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Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial
Conclusion In adult Marfan patients, losartan treatment reduces aortic root dilatation rate. After aortic root replacement, losartan treatment reduces dilatation rate of the aortic arch.
Source: European Heart Journal - December 1, 2013 Category: Cardiology Authors: Groenink, M., den Hartog, A. W., Franken, R., Radonic, T., de Waard, V., Timmermans, J., Scholte, A. J., van den Berg, M. P., Spijkerboer, A. M., Marquering, H. A., Zwinderman, A. H., Mulder, B. J. M. Tags: Congenital heart disease Source Type: research

Angiotensin receptor blockers: a panacea for Marfan syndrome and related disorders?
Abstract The study of mouse models for Marfan syndrome, an autosomal dominant connective tissue disorder caused by mutations in fibrillin-1 (FBN1), has shifted our understanding of the pathogenesis of thoracic aortic aneurysm significantly. Multiple lines of evidence support the notion that dysregulation of canonical and noncanonical transforming growth factor (TGF)β signaling is the responsible pathway in this and related thoracic aortic aneurysm conditions. This exciting knowledge has opened numerous new treatment options, including antagonism of the angiotensin II receptor blocker type 1 (AT1R). In this review...
Source: Drug Discovery Today - October 2, 2014 Category: Drugs & Pharmacology Authors: Loeys BL Tags: Drug Discov Today Source Type: research

Natural Course of Isolated Spontaneous Coronary Artery Dissection in Marfan Syndrome
Spontaneous coronary artery dissection (SCAD) is a rare disease resulting in acute coronary syndrome or sudden death 1. Connective tissue disorder is one of the suspected etiologies in SCAD, as pathological studies have suggested a relationship between SCAD and Marfan syndrome 2-3. Isolated SCAD with Marfan syndrome is nonetheless very rare, and few case reports diagnosed by angiography alone are available 1. To the best of our knowledge, no case reports on SCAD with Marfan syndrome have presented detailed information or described the long-term course 4-7.
Source: International Journal of Cardiology - September 25, 2014 Category: Cardiology Authors: Chisato Sato, Kohei Wakabayashi, Hiroshi Suzuki Tags: Letters to the Editor Source Type: research

Marfan syndrome: An eyesight of syndrome
Publication date: December 2014 Source:Meta Gene, Volume 2 Author(s): Ashok Kumar , Sarita Agarwal Marfan syndrome (MFS), a relatively common autosomal dominant hereditary disorder of connective tissue with prominent manifestations in the skeletal, ocular, and cardiovascular systems, is caused by mutations in the glycoprotein gene fibrillin-1 (FBN1). Aortic root dilation and mitral valve prolapse are the main presentations among the cardiovascular malformations of MFS. The revised Ghent diagnostics nosology of Marfan syndrome is established in accordance with a combination of major and minor clinical manifestations in va...
Source: Meta Gene - November 4, 2014 Category: Genetics & Stem Cells Source Type: research

Design and rationale of a prospective, collaborative meta-analysis of all randomized controlled trials of angiotensin receptor antagonists in Marfan syndrome, based on individual patient data: a report from the Marfan Treatment Trialists’ Collaboration
Publication date: Available online 12 February 2015 Source:American Heart Journal Author(s): Alex Pitcher , Jonathan Emberson , Ronald V. Lacro , Lynn A. Sleeper , Mario Stylianou , Lynn Mahony , Gail D. Pearson , Maarten Groenink , Barbara J. Mulder , Aeilko H. Zwinderman , Julie De Backer , Anne M. De Paepe , Eloisa Arbustini , Guliz Erdem , Xu Yu Jin , Marcus D. Flather , Michael J. Mullen , Anne H. Child , Alberto Forteza , Arturo Evangelista , Hsin-Hui Chiu , Mei-Hwan Wu , George Sandor , Ami B. Bhatt , Mark A. Creager , Richard B. Devereux , Bart Loeys , J. Colin Forfar , Stefan Neubauer , Hugh Watkins , Catherine ...
Source: American Heart Journal - February 14, 2015 Category: Cardiology Source Type: research

A deleterious gene-by-environment interaction imposed by calcium channel blockers in Marfan syndrome
Calcium channel blockers (CCBs) are prescribed to patients with Marfan syndrome for prophylaxis against aortic aneurysm progression, despite limited evidence for their efficacy and safety in the disorder. Unexpectedly, Marfan mice treated with CCBs show accelerated aneurysm expansion, rupture, and premature lethality. This effect is both extracellular signal-regulated kinase (ERK1/2) dependent and angiotensin-II type 1 receptor (AT1R) dependent. We have identified protein kinase C beta (PKCβ) as a critical mediator of this pathway and demonstrate that the PKCβ inhibitor enzastaurin, and the clinically available a...
Source: eLife - October 27, 2015 Category: Biomedical Science Authors: Doyle, J. J., Doyle, A. J., Wilson, N. K., Habashi, J. P., Bedja, D., Whitworth, R. E., Lindsay, M. E., Schoenhoff, F., Myers, L., Huso, N., Bachir, S., Squires, O., Rusholme, B., Ehsan, H., Huso, D., Thomas, C. J., Caulfield, M. J., Van Eyk, J. E., Judge Tags: Human biology and medicine Source Type: research

Mitral valve prolapse syndrome and MASS phenotype: Stability of aortic dilatation but progression of mitral valve prolapse
Conclusions The study provides evidence that MVPS and MASS are Marfan-like syndromes with stability of aortic dilatation but with progression of mitral valve prolapse. Echocardiographic characteristics of mitral valve disease rather than the type of syndrome, predict clinical progression of mitral valve prolapse.
Source: IJC Heart and Vasculature - January 21, 2016 Category: Cardiology Source Type: research

Genetic testing of the FBN1 gene in Chinese patients with Marfan/Marfan-like syndrome
Publication date: 1 August 2016 Source:Clinica Chimica Acta, Volume 459 Author(s): Hang Yang, Mingyao Luo, Qianlong Chen, Yuanyuan Fu, Jing Zhang, Xiangyang Qian, Xiaogang Sun, Yuxin Fan, Zhou Zhou, Qian Chang Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder typically involving the ocular, skeletal and cardiovascular systems, and aortic aneurysms/dissection mainly contributes to its mortality. Here, we performed genetic testing of the FBN1 gene in 39 Chinese probands with Marfan/Marfan-like syndrome and their related family members by Sanger sequencing. In total, 29 pathogenic/likely pa...
Source: Clinica Chimica Acta - May 30, 2016 Category: Laboratory Medicine Source Type: research

Pupillary manifestations of Marfan syndrome: from the Marfan eye consortium of Chicago.
CONCLUSIONS: Our data highlight pupillary parameters within a population of MFS patients under scotopic conditions. Constriction and dilation velocities were slower in the pediatric MFS patients compared to age- and gender-matched controls, and dilation velocities were slower in the adult MFS patients compared to age-matched controls. These findings, for the first time, quantitatively demonstrated differences in pupillary function in patients with MFS. PMID: 29336629 [PubMed - as supplied by publisher]
Source: Ophthalmic Genetics - January 18, 2018 Category: Opthalmology Tags: Ophthalmic Genet Source Type: research

Left subclavian artery dissection associated with connective tissue abnormalities resembling Marfan-like syndrome in an English bulldog
Publication date: Available online 5 March 2018 Source:Journal of Veterinary Cardiology Author(s): Ilaria Biasato, Renato Zanatta, Lorella Maniscalco, Rocchina Evangelista, Bryan Iotti, Selina Iussich The unexpected demise of a 12-year-old male neutered English bulldog solicited a gross examination, which revealed a blood-filled space occurring in the proximal left subclavian artery (LSA). It originated about 1 cm from the branching point of the vessel and progressively dilated for 3 cm distal to this origin. Histopathological investigation showed that the tunica media of the LSA was more than 50% split, with the blood-...
Source: Journal of Veterinary Cardiology - March 6, 2018 Category: Veterinary Research Source Type: research

Targeted deletion of fibrillin-1 in the mouse eye results in ectopia lentis and other ocular phenotypes associated with Marfan syndrome RESEARCH ARTICLE
ABSTRACT Fibrillin is an evolutionarily ancient protein that lends elasticity and resiliency to a variety of tissues. In humans, mutations in fibrillin-1 cause Marfan and related syndromes, conditions in which the eye is often severely affected. To gain insights into the ocular sequelae of Marfan syndrome, we targeted Fbn1 in mouse lens or non-pigmented ciliary epithelium (NPCE). Conditional knockout of Fbn1 in NPCE, but not lens, profoundly affected the ciliary zonule, the system of fibrillin-rich fibers that centers the lens in the eye. The tensile strength of the fibrillin-depleted zonule was reduced substantially, due ...
Source: DMM Disease Models and Mechanisms - January 25, 2019 Category: Biomedical Science Authors: Jones, W., Rodriguez, J., Bassnett, S. Tags: RESEARCH ARTICLE Source Type: research

Sleep disordered breathing in Marfan syndrome: Value of standard screening questionnaires
ConclusionA survey of SDB in a sample of persons with MFS reveals not only a high burden of SDB but also that conventional screening instruments have utility if adapted appropriately. Future studies should validate the utility of these screening tools given concerns that SDB may contribute to progression of aortic pathology in MFS.
Source: Molecular Genetics & Genomic Medicine - November 9, 2019 Category: Genetics & Stem Cells Authors: Mudiaga O. Sowho, Susheel Patil, Hartmut Schneider, Gretchen MacCarrick, Jason P. Kirkness, Lisa F. Wolfe, Laura Sterni, Peter A. Cistulli, Enid R. Neptune Tags: ORIGINAL ARTICLE Source Type: research

Ruptured popliteal artery aneurysm in a patient with a clinical diagnosis of Marfan syndrome
This report presents a case of a ruptured left popliteal artery aneurysm successfully treated in an 82-year-old patient clinically diagnosed with previously unknown Marfan syndrome.
Source: Jornal Vascular Brasileiro - October 16, 2020 Category: Surgery Source Type: research