Victor McKusick Professor of Medicine and Genetics, Department of Pediatrics, Johns Hopkins University School of Medicine; Investigator, Howard Hughes Medical Institute
Medical Genetics
Dr. Harry C. Dietz III, MD
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Harry Dietz is an expert in the field of Medical Genetics. He studied medicine at State University of New York Upstate Medical University. He received 4 awards: "Elected Member", "CMS Meaningful Use Stage 1 Certification", "Whos Who in Medicine and Healthcare" and "Investigator". Harry Dietz is a published physician. He published 98 publications, including: 'Mitral valve disease-morphology and mechanisms.'
Publications
- Mitral valve disease-morphology and mechanisms.
- High prevalence of cervical deformity and instability requires surveillance in loeys-dietz syndrome.
- Atenolol versus losartan in children and young adults with Marfan's syndrome.
- Histone deacetylase inhibition rescues structural and functional brain deficits in a mouse model of Kabuki syndrome.
- Mutations in SGOL1 cause a novel cohesinopathy affecting heart and gut rhythm.
- CT Angiographic Evaluation of Genetic Vascular Disease: Role in Detection, Staging, and Management of Complex Vascular Pathologic Conditions.
- Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-β expression and connective tissue features.
- Loeys-Dietz syndrome: a primer for diagnosis and management.
- Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma.
- Mitral valve disease in patients with marfan syndrome undergoing aortic root replacement.
- A Pkd1-Fbn1 Genetic Interaction Implicates TGF-β Signaling in the Pathogenesis of Vascular Complications in Autosomal Dominant Polycystic Kidney Disease.
- Increased fracture risk and low bone mineral density in patients with loeys-dietz syndrome.
- Genotype-phenotype correlation in patients with bicuspid aortic valve and aneurysm.
- TGFβ Receptor Mutations Impose a Strong Predisposition for Human Allergic Disease.
- LMNA-Associated Cardiocutaneous Progeria: An Inherited Autosomal Dominant Premature Aging Syndrome With Late Onset.
- Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy.
- GenTAC registry report: Gender differences among individuals with genetically triggered thoracic aortic aneurysm and dissection.
- Tetralogy of fallot and aortic root dilation: a long-term outlook.
- Mutations in the TGF-β repressor SKI cause Shprintzen-Goldberg syndrome with aortic aneurysm.
- Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm.
- Matrix-dependent perturbation of TGFβ signaling and disease.
- Angiotensin receptor blockade attenuates cigarette smoke-induced lung injury and rescues lung architecture in mice.
- Altered tissue behavior of a non-aneurysmal descending thoracic aorta in the mouse model of Marfan syndrome.
- The National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC): Results from phase I and scientific opportunities in p...
- Novel MYH11 and ACTA2 mutations reveal a role for enhanced TGF? signaling in FTAAD.
- Pravastatin reduces marfan aortic dilation.
- Mitral Valve Disease in Marfan Syndrome and Related Disorders.
- Perturbation of thymocyte development in nonsense-mediated decay (NMD)-deficient mice.
- Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism.
- Noncanonical TGFβ signaling contributes to aortic aneurysm progression in Marfan syndrome mice.
- Causes and histopathology of ascending aortic disease in children and young adults.
- New therapeutic approaches to mendelian disorders.
- Musculoskeletal findings of Loeys-Dietz syndrome.
- Improving clinical recognition of Marfan syndrome.
- Microfibril structure masks fibrillin-2 in postnatal tissues.
- Murine model of surgically induced acute aortic dissection type A.
- TGF-beta in the pathogenesis and prevention of disease: a matter of aneurysmic proportions.
- Multi-gram scale synthesis of FR180204.
- Inhibition of nonsense-mediated mRNA decay by the natural product pateamine A through eukaryotic initiation factor 4AIII.
- Growing rods for infantile scoliosis in Marfan syndrome.
- Extracellular microfibrils in vertebrate development and disease processes.
- Dual functions for LTBP in lung development: LTBP-4 independently modulates elastogenesis and TGF-beta activity.
- Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: analysis of early outcome.
- p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice.
- Histopathologic findings in ascending aortas from individuals with Loeys-Dietz syndrome (LDS).
- Molecular genetic and biochemical analyses of FGF23 mutations in familial tumoral calcinosis.
- Krit1 modulates beta 1-integrin-mediated endothelial cell proliferation.
- Targeted disruption of NeuroD, a proneural basic helix-loop-helix factor, impairs distal lung formation and neuroendocrine morphology in the neonatal lung.
- Angiotensin II blockade and aortic-root dilation in Marfan's syndrome.
- Synergistic heterozygosity for TGFbeta1 SNPs and BMPR2 mutations modulates the age at diagnosis and penetrance of familial pulmonary arterial hypertension.
- Recent advances in understanding Marfan syndrome: should we now treat surgical patients with losartan?
- Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome.
- Therapy of Marfan syndrome.
- Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.
- 2006 Curt Stern Award Address. Marfan syndrome: from molecules to medicines.
- Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance.
- Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in t...
- Loeys-Dietz syndrome: MDCT angiography findings.
- Manipulating mitotic recombination in the zebrafish embryo through RecQ helicases.
- Progressive dilation of the ascending aorta in children with isolated bicuspid aortic valve.
- Perturbations of vascular homeostasis and aortic valve abnormalities in fibulin-4 deficient mice.
- Symposium on the musculoskeletal aspects of Marfan syndrome: meeting report and state of the science.
- Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states.
- Interaction between krit1 and malcavernin: implications for the pathogenesis of cerebral cavernous malformations.
- Structural and functional genetic disorders of the great vessels and outflow tracts.
- Induction of macrophage chemotaxis by aortic extracts of the mgR Marfan mouse model and a GxxPG-containing fibrillin-1 fragment.
- Aneurysm syndromes caused by mutations in the TGF-beta receptor.
- DSG2 mutations contribute to arrhythmogenic right ventricular dysplasia/cardiomyopathy.
- Nuclear mRNA degradation pathway(s) are implicated in Xist regulation and X chromosome inactivation.
- Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome.
- Mutations in the facilitative glucose transporter GLUT10 alter angiogenesis and cause arterial tortuosity syndrome.
- Protrusio acetabuli in Marfan syndrome: age-related prevalence and associated hip function.
- Recent progress towards a molecular understanding of Marfan syndrome.
- Control of liver cell fate decision by a gradient of TGF beta signaling modulated by Onecut transcription factors.
- Identification of candidate regions for familial idiopathic scoliosis.
- Characterization of the symptoms associated with dural ectasia in the Marfan patient.
- Treatment of aortic disease in patients with Marfan syndrome.
- Association between a functional variant of the KLOTHO gene and high-density lipoprotein cholesterol, blood pressure, stroke, and longevity.
- A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2.
- Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome.
- Toward an understanding of dural ectasia: a light microscopy study in a murine model of Marfan syndrome.
- TGF-beta-dependent pathogenesis of mitral valve prolapse in a mouse model of Marfan syndrome.
- Fibrillin microfibrils: multipurpose extracellular networks in organismal physiology.
- Klotho gene variation and expression in 20 inbred mouse strains.
- Nonsense surveillance regulates expression of diverse classes of mammalian transcripts and mutes genomic noise.
- Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome.
- Surgery for aortic root aneurysm in children: a 21-year experience in 50 patients.
- KLOTHO allele status and the risk of early-onset occult coronary artery disease.
- Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome.
- Separable roles for rent1/hUpf1 in altered splicing and decay of nonsense transcripts.
- Report of a child with aortic aneurysm, orofacial clefting, hemangioma, upper sternal defect, and marfanoid features: possible PHACE syndrome.
- Growth and maturation in Marfan syndrome.
- Exosome-mediated recognition and degradation of mRNAs lacking a termination codon.
- An mRNA surveillance mechanism that eliminates transcripts lacking termination codons.
- Aortic root replacement in 271 Marfan patients: a 24-year experience.
- Association of human aging with a functional variant of klotho.
- The Genetic Basis of Aortic Aneurysm.
Schools
Upstate Medical University College of Health Professions
Johns Hopkins Hospital
Doctors Specialties
- Pediatrics
- Medical Genetics
Accepted Insurances
Awards
- Elected Member
- CMS Meaningful Use Stage 1 Certification
- Whos Who in Medicine and Healthcare
- Investigator
Education
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Johns Hopkins University -
State University of New York Upstate Medical University
Hospital
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Johns Hopkins Hospital
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