![Lisa R. Young, MD](/sites/default/files/styles/crop_and_center_300x375/public/providers-new-young-lisa.jpg?itok=0GwsqA3t)
Lisa R. Young
About Lisa R. Young
Lisa Young, MD, is Chief of the Division of Pulmonary and Sleep Medicine at Children’s Hospital of Philadelphia and Professor of Pediatrics at the Perelman School of Medicine at the University of Pennsylvania. Before joining CHOP, Dr. Young was an Associate Professor of Pediatrics, Medicine, and Cell & Developmental Biology at Vanderbilt University and Director of the Center for Childhood Lung Research and the Pediatric Rare Lung Diseases Clinical Program at Vanderbilt University Medical Center. She was an undergraduate at the University of Virginia (where she was a Jefferson Scholar), earned her MD at Duke University, completed residency training in Pediatrics and Internal Medicine at Duke University, and completed fellowship training in Pediatric Pulmonology and Adult Pulmonary/Critical Care Medicine at Cincinnati Children’s Hospital Medical Center and the University of Cincinnati. Following her fellowship training, she joined the faculty at the University of Cincinnati for six years, then relocated to Vanderbilt in 2011.
Dr. Young is an outstanding physician-scientist and clinician-educator. She has been well-funded by the NIH and other funding agencies for both laboratory and clinical research on a variety of lung diseases and is currently principal investigator on 2 NIH R01 awards, an NIH U54 project, and a K24 Midcareer Award in Patient-Oriented Research. She has particular interest in interstitial and other rare lung diseases including Hermansky-Pudlak syndrome, neuroendocrine cell hyperplasia of infancy, primary ciliary dyskinesia, bronchopulmonary dysplasia, and lymphangioleiomyomatosis.
Dr. Young has received multiple awards for her research, including the American Thoracic Society Robert B. Mellins Outstanding Achievement Award, the American Thoracic Society Public Advisory Roundtable Excellence Award, the LAM Foundation Scientific Advancement Award, and a Parker B. Francis Foundation Fellowship. She has received recognition for her clinical skills from Best Doctors in America, Cincinnati Magazine, and the Hermansky-Pudlak Syndrome Network.
Dr. Young is committed to improving child lung health through expanding the outstanding clinical, training, and research programs in the Division of Pulmonary Medicine at CHOP.
Titles
Chief, Division of Pulmonary and Sleep Medicine
Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania
Certifications
Internal Medicine – American Board of Internal Medicine
Pediatric Pulmonology – American Board of Pediatrics
Pediatrics – American Board of Pediatrics
Pulmonary Disease – American Board of Internal Medicine
Awards and Honors
2019, American Thoracic Society Fellow
2018, Polgar Lectureship in Pediatric Pulmonology, CHOP
2018, Janie Robinson and John Moore Lee Endowed Chairholder in the Department of Pediatrics, Vanderbilt, University
2017, Public Advisory Roundtable (PAR) Excellence Award, American Thoracic Society
2017, Scientific Advancement Award, The LAM Foundation
2016, Scientific Advancement Award, Hermansky-Pudlak Syndrome Network
2015, Doctor of the Year Award, Hermansky-Pudlak Syndrome Network
2015, Established Investigator Award, The LAM Foundation
2015, Jake Arricale Professorship in Pediatric Interstitial Lung Disease, Department of Pediatrics, Johns Hopkins University
2014, Robert B. Mellins, M.D. Outstanding Achievement Award in Pediatrics, American Thoracic Society
2014, Excellence Award, Hermansky-Pudlak Syndrome Network
2008, Early Career Development Award, Central Society for Clinical Research
2008, Young Investigator Award, 15th International Colloquium on Lung Airway Fibrosis (ICLAF)
2007-2010, Parker B. Francis Foundation Fellow
2007, Carl Booberg Research Award, American Thoracic Society
2005-2007, Procter Research Scholar, Cincinnati Children's Hospital Medical Center
1993, Raven Honor Society, University of Virginia
1989-1993, Jefferson Scholar, University of Virginia
1989-1993, Echols Scholar, University of Virginia
Leadership and Memberships
Memberships in Professional Organizations
International
2017-present, European Network for Translational Research in Children's and adult Interstitial Lung Diseases
National
2018-present, Boehringer Ingelheim, Pediatric Advisory Board
2015-2018, Data and Safety Monitoring Boards
- 2015-2018, Member, A Pilot Clinical Trial of COX-2 inhibition in LAM and TSC
- 2015-2018, Chair, Lymphangioleiomyomatosis (LAM) Pilot Study with Imatinib Mesylate (LAMP-1) Trial
2014-2018, NIH/NHLBI Grant Review, Standing Study Sections
- 2018, K23/24 Standing Member Conflict
- 2017, Lung Injury Repair and Remodeling, ad hoc Reviewer
- 2016, Lung Injury Repair and Remodeling, ad hoc Reviewer
2014-present, Hermansky-Pudlak Syndrome Network Medical Advisory Board
2012-present, The Tuberous Sclerosis Alliance
- 2012-present, Professional Advisory Board
2010-present, The Lymphangioleiomyomatosis (LAM) Foundation
- 2015-present, Executive Committee
- 2012-present, Board of Directors
- 2010-present, Scientific Advisory Board
2009-present, Society for Pediatric Research
2005-present, Children's Interstitial and Diffuse Lung Disease Research Network
- 2016-present, Director and Principal Investigator, Children's Interstitial and Diffuse Lung Disease National Registry
2005-present, American Thoracic Society
- 2018-2019, Chair, Program Committee, Respiratory Cell and Molecular Biology Assembly
- 2017-2018, Chair-elect, Program Committee, Respiratory Cell and Molecular Biology Assembly
- 2017-2018, Committee, Future Directions in Lung Precision Medicine Research
- 2015-2018, Planning Committee, Respiratory Cell and Molecular Biology Assembly
- 2014-present, Committee for Rare Lung Diseases
2004-present, Rare Lung Diseases Consortium, NIH Rare Disease Clinical Research Network
- 2015-present, Operations Committee
- 2014-present, PI, Project 3: Preparing for Treatment Trials in Hermansky Pudlak Syndrome
- 2014-present, Director, Pilot Program
Editorial and Academic Positions
Editorial Positions
2015-present, Editorial Board Member, Pediatric Pulmonology
Reviewer
2017-present, PNAS
2017-present, PLOS One
2016-present, Journal of Clinical Investigation - Insights
2016-present, Respirology
2015-present, Orphanet Journal of Rare Diseases
2014-present, Respiratory Medicine
2014-present, European Respiratory Journal
2014-present, Annals of the American Thoracic Society
2014-present, Chest
2014-present, Nature Medicine
2013-present, The Lancet Respiratory Medicine
2013-present, American Journal of Respiratory Cell and Molecular Biology
2013-present, American Journal of Respiratory and Critical Care Medicine
2013-present, Journal of Clinical Investigation
2012-present, Pediatric Pulmonology
2012-present, Pediatric Allergy, Immunology, and Pulmonology
2010-present, American Journal of Human Genetics
2010-present, Sarcoidosis, Vasculitis, and Diffuse Lung Diseases
2010-present, Archives of Diseases of Childhood
2010-present, American Journal of Physiology (Lung, Cellular, and Molecular Biology)
2010-present, Thorax
2010-present, Pediatrics
2008-present, Journal of Pediatrics
Research Interests
Hermansky-Pudlak syndrome
Idiopathic pulmonary fibrosis
Lymphangioleiomyomatosis
Primary ciliary dyskinesia
Interstitial lung disease
Lung development and bronchopulmonary dysplasia
Lung injury and repair
Education & training
Medical Degree
MD - Duke University, Durham, NC
Residency
Pediatrics and Internal Medicine - Duke University, Durham, NC (Assistant Chief Resident)
Fellowship
Pediatric Pulmonology and Adult Pulmonary/Critical Care Medicine - Cincinnati Children’s Hospital Medical Center and the University of Cincinnati, Cincinnati, OH
Publications
Publications
2019
N Gupta, HS Lee, LR Young, C Strange, J Moss, L Singer, K Nakata, A Barker, J Chapman, M Brantly, J Stocks, KK Brown, J Lynch, H Goldbery, G Downey, A Taveira-DaSilva, J Krischer, K Setchell, BC Trapnell, Y Inoue, and FX McCormack: "Analysis of the MILES Cohort Reveals Determinants of Disease Progression and Treatment Response in Lymphangioleiomyomatosis." Eur Respir J. 2019 Apr 4;53(4). pii: 1802066. doi: 10.1183/13993003.02066-2018. Print 2019 Apr.
Stearman RS, Cornelius AR, Young LR, Conklin DS, Mickler EA, Lu X, Hara N, Fettig LM, Phang TL, Geraci MW: "Familial Pulmonary Fibrosis and Hermansky-Pudlak Syndrome Rare Missense Mutations In Context" Am J Respir Crit Care Med April 2019.
Gupta N, Lee HS, Young LR, Strange C, Moss J, Singer LG, Nakata K, Barker AF, Chapman JT, Brantly ML, Stocks JM, Brown KK, Lynch JP, Goldberg HJ, Downey GP, Taveira-DaSilva AM, Krischer JP, Setchell K, Trapnell BC, Inoue Y, McCormack FX: "Analysis of the MILES Cohort Reveals Determinants of Disease Progression and Treatment Response in Lymphangioleiomyomatosis." European Respiratory Journal March 2019.
M Hirose, A Matsumuro, T Arai, C Sugimoto, M Akira, M Kitaichi, LR Young, FX McCormack, and Y Inoue: Serum Vascular Endothelial Growth Factor-D as a Diagnostic and Therapeutic Biomarker for Lymphangioleiomyomatosis. PLoS One. 2019 Feb 28;14(2):e0212776. doi: 10.1371/journal.pone.0212776. eCollection 2019.
Garg A, Sui P, Verheyden JM, Young, LR, Sun X.: "Consider the lung as a sensory organ: A tip from pulmonary neuroendocrine cells." Current Topics in Developmental Biology. Epub 132: 67-89, Jan 2019.
2018
S Kook, A Qi, P Wang, S Meng, P Gulleman, LR Young, and SH Guttentag: "Gene-edited MLE-15 Cells as a Model for the Hermansky Pudlak Syndromes" Amer Journal of Resp Cell and Mol Biol 58(5): 556-574, 2018.
RJ Nevel, ET Garnett, D Schaudies, and LR Young: "Growth Trajectories and Oxygen Use in Neuroendocrine Cell Hyperplasia of Infancy." Pediatric Pulmonol 53(5): 656-664, 2018.
JT Benjamin, R van der Meer, JC Slaughter, S Steele, EJ Plosa, JM Sucre, PE Moore, JL Aschner, TS Blackwell, and LR Young: "Inverse Relationship between Soluble RAGE and Risk for Bronchopulmonary Dysplasia" Am J Respir Crit Care Med 197(8): 197-8, 2018.
JS Sucre, GH Deutsch, C Jetter, N Ambalavanan, JT Benjamin, LA Gleaves, B Millis, LR Young, TS Blackwell, JA Kropski, and SH Guttentag: "A Shared Pattern of Beta-catenin Activation in Bronchopulmonary Dysplasia and Idiopathic Pulmonary Fibrosis." The American Journal of Pathology 188(4): 853-862, 2018.
JS Sucre, CS Jetter, H Loomans, J Williams, EJ Plosa, JT Benjamin, LR Young, JA Kropski, CL Calvi, S Kook, P Wang, L Gleaves, A Eskaros, L Goetzl, TS Blackwell, SH Guttentag, and A Ziilstra: "Successful Establishment of Primary Type 2 Alveolar Epithelium with 3D Organotypic Co-culture." Amer Journal of Resp Cell and Mol Biol 59(2): 158-166, 2018.
A Burman, JA Kropski, CL Calvi, AP Serezani, BD Poscoalino, W Han, T Sherrill, L Gleaves, WE Lawson, LR Young, TS Blackwell, and H Tanjore.: "Localized Hypoxia links ER stress to lung fibrosis through induction of C/EBP homologous protein." JCI Insight 3(16): doi: 10.1172, Aug 2018 Notes: epub ahead of print.
2017
MG O'Connor, JC Godfrey, S Chinnadurai, and LR Young: "Early Diagnosis of Primary Ciliary Dyskinesia: The Role of the Pediatrician" Clinical Pediatrics 56(10): 967-970, 2017.
JT Benjamin, R van der Meer, JC Slaughter, S Steele, EJ Plosa, JM Sucre, PE Moore, JL Aschner, TS Blackwell, and LR Young: "Inverse Relationship between Soluble RAGE and Risk for Bronchopulmonary Dysplasia" Am J Respir Crit Care Med 197(8): 1083-1086, 2017 Notes: Epub ahead of print.
JA Kropski, LR Young, JD Cogan, DB Mitchell, LH Lancaster, JA Worrell, C Markin, N Liu, WR Mason, TE Fingerlin, DA Schwartz, WE Lawson, TS Blackwell1, JA Phillips, III, and JE Loyd: "Genetic evaluation and testing of patients and families with idiopathic pulmonary fibrosis" American Journal of Respiratory and Critical Care Medicine 195(11): 1423-28, 2017.
C Lu, HS Lee, G Pappas, D Dilling, C Burger, A Shifren, S Veeraraghavan, JT Chapman, J Parambil, S Ruoss, LR Young, S Hammes, EJ Kopras, T Roads, JP Krischer, FX McCormack: "Trial of An Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis" Annals of the American Thoracic Society 14(3): 919-28, 2017.
AS Kristof, BJ Petrof, Q Hamid, M Kolb, JS Landry, A MacKenzie, FX McCormack, IJ Murawski, J Moss, F Rauch, IO Rosas, AJ Shapiro, BM Smith, DY Thomas, BC Trapnell, LR Young, Maimoona A Zariwala: "An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases" Annals of the American Thoracic Society 14(8): 1239-47, 2017.
2016
T Takada, A Mikami, N Kitamura, K Seyama, Y Inoue, K Nagai, M Suzuki, H Moriyama, K Akasaka, R Tazawa, T Hirai, M Mishima, M Hayashida, M HC Sugimoto, T Arai, N Hattori, K Watanabe, T Tamada, H Yoshizawa, K Akazawa, T Tanaka, K Yagi, LR Young, FX McCormack, and K Nakata: "Efficacy and safety of long-term sirolimus therapy in Asian patients with Lymphangioleiomyomatosis" Annals of the American Thoracic Society 13(11): 1912-1922, 2016.
S Kook, Ping Wang, LR Young, M Schwake, P Saftig, X Weng, Y Meng, D Neculai, MS Marks, L Gonzales, MF Beers, and S Guttentag: "Impaired Lysosomal Integral Membrane Protein 2-dependent Peroxiredoxin 6 delivery to lamellar bodies accounts for altered alveolar phospholipid content in Adaptor Protein-3-deficient pearl mice" J Biol Chem 291(16): 8414-27, 2016.
RJ Nevel, ET Garnett, JA Worrell, RL Morton, LM Nogee, TS Blackwell, and LR Young: "Persistent Lung Disease in Adults with NKX2.1 Mutation and Familial Neuroendocrine Cell Hyperplasia of Infancy (NEHI)" Annals of the American Thoracic Society 13(8): 1299-304, 2016.
R Zaynagetdinov, TP Sherrill, LA Gleaves, RP Hunt, W Han, AG McLoed, JA Saxon, H Tanjore, PM Gulleman, LR Young, TS Blackwell: "Chronic NF-κB activation links COPD and lung cancer through generation of an immunosuppressive microenvironment in the lungs" Oncotarget 7(5): 5470-82, 2016.
P Houin, RR Deterding, and LR Young: "Exacerbations in Neuroendocrine cell Hyperplasia of Infancy are Associated with Air-Trapping" Pediatric Pulmonology 51(3): E9-E12, 2016.
LR Young, BC Trapnell, KD Mandl, DT Swarr, JA Wambach, CJ Blaisdell: "Accelerating Scientific Advancement for Pediatric Rare Lung Disease Research: Report from an NIH-NHLBI Workshop, September 3-4, 2015" Annals of the American Thoracic Society 13(12): 385-393, 2016.
LR Young*, PM Gulleman, CW Short, H Tanjore, TP Sherrill, A Qi, AP McBride, R Zaynagetdinov, JT Benjamin, WE Lawson, SV Novitskiy, and TS Blackwell.: "Epithelial-Macrophage Interactions Determine Pulmonary Fibrosis Susceptibility in Hermansky-Pudlak Syndrome" Journal of Clinical Investigation - Insights 1(17): e88947, 2016 Notes: *corresponding author.
KM McDowell, AH Jobe, M Fenchel, WD Hardie, T Gisslen, LR Young, CA Chougnet, SD Davis, and SG Kallapur: "Pulmonary morbidity in infancy after exposure to chorioamnionitis in late preterm infants" Annals of the American Thoracic Society 13(6): 867-76, 2016.
KJ O'Brien, J Lozier, AR Cullinane, B Osorio, K Nghiem, V Speransky, W Zein, JC Mullikin, A. Neff, MCV Malicdan, WA Gahl, LR Young, BR Gochuico: "Identification of a novel mutation in Hermansky-Pudlak Syndrome-6 in a Patient with Hemophilia B and oculocutaneous albinism" Molecular Genetics and Metabolism S1096-7192(16): 30211-6, 2016.
JT Benjamin, R van der Meer, AM Im, EJ Plosa, R Zaynagetdinov, A Burman, ME Havrilla, LA Gleaves, VV Polosukhin, GH Deutsch, H Yanagisawa, JM Davidson, LS Prince, LR Young, and TS Blackwell: "Epithelial-Derived Inflammation Disrupts Elastin Assembly and Alters Saccular Stage Lung Development" American Journal Pathology 186(7): 1786-800, 2016.
GW Vicary, Y Vergne, A Santiago-Cornier, LR Young, and J Roman: "Pulmonary Fibrosis in Hermansky-Pudlak Syndrome" Annals of the American Thoracic Society 13(10): 1839-1846, 2016.
GH Deutsch and LR Young: "Lipofibroblast Phenotype in Pulmonary Interstitial Glycogenosis" American Journal of Respiratory and Critical Care Medicine 193(6): 694-6, 2016.
JA Saxon, TP Sherrill,VV Polosukhin, J Sai, R Zaynagetdinov, AG McLoed, P Gulleman, W Barham, DS Cheng, RP Hunt, LA Gleaves, A Richmond, LR Young, FE Yull, and TS Blackwell: "Epithelial NF-κB signaling promotes EGFR-driven lung carcinogenesis via macrophage recruitment" Oncoimmunology 5(6): e1168549, Mar 2016.
2015
W Han, R Zaynagetdinov, FE Yull, VV Polosukhin, LA Gleaves, H Tanjore, LR Young, TE Peterson, HC Manning, LS Prince and TS Blackwell: "Molecular imaging of folate receptor beta positive macrophages during acute lung inflammation" American Journal of Respiratory Cell and Molecular Biology 53(1): 50-9, 2015.
N Gupta, R Miraj, D Tanase, LE James, K Seyama, DA Lynch, M Akita, CA Meyer, SJ Ruoss, CD Burger, LR Young, KF Almoosa, S Veeraraghavan, AF Barker, AS Lee, DF Dilling, Y Inoue, CJ Cudzilo, MA Zafar, and FX McCormack: "Accuracy of high resolution CT chest in diagnosing diffuse cystic lung diseases" European Respiratory Journal 46(4): 1196-9, 2015.
MG O'Connor, M Wurth, and LR Young: "Rare Becomes More Common: Recognizing Neuroendocrine Cell Hyperplasia of Infancy (NEHI) in Everyday Pulmonary Consultations" Annals of the American Thoracic Society 12(11): 1730-2, 2015.
LL Fan, MK Dishop, C Galambos, FB Askin, FV White, C Langston, DR Liptzin, ME Kroehl, GH Deutsch, LR Young, G Kurland, J Hagood, S Dell, BC Trapnell, RR Deterding: "Diffuse Lung Disease in Biopsied Children 2-18 Years of Age: Application of the ChILD Classification Scheme" Annals of the American Thoracic Society 12(10): 1498-505, 2015.
JD Cogan, JA Kropski, M Zhao, DB Mitchell, L Rives, C Markin, ET Garnett, KH Montgomery, WR Mason, DE McKean, J Powers, E Murphy, LM Olson, L Choi, LR Young, LH Lancaster, MP Steele, TE Fingerlin, M Schwarz, DA Schwartz, WE Lawson, JE Loyd, Z Zhao, JA Phillips III, and TS Blackwell: "Rare Variants in RTEL1 are Associated with Familial Interstitial Pneumonia" American Journal of Respiratory and Critical Care Medicine 161(6): 646-55, 2015.
HN Purcell, A Whisenhunt, J Cheng, S. Dimitriou, LR Young, and DH Grossoehme: "Parents' use of faith following child's rare disease diagnosis (NEHI)" Journal of Health Care Chaplaincy 21(1): 25-38, 2015.
B Lo, K Zhang, W Lu, L Zheng, Q Zhang, C Kanellopoulou, Y Zhang, Z Liu, JM Fritz, R Marsh, A Husami, D Kissell, S Nortman, V Chaturvedi, H Haines, LR Young, J Mo, AH Filipovich, JJ Bleesing, P Mustillo, M Stephens, CM Rueda, CA Chougnet, K Hoebe, J McElwee, JD Hughes, E Karakoc-Aydiner, HF Matthews, VK Rao, S Price, H Su, V Koneti Rao, MJ Lenardo, and MB Jordan.: "Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy" Science 349(6246): 436-440, 2015.
JA Kropski, JM Pritchett, DF Zoz, PF Crossno, C Markin, ET Garnett, AL Degryse, DB Mitchell, VV Polosukhin, OB Rickman, L Choi, DS Cheng, ME McConaha, BR Jones, LA Gleaves, JA Worrell, JF Solus, LB Ware, CM Stein, JW Lee, PP Massion, R Zaynagetdinov, ES White, JE Johnson, SD Groshong, LH Lancaster, LR Young, MP Steele, JA Phillips III, JD Cogan, JE Loyd, WE Lawson, and TS Blackwell: "Extensive phenotyping of individuals at-risk for Familial Interstitial Pneumonia reveals clues to the pathogenesis of interstitial lung disease" American Journal of Respiratory and Critical Care Medicine 191(4): 417-26, 2014.
2014
EJ Plosa, LR Young, PM Gulleman, VV Polosukhin, R Zaynagetdinov, JT Benjamin, AM Im, R van der Meer, LA Gleaves, N Bulus, W Han, LS Prince, TS Blackwell, and R Zent: "Epithelial b1 integrin is required for lung branching morphogenesis and alveolarization." Development 141(24): 4751-62, 2014.
2013
MA Zafar, FX McCormack, S Rahman, C Tencza, KA Wikenheiser-Brokamp, LR Young, Y Shizukuda, and JM Elwing: "Pulmonary Vascular Shunts in Exercise Intolerant Patients with Lymphangioleiomyomatosis" American Journal of Respiratory and Critical Care Medicine 188(9): 1167-70, 2013.
LR Young, GH Deutsch, R Bokulic, A Brody, and LM Nogee: "A mutation in TTF1/NKX2.1 is associated with familial Neuroendocrine Cell Hyperplasia of Infancy (NEHI)" Chest 144(4): 1199-1206, 2013.
JJ Swigris, HS Lee, M Cohen, Y Inoue, J Moss, L Singer, LR Young, and FX McCormack: "St. George's Respiratory Questionnaire has Longitudinal Construct Validity in Lymphangioleiomyomatosis" Chest 143(6): 1671-8, 2013.
JJ Soares, GH Deutsch, PE Moore, MF Fazili, ED Austin, RF Brown, AG Sokolow, MA Hilmes, and LR Young: "Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis" Pediatrics 123(4): 684-91, 2013.
JA Kropski, WE Lawson, LR Young, and TS Blackwell: "Genetic Studies in Pulmonary Fibrosis Provide Clues for Understanding IPF Pathogenesis" Disease Models and Mechanisms(1), 9-17, 2013.
FX McCormack, C Wilmott, LR Young, and K Wikenheiser-Brokamp: "An Atoll Variant that Mocks" American Journal of Respiratory and Critical Care Medicine, 188(1): 110, 2013.
C Cudzilo, RV Szczesniak*, AS Brody, M Rattan, DA Krueger, JJ Bissler, DN Franz, FX McCormack,and LR Young: "Lymphangioleiomyomatosis Screening in Women with Tuberous Sclerosis Complex" Chest 144(2): 578-85, 2013.
A Hamvas*, RR. Deterding*, SE Wert, FV White, MK Dishop, DN Alfano, AC Halbower, B Planer, MJ Stephan, DA Uchida, LD Williames, JA Rosenfeld, RR Lebel, LR Young, FS Cole, and LM Nogee: "Heterogeneous pulmonary phenotypes associated with the Brain-Thyroid-Lung Syndrome and mutations in the thyroid transcription factor gene NKX2-1" Chest 144(3): 794-804, 2013 Notes: **This article had an accompanying editorial.
LR Young, HS Lee, Y Inoue, J Moss, LG Singer, C Strange, K Nakata, AF Barker, JT Chapman, ML Brantly, JM Stocks, KK Brown, JP Lynch, III, HJ Goldberg, GP Downey, JJ Swigris, AM Taveira-DaSilva, JP Krischer, BC Trapnell, FX McCormack: "Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) Trial" The Lancet Respiratory Medicine 1(6): 445-452, Aug 2013 Notes: This article had an accompanying editorial and was recommended by The Faculty of 1000.
2012
R Meraj, KA Wikenheiser-Brokamp, LR Young, and FX McCormack: "Lymphangioleiomyomatosis: New Concepts in Pathogenesis, Diagnosis, and Treatment" Seminars in Respiratory and Critical Care Medicine 33(5): 486-97, 2012.
R Meraj, K Wikenheiser-Brokamp, LR Young, S Byrnes, and FX McCormack: "Utility of Transbronchial Biopsy in the Diagnosis of Lymphangioleiomyomatosis" Frontiers in Medicine 4: 395-405, 2012.
LR Young*, PM Gulleman, JP Bridges, TE Weaver, GH Deutsch, TS Blackwell, and FX McCormack: "The Pulmonary Epithelium Determines Susceptibility to Lung Fibrosis in Hermansky Pudlak Syndrome" American Journal of Respiratory and Critical Care Medicine 186(10): 1014-24, 2012 Notes: *corresponding author* This article had an accompanying editorial and was recommended by The Faculty of 1000.
2011
LR Young, A Brody, J Acton, T Inge, R Bokulic, C Langston and GH Deutsch: "Neuroendocrine Cell Distribution and Frequency Distinguish Neuroendocrine cell Hyperplasia of Infancy from Other Pulmonary Disorders" Chest 139(5): 1060-71, 2011.
FX McCormack, Y Inoue, J Moss, L Singer, C Strange, K Nakata, A Barker, J Chapman, ML Brantly, JM Stocks, KK Brown, JP Lynch, H Goldberg, LR Young, BW Kinder, EJ Sullivan, TV Colby, R McKay, MM Cohen, L Korbee, AM Taveira-DaSilva, H-S Lee, JP Krischer, and BC Trapnell: "Efficacy and Safety of Sirolimus in Lymphangioleiomyomatosis" New England Journal of Medicine 364(17): 1595-606, 2011.
2010
T Suzuki, T Sakagami, LR Young, RE Wood, BC Carey, C Chalk, M Luisetti, SE Wert, BK Rubin, K Kevill, Carrie Stevens, JA Whitsett, LM. Nogee, and BC Trapnell: "Hereditary Pulmonary Alveolar Proteinosis: Presentation, Pathogenesis, Diagnosis and Therapy" American Journal of Respiratory and Critical Care Medicine, 182(10): 1292-304, 2010.
LR Young, R VanDyke, PM Gulleman, Y Inoue KK Brown, LS Schmidt, WM Linehan, F Hajjar, BW Kinder, BC Trapnell, JJ Bissler, DN Franz, and FX McCormack: "Vascular Endothelial Growth Factor D for the Serodiagnosis of Lymphangioleiomyomatosis" Chest 138(3): 674-81, 2010.
GH Deutsch and LR Young: "Pulmonary Interstitial Glycogenosis: Words of Caution" Pediatric Radiology 40(9): 1562-5, 2010.
AS Brody, RP Guillerman, TC Hay, BD Wagner, LR Young, GH Deutsch, LL Fan, and RR Deterding: "Neuroendocrine Cell Hyperplasia of Infancy: Diagnosis with High resolution chest CT" American Journal of Roentgenology 194: 238-44, 2010.
BW Kinder, AC Sherman, LR Young, JT Hagaman, N Oprescu, S Byrnes, and FX McCormack: "Predictors, Barriers and Motivating Factors for Clinical Trial Participation in Lymphangioleiomyomatosis" Respiratory Medicine 104(4): 578-83, Apr 2010.
2009
CC Caldwell, Y Chen, MT Borchers, H Goetzmann, LR Young, DJ Hassett, D Mavrodi, L Thomashow, and GW Lau: "Pseudomonas aeruginosa Exotoxin Pyocyanin Causes Cystic Fibrosis Airway Pathogenesis" American Journal of Pathology 175(6): 2473-88, Dec 2009.
GH Deutsch and LR Young: "Histologic Resolution of Pulmonary Interstitial glycogenosis" Pediatric and Developmental Pathology 12(6): 475-80, Nov-Dec 2009.
AL Snow, RA Marsh, P Roehrs, D Dahr, S Krummey, LR Young, J van Hoff, K Zhang, AH Filipovich, HC Su, J Bleesing, and MJ Leonardo: "Restimulation-induced apoptosis of T cells is impaired in patients with X-linked lymphoproliferative disease caused by SAP deficiency" Journal of Clinical Investigation 119(10): 2976-89, Oct 2009.
LR Young, DN Franz, P Nagarkatte, C Fletcher, KA Wikenheiser-Brokamp, MD Galsky, TC Corbridge, AP Lam, MJ. Gelfand, and FX McCormack: "Utility of FDG-PET in Sporadic and Tuberous Sclerosis Associated Lymphangioleiomyomatosis" Chest 136(3): 926-33, Sept 2009.
V Schmithorst ,TA Altes, LR Young, FX McCormack, and AS Brody: "An Automated Algorithm for Quantifying the Extent of Cystic Change On Volumetric Chest CT: Initial Results in Lymphangioleiomyomatosis" AJR American Journal of Roentgenology 192(4): 1037-44, Apr 2009.
2008
JJ Bissler, FX McCormack*, LR Young*, JM Elwing, G Chuck, JM Leonard, VJ Schmithorst, T Laor, AS Brody, JA Bean, S Salisbury, and DN Franz*.: "Sirolimus for Angiomyolipomata in Patients with Tuberous Sclerosis Complex and Lymphangioleiomyomatosis" New England Journal of Medicine 358: 140-151, 2008 Notes: *denotes equal contribution.
LR Young, Y Inoue, and FX McCormack: "Diagnostic Potential of Serum VEGF-D for Lymphangioleiomyomatosis." New England Journal of Medicine 358(2): 199-200, Jan 2008.
2007
GH Deutsch*, LR Young*, RR Deterding, LL Fan, SD Dell, JA Bean, AS Brody, LM Nogee, BC Trapnell, C Langston and the Pathology Cooperative Group: "Cross-sectional Evaluation of Diffuse Parenchymal Lung Disease in Young Children: Application of a Novel Clinical-Pathologic Classification Scheme" American Journal of Respiratory and Critical Care Medicine 176(11): 1120-8, 2007 Notes: *denotes equal contribution as first authors.
LR Young, R Pasula, PM Gulleman, GH Deutsch, and FX McCormack: "Susceptibility of Hermansky-Pudlak Mice to Bleomycin-Induced Type II Cell Apoptosis and Fibrosis" American Journal of Respiratory Cell and Molecular Biology 37(1): 67-74, Jul 2007.
HD Thomas, K Risma, TB Graham, AS Brody, LR Young, and PM Joseph: "A Kindred of Children with Interstitial Lung Disease" Chest 132(1): 221-30, Jul 2007.
2006
KF Almoosa, J Mendez, JT Huggins, LR Young, M Coutinho, S Byrnes, E Sullivan, J Ryu, SP Bar-Ziv, J Maurer, FX McCormack, and S Sahn: "Pneumothorax in Lymphangioleiomyomatosis" Chest 129: 1274-1281, 2006.
F Kong, LR Young, H Ran, M Meyers, Y Chen, P Joseph, DJ Hassett, and GW Lau.: "Pseudomonas Aeruginosa Pyocyanin Inactivates Lung Epithelial V-ATPase Activity, Thereby Inhibiting Expression and Trafficking of the Cystic Fibrosis Transmembrane Regulator" Cellular Microbiology 8(7): 1121-1133, 2006.
MT Borchers, NL Harris, SC Wesselkamper, S Zhang, Y Chen, LR Young, and GW Lau: "The NKG2D Activating Receptor Mediates Pulmonary Clearance of Pseudomonas aeruginosa" Infection and Immunity 74(5): 2578-86, May 2006.
LR Young, K Almoosa, SP Bar-Ziv, M Coutinho, FX McCormack, and S Sahn: "Patient Perspectives on Pneumothorax and Management in Lymphangioleiomyomatosis" Chest 129(5): 1267-73, May 2006.
LR Young, MT Borchers, R Gibbons, H Allen, and FX McCormack.: "Lung Restricted Macrophage Activation in the Pearl Mouse Model of Hermansky Pudlak Syndrome" Journal of Immunology 1;176(7): 4361-8, Apr 2006.
2003
LR Young, D Hadjiliadis, RD Davis, and SM Palmer: "Lung Transplantation Exacerbates Gastroesophageal Reflux Disease" Chest 124(5): 1689-93, Nov 2003.
1999
E Bennett-Guerrero, I. Welsby, TJ Dunn, LR Young, TA Wahl, TL Diers, BG Phillips-Bute, MF Newman, and MG Mythen: "The use of a postoperative morbidity survey to evaluate patients with prolonged hospitalization after routine, moderate-risk, elective surgery" Anesthesia and Analgesia 89(2): 514-9, Aug 1999.
JA Voynow, LR Young, Y Wang, T Horger, MC Rose, and BM Fischer.: "Neutrophil elastase increases MUC5AC mRNA and protein expression in respiratory epithelial cells" American Journal of Physiology 276(5): L835-L843, May 1999.
Abstracts (includes Posters and Scientific Presentations)
2017
JT Benjamin, R V Meer, S Steele, A Agthe, PE Moor, JL Aschner, LR Young, and TS Blackwell.: Tracheal Aspirate esRAGE is Reduced in Preterm Infants at Risk for Severe BPD. Pediatric Academic Societies, San Francisco, CA, 2017 Notes: poster.
A Qi, P Gulleman, S Jackson, W Han, E Plosa, J Benjamin, B Gochuico, W Gahl, S Kook, SH Guttentag, TS Blackwell, and LR Young.: Epithelial Nox4 Contributes to Fibrotic Susceptibility in Hermansky Pudlak Syndrome Mice. Gordon Research Conference on Lung Development, Injury, and Repair, New London, NH, Aug 2017 Notes: poster.
S Kook, A Qi, P Gulleman, LR Young, and S Guttentag.: Generation of mouse lung epithelial cell models of Hermansky-Pudlak syndrome (HPS) via CRISPR-Cas9 mediated mutation in MLE15. American Thoracic Society International Conference, Washington DC, May 2017 Notes: poster.
RJ Nevel, E Garnett, AB Arkon, and LR Young.: Neuroendocrine Cell Hyperplasia of Infancy (NEHI): Does Failure to Thrive Predict or Influence Pulmonary Morbidity? American Thoracic Society International Conference, Washington DC, May 2017 Notes: poster.
JJ Soares, J Debley, B Kinghorn, A Stevens, M Dishop LR Young, and GH Deutsch.: Isolated and Immune-mediated Pulmonary Capillaritis in Children. American Thoracic Society International Conference, Washington DC, May 2017 Notes: poster.
JA Kropski, C Markin, ET Garnett, N Liu, DB Mitchell, JA Worrell, JF Solus, JA Phillips III, JD Cogan,LR Young, MP Steele, JE Loyd, WE Lawson, TS Blackwell.: Early interstitial changes in family members of patients with Familial Interstitial Pneumonia. American Thoracic Society International Conference, Washington DC, May 2017 Notes: poster.
Lectures by Invitation
2019
Young LR. "The transforming landscape of ILD in children.", Invited speaker at symposium Infant and Child Lung Disorders: Genetics, Diagnostics and Tomorrow's Care,Stanford University, Palo Alta, California. Mar, 2019
Young LR. "Hermansky Pudlak Syndrome: From Pathogenesis Towards Trials". Invited speaker, Lung Institute, Cedars-Sinai Medical Center, Los Angeles, CA. Jan, 2019.
2018
Young LR. "The Cellular Basis of HPS Lung Disease." NIH/HPS Network Workshop on Gene Therapy for Hermansky Pudlak Syndrome. Bethesda, MD. Dec, 2018.
Young LR. "Multicenter Research in Pediatric Lung Disease: Where We Stand and Why", The 6th Annual Contemporary Management of Aerodigestive Disease in Children, Nashville, TN. Dec, 2018.
Young LR. "Hermansky Pudlak Syndrome: Looking to Treatment Trials." Invited speaker, The Rare Lung Diseases Consortium International Conference, Covington, KY. Sep, 2018.
Young LR. "A national registry for childhood interstitial and diffuse lung diseases in the United States." European Respiratory Society International Congress, Paris, France. Sep, 2018.
Young LR. 'Biomarkers for Lymphangioleiomyomatosis.' European Respiratory Society International Congress, Paris, France. Sep, 2018.
Young LR. "Childhood Interstitial Lung Diseases." Invited speaker, The Rare Lung Diseases Consortium International Conference, Covington, KY. Sep, 2018.
Young LR. "Neuroendocrine cell Hyperplasia of Infancy and Other Rare Lung Diseases." Invited speaker, FASEB Scientific Research Conference, The Lung Epithelium in Health and Disease, Olean, NY. Jul, 2018.
Young LR. "Alveolar Epithelial Cells: The Epi-Center of Lung Fibrosis." Featured Speaker at the 2018 American Thoracic Society International Conference, San Diego, CA. May, 2018.
Young LR. "Neuroendocrine cell Hyperplasia of Infancy: What's New and What's Nebulous." Invited speaker, American Thoracic Society International Conference, San Diego, CA. May, 2018.
Young LR. "The National Registry for Childhood Interstitial and Diffuse Lung Diseases: An Initial Report from the chILD Research Network." The American Thoracic Society International Conference, San Diego, CA. May, 2018.
Young LR. "Pulmonary Fibrosis in Hermansky Pudlak Syndrome: From Pathogenesis Towards Trials." Invited speaker, The Saban Research Institute of Children's Hospital Los Angeles, CA. Apr, 2018.
Young LR. "Rare Lung Diseases: Insights and Impact on Child Lung Health." Pediatric Grand Rounds, Polgar Lectureship in Pediatric Pulmonology, Children's Hospital of Philadelphia, Philadelphia, PA. Apr, 2018.
Young LR. "Hermansky Pudlak Syndrome: Mechanisms of Pulmonary Fibrosis." Research seminar, Children's Hospital of Philadelphia, Philadelphia, PA. Apr, 2018.
Young LR. "Hermansky Pudlak Syndrome Research Centers in The Rare Lung Diseases Consortium." HPS Research Conference, Uniondale, NY. Mar, 2018.
2017
Young LR. "Hermansky-Pudlak Syndrome and Rare Lung Diseases." Pulmonary Fibrosis Foundation Summit, Nashville, TN. Nov, 2017.
Young LR. "Familial Interstitial Lung Disease." European Network for Translational Research in Children's and Adult Interstitial Lung Disease, Plenary lecture, Prague, Czech Republic. Nov, 2017.
Young LR. "Progress and Unmet Needs in chILD: Updates from the chILDRN-U.S." European Network for Translational Research in Children's and Adult Interstitial Lung Disease, Plenary lecture, Prague, Czech Republic. Nov, 2017.
Young LR. "Rare Lung Diseases: Pathogenesis Towards Trials." Invited seminar, National Jewish Health, Denver, CO. Aug, 2017.
Young LR. "Hermansky Pudlak Syndrome: From Pathogenesis Towards Trials." Rare Pediatric Respiratory Disease: Science Shapes Precision Care conference. San Diego, CA. Jul, 2017.
Young LR. "Roadmap for Successful LAM Clinic Visits." 2017 International Research Conference on Tuberous Sclerosis and LAM. Washington, DC. Jun, 2017.
Young LR. "Biomarkers for Rare Lung Diseases." American Thoracic Society Project: An Official Research Statement of the ATS and NHLBI: Current Status and Future Directions in Lung Precision Medicine Research; ATS International Conference Workshop, Washington, DC. May, 2017.
Young LR. "Rare Lung Diseases: Pathogenesis Towards Trials." Invited seminar, Boston Children's Hospital, Harvard Medical School, Boston, MA. May, 2017.
Young LR. "Hermansky-Pudlak Syndrome Centers Report: An Update from The Rare Lung Diseases Consortium." 2017 Annual HPS Network Conference, Uniondale, NY Mar, 2017.
Young LR. "Research Summary of the Meeting of the Minds." 2017 Annual Hermansky-Pudlak Syndrome Network Conference, Uniondale, NY. Mar, 2017.
Young LR. "Hermansky Pudlak Syndrome: Update from Rare Lung Diseases Consortium." HPS Research Conference, Uniondale, NY. Mar, 2017.
Editorials, Reviews, Chapters
2018
LR Young and GH Deutsch: "Childhood Interstitial Lung Diseases: Disorders More Prevalent in Infancy." In Kendig and Chernick's Disorders of the Respiratory Tract in Children, 3rd edition. Elsevier. Wlimott, Boat, Bush, Chernick, Deterding, and Ratjen (eds.). 2018.
DL Lesser, LR Young, and JS Hagood: "Rare Childhood Lung Disorders." In Kendig and Chernick's Disorders of the Respiratory Tract in Children, 3rd edition. Elsevier. Wlimott, Boat, Bush, Chernick, Deterding, and Ratjen (eds.). 2018.
2017
LR Young and GH Deutsch: "Childhood Interstitial Lung Diseases: Disorders More Prevalent in Infancy" Kendig and Chernick's Disorders of the Respiratory Tract in Children, 3rd edition. Wlimott, Boat, Bush, Chernick, Deterding, and Ratjen, Elsevier, (eds.). 2017.
DL Lesser, LR Young, and JS Hagood: "Rare Childhood Lung Disorders" Kendig and Chernick's Disorders of the Respiratory Tract in Children, 3rd edition. Wlimott, Boat, Bush, Chernick, Deterding, and Ratjen, Elsevier, (eds.). 2017.
2016
JZ Berg and LR Young: "Lymphangioleiomyomatosis" Gender, Sex Hormones, and Respiratory Disease. Anna Hemnes (eds.). Springer International Publishing, 2016
2012
LR Young and GH Deutsch: "Childhood Interstitial Lung Diseases: Disorders More Prevalent in Infancy" Kendig and Chernick's Disorders of the Respiratory Tract in Children, 2nd edition. Wlimott, Boat, Bush, Chernick, Deterding, and Ratjen, (eds.). Elsevier, 2012.
LR Young: "Childhood Interstitial Lung Diseases" Diffuse Lung Disease: A Practical Approach,2nd edition. Robert P. Baughman and Roland M. du Bois (eds.). Springer Scientific, 2012.
DL Lesser, LR Young, and JS Hagood: "Rare Childhood Lung Disorders" Kendig and Chernick's Disorders of the Respiratory Tract in Children, 2nd edition. Wlimott, Boat, Bush, Chernick, Deterding, and Ratjen (eds.). Elsevier, 2012.
2011
BC Trapnell and LR Young: "Pulmonary Alveolar Proteinosis" Rudolph's Pediatrics, 21st edition. Rudolph, Rudolph, Hostetter, Lister, and Siegel (eds.). McGraw-Hill, 2011.
2010
LR Young and WA Gahl: "Hermansky-Pudlak Syndrome" Molecular Basis of Rare Lung Diseases. FX McCormack, BC Trapnell, and RJ Panos (eds.). Humana Press, 2010.
2005
LR Young and FX McCormack: "Hermansky-Pudlak Syndrome" Respiratory Genetics. E. Silverman (eds.). Hodder Arnold Publishers(UK), 2005.
2000
LR Young and Scott M. Palmer: "Community Respiratory Viral Infections: An Overlooked Cause of Morbidity and Mortality in Transplant Recipients" Infectious Complications in Transplant Recipients. N. Singh (eds.). Kluwer Academic Publishers, 2000 Notes: for the series: Perspectives on Critical Care Infectious Diseases edited by J. Rello.