Ferkol, Sharon D Dell, Carlos E Milla, Scott H Randell, Weining Yin, Aruna Sannuti, Hilda M Metjian, Peadar G Noone, Peter J Noone, Christina A Olson, Michael V Patrone, Hong Dang, Hye-Seung Lee, Toby W Hurd, Heon Yung Gee, Edgar A Otto,  Jan Halbritter, Stefan Kohl, Martin Kircher, Jeffrey Krischer, Michael J Bamshad, Deborah A Nickerson,16 Friedhelm Hildebrandt,Jay Shendure,  Maimoona A Zariwala.   Mutations in RSPH1 Cause Primary Ciliary Dyskinesia with a Unique Clinical and Ciliary Phenotype. (47 pages, 59 refs).   Am J Respir Crit Care Med. 2014 Mar 15;189(6):707-17. doi: 10.1164/rccm.201311-2047OC.PMID:24568568  [PubMed - in process]

 3. Austin-Tse C, Halbritter J, Zariwala MA, Gilberti RM, Gee HY, Hellman N, Pathak N, Liu Y, Panizzi JR, Patel-King RS, Tritschler D, Bower R, O'Toole E, Porath JD, Hurd TW, Chaki M, Diaz KA, Kohl S, Lovric S, Hwang DY, Braun DA, Schueler M, Airik R, Otto EA, Leigh MW, Noone PG, et al.  Screen Plus Human Mutational Analysis Identifies C21orf59 and CCDC65 Defects as Causing Primary Ciliary Dyskinesia. Am J Hum Genet. 2013 Oct 3;93(4):672-86.

4. Leonard J Lobo and Peadar G Noone.  Infectious Considerations for Patients with Cystic Fibrosis Undergoing Lung Transplantation.  The Lancet Respiratory Medicine 2013 doi:10.1016/S0140-6736(08)61345-8 (10 print pages;  note this paper was featured on the cover with specially commissioned cover art)

5. LJ Lobo, LC Chang, CR Esther Jr., PH Gilligan, Z Tulu, and PG Noone (Sr Author). Lung Transplant Outcomes in Cystic Fibrosis Patients with Pre-Operative Mycobacterium abscessus Respiratory Infections.   Clin Transplantation 2013: 27 (4); 523-9.    DOI: 10.1111/ctr.12140. 

6. D Antony, A Becker-Heck, MA Zariwala, M Schmidts, A Onoufriadis, M Forouhan, R Wilson, T Cox, A Dewar, C Jackson, P Goggin, NT Loges, H Olbrich, M Jaspers, M Jorissen, MW Leigh, WE Wolf, MLA Daniels,  PG Noone, TW Ferkol, SD Sagel, M Rosenfeld, A Rutman, A Dixit, C O’Callaghan, JS Lucas, C Hogg, PJ Scambler, RD Emes, EMK Chung A Shoemark MR Knowles H Omran HM Mitchison.  Mutations in CCDC39 and CCDC40 are the Major Cause of Primary Ciliary Dyskinesia with Axonemal Disorganization and Absent Inner Dynein Arms.  Human Mutations.  2013 Mar;34(3):462-472.

7. Burrows E, Southern K, Noone PG.  Sodium channel blockers for cystic fibrosis.  Cochrane Database Syst Rev. 2012 Mar 14;3:CD005087. 

 8. Esther CR Jr, Esserman DA, Gilligan P, Kerr A, Noone PG. Chronic Mycobacterium abscessus infection and lung function decline in cystic fibrosis.  J Cyst Fibros. 2010 Mar; 9(2):117-23.

 9. Neuringer IP, Noone P, Cicale RK, Davis K, Aris RM. Managing complications following lung transplantation.  Expert Rev Respir Med. 2009 Aug;3(4):403-23. doi: 10.1586/ers.09.27.

 10.  Amin R, Noone PG, Ratjen F.Cochrane Database Syst Rev. Chemical pleurodesis versus surgical intervention for persistent and recurrent pneumothoraces in cystic fibrosis.  Cochrane Database Syst Rev. 2012 Dec 12;12: CD007481. doi: 10.1002/14651858.CD007481.pub3. Review. PMID: 23235645  

 11. Kennedy MP, Omran H, Leigh MW, Dell S, Morgan L, Zariwala MA, Molina PL, Minnix SL, Severin T, Ahrens P, Lange L, Noone PG, Knowles MR.  Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia.  Circulation. 2007 Jun 5;115(22):2814-21.

 12. Kennedy MP,Noone PG,Leigh MW, Zariwala MA, Minnix SL, Knowles MR, Molina PL. High-Resolution CT of Patients with Primary Ciliary Dyskinesia.  Am J Roentgenol. 2007 May;188(5):1232-8.

13. Kennedy, M. P., P. G. Noone, J. Carson, P. L. Molina, A. Ghio, M. A. Zariwala, S. L. Minnix, and M. R. Knowles. 2007. Calcium stone lithoptysis in primary ciliary dyskinesia. Respir Med 101:76-83.

14. Zariwala MA, Leigh MW, Ceppa F, Kennedy MP, Noone PG, et al.  Mutations of DNAII in primary ciliary dyskinesia:  Evidence of founder effect in a common mutation.  Am J Respir Crit Care Med. 2006; 174:  858-866. 

 15. Hornef N, Olbrich H, Horvath J, Zariwala MA, Fliegauf M, Loges NT, Wildhaber J, Noone PG, Kennedy M, Antonarakis SE, Blouin JL, Bartoloni L, Nublein T, Ahrens P, Griese M, Kuhl H, Sudbrak R, Knowles MR, Reinhardt R, Omran H.  DNAH5 Mutations are a Common Cause of Primary Ciliary Dyskinesia with Outer Dynein Arm Defects.  Am J Respir Crit Care Med. 2006; 174:  120-126.

 16. Macartney C, Burke E, Elborn S, Magee N, Noone PG, Gleadhill I, Allen D, Kettle P, Drake M. Department of Haematology.  Bronchiolitis obliterans organizing pneumonia in a patient with non-Hodgkin's lymphoma following R-CHOP and pegylated filgrastim.  Leuk Lymphoma. 2005 Oct;46(10):1523-6.

17. Chmura K, Chan ED, Noone PG, Zariwala M, Winn RA, Knowles MR, Iseman MD, Gardner EM.  A middle-aged woman with recurrent respiratory infections. Respiration. 2005 Jul-Aug;72 (4):427-30

 18. Standaert TA, Boitano L, Emerson J,  Milgram L, Konstan M,  Hunter J,  Bercalz PY, Brass-Ernst L, Zeitlin P, Hammond K, Davies Z, Foy C, Noone PG, Knowles MR. Standardized procedure for measurement of nasal potential difference:  An outcome measure in multi-center Cystic Fibrosis clinical trials.  Pediatr Pulmonol 2004; 37:  385-392

 19. Zariwala M, O'Neal WK, Noone PG, Leigh MW, Knowles MR, Ostrowski LE. Investigation of the possible role of a novel gene, DPCD, in Primary Ciliary Dyskinesia. Am J Respir Cell Mol Biol. 2004; Apr; 30(4):428-34.

20. PG Noone: Leigh MW, Sannuti A, Minnix SL, Carson JL, Hazucha M, Zariwala M, Knowles MR.  Primary ciliary dyskinesia:  Diagnostic and phenotypic features.  Am J Respir Crit Care Med 2004; 168:459-467. 

 21. Kispert A, Petry M, Olbrich H, Volz A, Ketelsen UP, Horvath J, Melkaoui R, Omran H, Zariwala M, Noone PG, Knowles M. Genotype-phenotype correlations in PCD patients carrying DNAH5 mutations. Thorax. 2003 Jun; 58(6):552-4.

 22. Olbrich H, Haffner K, Kispert A, Volkel A, Volz A, Sasmaz G, Lehrach H, Konietzko N, Zariwala M, Noone PG, Knowles MR, Mitchison H, Chung E, Hildebrandt F, Sudbrak R, Omran H.  Mutations of DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry.  Nature Genetics 2002;30:143-144.  [and]

 23. Cohn JA, Noone PG, Jowell PS.   Idiopathic pancreatitis related to CFTR: complex inheritance and identification of a modifier gene. J Investig Med 2002 ;50:247S-255S.  

24. Noone PG, Zariwala M, Sannuti A, Minnix S, Leigh MW, Carson , Knowles MR. Mutations in DNAI1 (IC78) Cause Primary Ciliary Dyskinesia. Chest 2002; 121:97S-98S. 

25. Noone PG, Knowles MRCFTR-opathies”:  Disease phenotypes associated with CFTR gene mutations.  Respir Res 2001, 2: 328-332.  

26. Southern KW, Noone PG, Bosworth DG, LeGrys VA, Knowles MR, Barker P.  A modified technique for measurement of nasal transepithelial potential difference in infants.   J Pediatr 2001; 139:353-358.

27. Noone PG,  Zhou Z, Silverman LM, Jowell PS, Knowles MR, Cohn JA.  Cystic Fibrosis Gene Mutations and Pancreatitis Risk:  Relation to Epithelial Ion Transport and Trypsin Inhibitor Gene Mutations.   Gastroenterology 2001; 121:1310-1315.

 28. Noone PG, Zariwala M (co-first authors), Sannuti A, Minnix S, Zhou Z, Leigh MW, Hazucha M, Carson JL, Knowles MR.  Germline Mutations in DNAI1 (IC78) Cause Primary Ciliary Dyskinesia.  Am J Resp Cell Mol Biol 2001; 25:577-583.

 29. Noone PG, Hamblett N, Accurso F, Aitken M, Boyle M, Dovey M, Gibson R, Johnson C,  Kellerman D,  Konstan M, Milgram L,  Mundahl J,  Rodman D,  Williams JW, Wilmott R, 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 2001; 32:122-128.

 30. Noone PG, Pue CA, Zhou Z, Friedman KJ, Wakeling EL, Ganeshananthan M, Simon RH, Silverman LM, Knowles MR. Lung Disease associated with the IVS8 5T allele of the CFTR Gene.   Am J Resp Crit Care Med 2000; 162:1919-1924.

31. Regnis J, Zeman KL, Noone PG, Knowles MR, Bennett WD.  Prolonged airway retention of insoluble particles in cystic fibrosis versus primary ciliary dyskinesia.  Adv Exp Biol.  2000; 26:149-162.

32. Noone PG, Hohneker KW, Gipson C, Foy C, Schwartzbach C, Efthimiou J, Pearlman R, Johnson LG,  Zhou Z,  Jones K, Noah TL, Leigh MW, Boucher RC, Knowles MR.  Safety and biological efficacy of a lipid-CFTR complex for gene transfer in the nasal epithelium of adult patients with cystic fibrosis.  Molecular Therapy. [The Journal of the Am Soc of Gene Therapy] 2000; 1: 105-114

33. Campbell, PW, Saiman, L editors, co-authors:   …..Noone, PG….et al. Use of aerosolized antibiotics in CF patients: Chest 1999;116:775-788. Consensus statement (by invitation / peer reviewed).

34. Noone PG, Bennett WD, Regnis J, Zeman KL, Carson JL, Boucher RC, Knowles MR.  Effects of aerosolized uridine-5’-triphosphate (UTP) on airway clearance during cough in patients with primary ciliary dyskinesia. Am J Resp Crit Care Med 1999;160:144-149.

 35. Noone PG, Bali D, Carson JL, Sannuti A, Gipson CL, Ostrowski LE, Bromberg PA, Boucher RC, Knowles MR.  Discordant organ laterality in monozygotic twins with Primary Ciliary Dyskinesia.  Am J Med Genetics 1999; 82:155-160.

 36. Cohn JA, Friedman KJ, Noone PG, Knowles MR, Silverman LM, Jowell PS.  Relations between mutations in the cystic fibrosis gene and idiopathic pancreatitis.  New England Journal of Medicine 1998;339:653-8.

37. Brinson G, Noone PG (senior corresponding author), Mauro M, Knowles MR, Yankaskas JR, Jacques P.  Bronchial artery embolization for hemoptysis in cystic fibrosis.  Am J Resp Crit Care Med 1998; 157:1951-1958.

 38. Knowles MR, Noone PG, Hohneker KW, Johnson LG, Boucher RC, Efthimou J. Crawford, C, Brown R, Schwartzbach C, Pearlman R.  A double blind placebo controlled dose ranging study to evaluate the safety and biologic efficacy of the lipid-DNA complex GR213487B in the nasal epithelium of adult patients with CF. Human Gene Therapy 1998; 9:249-269.

39. Noone PG, Regnis JA, Brouwer KR, Xinrong Liu, Robinson M, Knowles MR.  Airway deposition and systemic pharmacokinetics of amiloride following aerosolization with an ultrasonic nebulizer to normal airways. Chest 1997;112:1283-1290.

40. Toso C, Williams D, Noone PG.   Inhaled antibiotics in cystic fibrosis: A review.   Annals of Pharmacotherapy 1996;30:840-50.

 41. Knowles MR, Olivier K, Noone PG, Boucher RC.  Pharmacologic modulation of salt and water in the airway epithelium in cystic fibrosis. Am J Respir Crit Care Med 1995;151:S65-S69.

 42. Noone PG, King M, Loftus BG. Benign neonatal sleep myoclonus. Ir Med J 1995;88:172.

 43. Knowles MR, Noone PG. Evaluation of the patient with borderline sweat test results. Ped Pulm 1994; 10: 141-2.

44. Noone PG, Olivier KN, Knowles MR. Modulation of the ionic milieu of the airway in health and disease.  Annual Review of Medicine 1994;45: 421-434.

 45. Noone PG, Knowles MR. Trends in therapy of abnormal airway epithelial ion and liquid transport. Monaldi Archives of Chest Diseases 1993; 48: 140-143.

 46. Knowles MR, Olivier KN, Hohneker K, Noone PG, Mason S, Boucher RC. Acute safety and effects of aerosolized uridine 5-triphosphate (UTP) plus or minus amiloride.  Ped Pulm 1993; 9:148-9.

 47. Noone PG, O' Briain DS, Clancy L.  Adenocarcinoma of the lung in association with chronic sarcoidosis.  Ir Med J 1993;  86: 27-28.

 48. Tyrell J, Noone PG, Prichard JS.  Thoracic actinomycosis complicated by actinobacillus actinomycetemcomitans. Respiratory Medicine 1992; 86: 341-343. 

Book Chapters:

1.  Lobo J, Rojas-Balcazar JM, Noone PG (Sr).  Recent Advances in Cystic Fibrosis. Clin Chest Medicine 2012 Jun; 33(2): 307-28.

2. LJ Lobo, M. Ziriwla, Noone PG. Ciliary Dyskinesias; Primary Ciliary Dyskinesia in Adults. Monograph, Eur Resp Soc (Bronchiectasis) 2011,  Chapter 9, pp 130-149. 

3. Noone PG, Zariwala M, Knowles M. Primary Ciliary Dyskinesia.  In: Runge M, Patterson C, eds (Noone PG, Boucher RC section editors). Principles of Molecular Medicine, 2nd ed., Totowa, New Jersey: Humana Press, 2006, pp. 239-250.

4. Bennett WD, Noone PG, Knowles MR, Boucher RC. Regulation of Mucociliary Clearance by Purinergic Receptors. In Cilia and Mucus: From Development to Respiratory Defense, ed. Salathe M, Marcel Dekker, Inc. NY, 2001, pp 347-360. 

 5. Noone PG, Knowles MR.   Standard Therapy of Cystic Fibrosis Lung Disease. In:  Cystic fibrosis in adults. Yankaskas JR, Knowles MR, eds. Lippincott-Raven Publishers, Philadelphia, 1999; pp 145-173.

 6. Noone PG, Bresnihan B.  Rheumatologic disease in adults with CF. In:  Cystic fibrosis in adults. Yankaskas JR, Knowles MR, eds.  Lippincott-Raven Publishers, Philadelphia, 1999; pages 439-447.

 7. Knowles MR, Noone PG, Bennett WD, Boucher RC.  Mucociliary and Cough Clearance:  Role of Ion Transport and the P2Y2 Receptor-Mediated System.  In:  Cilia, Mucus, and Mucociliary Interactions.  Baum G, ed.  Marcel Dekker, Inc., New York 1998: pp 307-315.

8. Noone PG.  MD thesis:  Pharmacokinetic and Efficacy Studies of New Treatments for Cystic Fibrosis and Primary Ciliary Dyskinesia 1997, NUIG. (157 pages)

 9. Noone PG, Sicilian L.  Interstitial Lung Disease. In:  Difficult Diagnosis 11. Taylor Ed.  WB Saunders, Philadelphia 1992; pp 266-274.