Pediatric card Dietz wins $100,000 honor

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Harry C. Dietz, MD, a professor of pediatric cardiology and genetics at Johns Hopkins University School of Medicine in Baltimore, has received a $100,000 research grant from the University of Michigan's A. Alfred Taubman Medical Research Institute in Ann Arbor.

The Taubman Prize was established to annually recognize clinician-researchers who strive to transform laboratory discoveries into clinical applications for patients suffering from disease.

Dietz’s research interests include aortic aneurysm and related tissue disorders, with a focus on the development and homeostasis of the arterial wall. His research team’s overall goal is to understand genetic factors that predispose people to aortic aneurysm, a condition that accounts for 1 percent to 2 percent of deaths in industrialized countries.

His lab is the first to determine that some defects in the human body's connective tissue can be modified with medication. The discovery overturned decades of conventional wisdom and has implications for the treatment of genetic connective tissue disorders.

He began his research career studying Marfan syndrome, a genetic disease that includes aortic aneurysm as part of the condition and that is caused by mutations in a single gene. Working with his Johns Hopkins colleague, Bart Loeys, MD, PhD, Dietz identified a novel and aggressive aneurysm phenotype, Loeys-Dietz syndrome.

In studying Marfan syndrome and related disorders at the molecular level, Dietz and his team have found that the lack of a certain connective-tissue protein adversely affects the development of various body tissues. They also made the key discovery that this condition is responsive to medication. Up until then, physicians had believed that manifestations of these disorders would be difficult or impossible to modify.

Dietz's research has led to a clinical trial using the blood pressure drug losartan (Cozaar, Merck) in Marfan syndrome patients at risk for aortic aneurysm, which currently is taking place at 20 sites in the U.S.

As part of the inaugural Taubman award, Dietz will appear as keynote speaker Oct. 11 at the Taubman Institute's annual symposium in Ann Arbor. The Taubman Institute supports the research of 18 doctor-scientists at the University of Michigan on diseases such as stroke, cardiovascular disease, diabetes and obesity, among others.