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Harry C. Dietz
Johns Hopkins University School of Medicine
327Publications
86H-index
30.8kCitations
Publications 327
Newest
#1Jong-Sung Park (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 1
#2Yumin Oh (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 12
Last.Hyoung-Tae An (JHUSOM: Johns Hopkins University School of Medicine)
view all 10 authors...
Scleroderma is an autoimmune rheumatic disorder accompanied by severe fibrosis in skin and other internal organs. During scleroderma progression, resident fibroblasts undergo activation and convert to α-smooth muscle actin (α-SMA) expressing myofibroblasts (MFBs) with increased capacity to synthesize collagens and fibrogenic components. Accordingly, MFBs are a major therapeutic target for fibrosis in scleroderma and treatment with blocking MFBs could produce anti-fibrotic effects. TLY012 is an e...
Abstract Objectives We sought to develop strategies for management of the aortic arch in patients with Loeys-Dietz syndrome (LDS) through a review of our clinical experience with these patients and a comparison with our experience in Marfan syndrome patients (MFS). Methods We reviewed hospital and follow-up records of 79 LDS patients and compared them with 256 Marfan syndrome patients who served as reference controls. Results In the LDS group, 16% of patients presented initially with acute aorti...
#1Aude Beyens (UGent: Ghent University)H-Index: 1
#2Juliette Albuisson (University of Paris)H-Index: 3
Last.Tiffany BusaH-Index: 1
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In the published version of this paper the author Neus Baena's name was incorrectly given as Neus Baena Diez. This has now been corrected in both the HTML and PDF versions of the paper.
#1Alexander P. Nissen (University of Texas Health Science Center at Houston)H-Index: 1
#2Van Thi Thanh Truong (University of Texas Health Science Center at Houston)H-Index: 6
Last.Siddharth K. Prakash (University of Texas Health Science Center at Houston)H-Index: 21
view all 7 authors...
Abstract Objective Bicuspid aortic valve (BAV) is a common risk factor for thoracic aortic aneurysm and dissection. Guidelines for elective ascending aortic intervention (AAI) in BAV are derived from limited evidence, and the extent of practice variation due to patient and provider characteristics is unknown. Using data from two large cardiovascular registries (BAVCon and GenTAC), we investigated factors that influence decisions for AAI. Methods All BAV cases with known aortic diameters and surg...
#1David Kim (JHUSOM: Johns Hopkins University School of Medicine)
#2James Daniel Beckett (JHUSOM: Johns Hopkins University School of Medicine)
Last.Jonathan T. Butcher (Cornell University)H-Index: 29
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Fibrosis is a common pathologic outcome of chronic disease resulting in the replacement of normal tissue parenchyma with a collagen-rich extracellular matrix produced by myofibroblasts. Although the progenitor cell types and cellular programs giving rise to myofibroblasts through mesenchymal transition can vary between tissues and diseases, their contribution to fibrosis initiation, maintenance, and progression is thought to be pervasive. Here, we showed that the ability of transforming growth f...
#1Ilse Luyckx (University of Antwerp)H-Index: 4
#2Gretchen MacCarrick (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 3
Last.Natzi SakalihasanH-Index: 28
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Progressive dilatation of the thoracic aorta leads to thoracic aortic aneurysm (TAA), which is often asymptomatic but predisposes to lethal aortic dissections and ruptures. TAA is a common complication in patients with bicuspid aortic valve (BAV). Recently, rare loss-of-function SMAD6 variants were shown to contribute significantly to the genetic aetiology of BAV/TAA. Intriguingly, patients with craniosynostosis have also been reported to be explained molecularly by similar loss-of-function SMAD...
#1Morgan B. Elliott (Johns Hopkins University)H-Index: 2
#2Brian Ginn (Johns Hopkins University)H-Index: 4
Last.Hai-Quan Mao (Johns Hopkins University)H-Index: 48
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Despite significant research efforts, clinical practice for arterial bypass surgery has been stagnant, and engineered grafts continue to face postimplantation challenges. Here, we describe the development and application of a durable small-diameter vascular graft with tailored regenerative capacity. We fabricated small-diameter vascular grafts by electrospinning fibrin tubes and poly(e-caprolactone) fibrous sheaths, which improved suture retention strength and enabled long-term survival. Using s...
#1Joseph Y. Shin (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 1
#2James Daniel Beckett (JHUSOM: Johns Hopkins University School of Medicine)
Last.Michael Beer (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 40
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In systemic sclerosis (SSc), previously healthy adults develop an inflammatory prodrome with subsequent progressive fibrosis of the skin and viscera. SSc has a weak signature for genetic contribution, and there are few pathogenic insights or targeted treatments for this condition. Here, chromatin accessibility and transcriptome profiling coupled with targeted epigenetic editing revealed constitutive activation of a previously unannotated transforming growth factor–β2 ( TGFB2 ) enhancer maintaine...
#1Caitlin J. Bowen (JHUSOM: Johns Hopkins University School of Medicine)
#2Juan Francisco Calderón Giadrosic (JHUSOM: Johns Hopkins University School of Medicine)
Last.Harry C. Dietz (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 86
view all 8 authors...
Vascular Ehlers-Danlos Syndrome (vEDS) is an autosomal-dominant connective tissue disorder caused by heterozygous mutations in the COL3A1 gene. Currently, loss of structural integrity of the extracellular matrix is believed to drive the signs and symptoms of this condition, including spontaneous arterial dissection and/or rupture, the major cause of mortality. Using novel mouse models of vEDS that carry heterozygous Col3a1 glycine substitutions, we show that signaling abnormalities in the PLC/IP...
#1Jennifer Habashi (Johns Hopkins University)H-Index: 2
#2Elena MacFarlane (Johns Hopkins University)H-Index: 1
Last.Graham Rykiel (Johns Hopkins University)H-Index: 1
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Women with Marfan syndrome (MFS) are at high risk for pregnancy-associated aortic dissection. Pathogenic models that singularly invoke hemodynamic stress are difficult to reconcile with predominant postnatal occurrence of aortic tear, often occurring weeks to months after delivery. In consideration of events that peak at term, are sustained after delivery, and might synergize with previously defined signaling pathways implicated in aneurysm progression, we examined the hormone oxytocin, which in...
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