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Harry C Dietz, III, M.D.

Co-Director, Medical Genetics Fellowship Training Program
Professor of Pediatrics

Male

Appointment Phone

410-955-3071

Main Location

The Johns Hopkins Hospital

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Call 410-464-6641 (8a.m. to 6p.m., EST, Mon-Fri)

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Titles

  • Co-Director, Medical Genetics Fellowship Training Program
  • Associate Director, Human Genetics Graduate Training Program
  • Director, William S. Smilow Center for Marfan Syndrome Research Institute of Genetic Medicine
  • Investigator, Howard Hughes Medical Institute
  • Victor A. McKusick Professor of Medicine and Genetics
  • Professor of Pediatrics
  • Assistant Professor of Neurosurgery
  • Associate Professor of Medicine
  • Joint Appointment in Molecular Biology and Genetics

Centers & Institutes

Departments

Locations

The Johns Hopkins Hospital

Appointment Phone: 410-955-3071

600 N. Wolfe Street
Sheikh Zayed Tower
Baltimore, MD 21287 map
Phone: 410-614-0701
Fax: 410-614-9246

Expertise

Aortic Aneurysms, Marfan Syndrome, Medical Genetics, Pediatric Cardiology

Research Interests

Pathogenesis of Marfan syndrome; Molecular basis of inherited disorders of vascular development and homeostasis; Genetics of aging; Nonsense-mediated mRNA decay

Biography

Dr. Harry “Hal” Dietz is a professor of pediatrics, an associate professor of medicine and an assistant professor of neurological surgery at the Johns Hopkins University School of Medicine. He is the Victor A. McKusick Professor of Genetics and also serves as the director of the William S. Smilow Center for Marfan Syndrome Research. 

Recognized as the world’s leading authority on Marfan syndrome, Dr. Dietz conducted genomic mapping and research on therapeutic agents for deficiencies in the genetic protein fibrillin, which is linked to Marfan syndrome. His research contributions have made diagnosis of the condition patient-specific and accurate. 

He has received more than 50 national and international awards and honors, including the Antoine Marfan Award from the National Marfan Foundation and the Art of Listening Award from the American Heart Association.

Dr. Dietz is an ad hoc reviewer for 19 different organizations, including the American Journal of Cardiology, the Archives of Pediatric and Adolescent Medicine and the Journal of Clinical Investigation. Over more than two decades, Dr. Dietz has mentored approximately 50 predoctoral and postdoctoral researchers. He has authored 213 original publications in peer-reviewed journals, 22 textbook chapters and 226 abstracts, and has also delivered more than 350 lectures on Marfan syndrome and related genetic disorders.

Dr. Dietz completed his B.S. at Duke University and received his M.D. from the SUNY Upstate School of Medicine. He completed a pediatric residency and a cardiology fellowship at Johns Hopkins University before joining the faculty in 1992. He is also board-certified by the American Board of Pediatrics.

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    Additional Information

  • Education +

    Degrees

    • SUNY Upstate Medical University / MD (1984)

    Residencies

    • Johns Hopkins University School of Medicine / Pediatrics (1989)

    Fellowships

    • Johns Hopkins University School of Medicine / Pediatrics (1992)
  • Research & Publications +

    Research Summary

    Dr. Dietz and his laboratory team are interested in the development and homeostasis of the arterial wall.

    One goal is to understand genetic factors that predispose to aortic aneurysm, a condition accounting for 1-2 percent of deaths in industrialized countries. Their initial approach has been to study Marfan syndrome (MFS), a genetic disease that includes aortic aneurysm as part of the condition and that is caused by mutations in a single gene. It is anticipated that a comprehensive understanding of the cause, progression, and modulation of MFS will promote a greater understanding of vascular wall biology.

    Other vascular disorders currently being studied include: familial tetralogy of Fallot, cerebral cavernous malformation, a novel dominant syndrome of premature aging that prominently includes the cardiovascular system, the association of bicuspid aortic valve with aneurysm, and a novel and aggressive aneurysm phenotype called Loeys-Dietz syndrome.

    During their study of MFS, the Dietz lab recognized that a particular type of mutation is associated with very low levels of mutant RNA and tends to cause very mild forms of the disease.

    A second major interest of the laboratory is to understand the mechanism of nonsense-mediated mRNA decay; to evaluate its basic biologic purpose; and to assess its role as a potent modulator of disease severity in a wide variety of genetic disorders.

    Selected Publications View all on PubMed

    "The Extracellular Matrix in Homeostatic, Autoimmune and Fibrotic Disease." Northwestern Lectures in Life Sciences, Northwestern University, Chicago, IL, January 2015.

    "Found in Translation: New Insights into the Pathogenesis and Treatment of Marfan Syndrome and Related Disorders." Laennec Clinician/Educator Lecture, American Heart Association Scientific Sessions, Chicago, IL, November 2014.

    "Found in Translation: New Insights into the Pathogenesis and Treatment of Marfan Syndrome and Related Disorders." Stevenson Lecture, University of Western Ontario, London, Ontario, November 2014.

    "Found in Translation: New Insights into the Pathogenesis and Treatment of Marfan Syndrome and Related Disorders." Centro de Biología Molecular "Severo Ochoa" Consejo Superior de Investigaciones Científicas Madrid, Spain, October 2014.

    "Conditional Provocations in a Knock-In-Mouse Model of Marfan Syndrome" (Basic Science Session), " New Therapeutic Opportunities in MFS and LDS as Revealed by Modifier Studies in Patients and Mouse Models." (Mechanisms and Options for Therapeutic Interventions Session), 9th International Research Symposium on Marfan Syndrome and Related Disorders, The Marfan Foundation, Paris (France), September 2014.

    "Marfan & Loeys-Dietz." 5th Annual New England Symposium on Marfan Sydrome & Related Disorders. Ambrey Genetics, Marfan Foundation, MA Chapter and the NH/VT Network of the Marfan Foundation, Manchester, NH, September 2014.

    "Found in Translation: New Insights into the Pathogenesis and Treatment of Marfan Syndrome and Related Disorders." American Heart Association, BCVS Meeting (Basic Cardiovascular Sciences), Las Vegas, NV, July, 2014

    "Found in Translation: New Insights into the Pathogenesis and Treatment of Marfan Syndrome and Related Disorders." Charles R. Ross Memorial, Student Research Celebration, Upstate Medical University, Syracuse, NY, April 2014.

    "The Extracellular Matrix in Homeostatic, Autoimmune and Fibrotic Disease Process." Christian J. 2014 Lambertsen Honorary Lecture, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, April 2014.

    "The Extracellular Matrix in Homeostatic, Autoimmune and Fibrotic Disease Processes." Robert Berliner Professorship, Yale University, New Haven, CT, April 2014.

    "New Insights Regarding the Pathogenesis and Modification of Marfan Syndrome and Related Disorders." 78th Annual Scientific Meeting of the Japanese Circulation Society, Tokyo, Japan, March 2014.

  • Academic Affiliations & Courses +

    Graduate Program Affiliation

    Preceptor, Predoctoral Research Training Programs

    • Human Genetics
    • Biochemistry, Cellular & Molecular Biology
    • Cellular and Molecular Medicine

    Fellowship Training

    • Division of Pediatric Cardiology
    • Division of Pulmonary and Critical Care Medicine
    • Department of Surgery

    Courses and Syllabi

    Molecules & Cells (Course Lecturer)

    Mutation in Human Disease

    Marfan Syndrome, Clinical Correlations

  • Activities & Honors +

    Honors

    • Alpha Omega Alpha, SUNY Upstate School of Medicine, 1983
    • Summa Cum Laude, SUNY Upstate School of Medicine, 1984
    • Pediatric Chief Resident, 1988
    • Harriet Lane Research Fellowship, Department of Pediatrics, 1990
    • First Richard Starr Ross Research Scholar, Johns Hopkins Medical Institutions, 1992
    • Richard D. Rowe Award, Society for Pediatric Research, 1992
    • Editorial Board, Genomics, 1993
    • Patrick John Niland Memorial Lecture, University of Michigan, 1993
    • Professional Advisory Board, National Marfan Foundation, 1993
    • Young Investigator Award, Society for Pediatric Research, 1993
    • Antoine Marfan Award, National Marfan Foundation, 1994
    • Visiting Professorship, Northwestern University School of Medicine, 1995
    • Visiting Professorship, Cleveland Clinic, 1996
    • Winner Memorial Lecture, Univ of Maryland School of Medicine, 1997
    • Appointed to the Howard Hughes Medical Institute, 1997
    • Department of Energy BER 50th Anniversary Celebration, National Academy of Sciences, 1997
    • Editorial Board, Human Molecular Genetics, 1997
    • Elster Memorial Lecture, Mount Sinai University School of Medicine, 1997
    • Visiting Professorship and Memorial Lecture, Hospital for Sick Children, 1997
    • Chair, Professional Advisory Board, National Marfan Foundation, 1998
    • Editorial Board, Circulation Research, 1999
    • Director, William S. Smilow Center for Marfan Syndrome Research, 2000
    • Editorial Board, Pediatric Research, 2001
    • Pauline Wilson Horner Lecture, Case Western Reserve University, 2001
    • Chair, Scientific Committee, Agarini Foundation , 2002
    • Editorial Board, American Journal of Human Genetics, 2002
    • Inductee, Society for Pediatric Research, 2002
    • Inductee, American Society for Clinical Investigation, 2003
    • Board of Scientific Counselors, National Human Genome Research Institute, 2004
    • First Victor A. McKusick Professor of Medicine and Genetic, 2004
    • Editorial Board, American Journal of Medical Genetics, 2005
    • Elected to the Board, American Society for Clinical Investigation, 2005
    • Inductee, American Association for the Advancement of Science, 2005
    • Wilson Lecture, Case Western Reserve University, 2005
    • Cazden Lecture, Oregon health Sciences University, 2006
    • Meinhard Robinow Lecture, Dayton Children's Hospital, 2006
    • Nahum J. Winer Memorial Lecture, New York Academy of Medicine, 2006
    • Recipient of the Curt Stern Award, American Society of Human Genetics, 2006
    • Thomas P. Graham Jr. Lecture, Vanderbilt University, 2006
    • Chair, Board of Scientific Counselors, National Human Genome Research Institute, 2007
    • Congressional Biomedical Research Caucus Symposium, Joint Steering Committee on Public Policy, 2007
    • Editorial Board, Clinical and Translational Science, 2007
    • Edmund R. McCluskey Memorial Lectureship, University of Pittsburgh, 2007
    • Elected to the Board, American Society of Human Genetics, 2007
    • Isadore Rosenfeld Visiting Professorship, Weil Medical College of Cornell University, 2007
    • Kathryn L. Ober Visiting Professor, Indiana University, 2007
    • Paulette Shirley Pritchett Endowed Lecture in Pathology, University of Alabama at Birmingham, 2007
    • Professorship, Princess Lilian Foundation , 2007
    • Robert M. Jeresaty Lecture, St. Francis Medical Center, 2007
    • Scientific Advisory Board, Center for Preterm Birth Research at Washington University in St. Louis, 2007
    • Whitehead Symposium XXV, Massachusetts Institute of Technology, 2007
    • Hero with a Heart Honoree, National Marfan Foundation , 2008
    • Humanitarian Award, American Skin Association, 2008
    • Inductee, National Academy of Sciences Institute of Medicine, 2008
    • George E. Brown Memorial Lecturer Award, American Heart Association, 2009
    • Heritage Award, Johns Hopkins University, 2009
    • Art of Listening Award, Genetic Alliance, 2010
    • Niccolo Paganini Prize, SIMA Congreso Internacional de Síndrome de Marfan, 2010
    • Inductee, The National Academy of Sciences, 2011
    • Pruzansky Lecture (Annual Clinical Genetics Meeting) - ACMG, 2011
    • Colonel Harland Sanders Lifetime Achievement Award in Medical Genetics, 2012
    • March of Dimes and American College of Medical Genetics, 2012
    • Winner - Taubman Prize for Excellence in Translation Medical Science, 2012
    • Inductee, Association of American Physician, 2013
    • Winner – Pasarow Award in Cardiovascular Research, 2013
    • Clinical Cardiology Laennec Clinician/Educator Lecture Award, 2014
    • Winner – Harrington Prize, American Society for Clinical Investigation and the Harrington Institute, 2014
    • Winner – InBev, Baillet Latour Health Prize, 2014

    Memberships

    • American Society for Clinical Investigation
    • American Association for the Advancement of Science, Fellow
    • American Heart Association, Fellow
    • The American Society of Human Genetics
    • Association of American Physicians
    • National Academy of Sciences
    • Charter Fellow, Council on Basic Cardiovascular Sciences

    Professional Activities

    • Director, Professional Advisory Board National Marfan Foundation
    • Howard Hughes Medical Institute
    • Board of Governors, National Human Genome Research Institute
    • Editorial Boards, Genomics
    • Editorial Board, American Journal of Medical Genetics
    • Editorial Board, Human Molecular Genetics
    • Editorial Board, Pediatric Research
    • Editorial Board, Circulation Research
    • Editorial Board, American Journal of Human Genetics
    • Editorial Board, Clinical and Translational Science
    • Editorial Board, eLife
    • Editorial Board, Science Translation Medicine
    • Professional Advisory Board, National Marfan Foundation, 1997
    • Induced Mutant Repository Advisory Board, Jackson Laboratories
    • Member, Review Committee, Young Investigator's Day Award
    • Member, Research Prize Committee, David Israel Macht Memorial
    • Member, Michael A. Sarnoff Research Award Committee
    • Member, Human Genetics Selection Committee
    • Member, Medical Scientist Training Program Steering Committee (M.D./Ph.D.)
    • Associate Director, Human Genetics Predoctoral Training Program
    • The Genome Action Coalition, American Society of Human Genetics Representative
    • Scientific Committee, Agarini Foundation
    • Recruitment Committee, McKusick-Nathans Institute of Genetic Medicine
    • Evaluation Committee for Specialized Centers of Research (SCOR) Program in Cardiovascular Disease, National Institutes of Health Division of Heart and Vascular Diseases
    • Member, Mammalian Genetics Study Section, National Institutes of Health, 2002 - 2005
    • Chairman Recruitment Committee, Department of Molecular Biology and Genetics
    • Chair, Board of Scientific Counselors, National Human Genome Research Institute
    • Member, Advisory Board, Gene Vector Core
    • Director, William S. Smilow Center for Marfan Syndrome Research
    • Member, Professional Advisory Board, Ehlers-Danlos Syndrome National Foundation Institute of Medicine (IOM) Committee on Accelerating Research and Development for Rare Diseases and Orphan Products
    • Chair, National of Arthritis and Musculoskeletal and Skin Diseases Advisory Council of the National Institutes of Health (NIAMS)
    • Medical Advisory Council, Global Fibrosis Foundation
    • Editor, Science Translation Medicine
    • Nominating Committee, American Society of Human Genetics
    • The Research Committee of the Cincinnati Medical Center Board of Trustees
    • Member, Search and Screen Committee, Medical Physiology and Metabolism Section, National Academy of Sciences
    • Member, National Organization for Rare Disorders (NORD) Scientific Advisory Committee
    • President, The American Society of Human Genetics
    • Directorship Search Committee, McKusick-Nathans Institute of Genetic Medicine, 1999 - 2000
    • American Society of Human Genetics Nomination Committee, 1996 - 1999
    • Ad hoc reviewer, American Journal of Cardiology
    • Ad hoc reviewer, American Journal of Human Genetics
    • Ad hoc reviewer, American Journal of Medical Genetics
    • Ad hoc reviewer, Archives of Pediatric and Adolescent Medicine
    • Ad hoc reviewer, Cell
    • Ad hoc reviewer, Circulation
    • Ad hoc reviewer, Clinical Genetics
    • Ad hoc reviewer, Developmental Dynamics
    • Ad hoc reviewer, European Journal of Human Genetics
    • Ad hoc reviewer, Human Molecular Genetics
    • Ad hoc reviewer, Human Mutation
    • Ad hoc reviewer, Journal of Clinical Investigation
    • Ad hoc reviewer, Nature Genetics
    • Ad hoc reviewer, Nature Medicine
    • Ad hoc reviewer, New England Journal of Medicine
    • Ad hoc reviewer, Nucleic Acids Research
    • Ad hoc reviewer, Proceedings of the National Academy of Sciences (USA)
    • Ad hoc reviewer, RNA
    • Ad hoc reviewer, Science
    • Heart Disease in the Young, American Heart Association Study Section, 1996 - 1999
  • Videos & Media +

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    “The Future of Ehlers-Danlos Syndrome”

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