Marfan Syndrome (Marfan's Syndrome)

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765 relevant articles (11 outcomes, 42 trials/studies) found for this Disease

Description: An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs, and enlarged DURA MATER (dural ectasia). Marfan syndrome is due to mutations of gene encoding FIBRILLIN, a major element of extracellular microfibrils of connective tissue.

Also Known As:

Marfan's Syndrome; Marfans Syndrome; Syndrome, Marfan; Syndrome, Marfan's

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Aneurysm (Aneurysms)
2.	Aortic Aneurysm (Aneurysm, Aortic)
3.	Rupture
4.	Thoracic Aortic Aneurysm
5.	Chest Pain (Chest Pains)

Experts

1.	Dietz, Harry C: 8 articles (06/2008 - 03/2003)
2.	Judge, Daniel P: 4 articles (06/2008 - 12/2004)
3.	Ramirez, Francesco: 3 articles (01/2008 - 03/2003)
4.	Haverich, Axel: 3 articles (12/2005 - 11/2002)
5.	Karck, Matthias: 3 articles (12/2005 - 11/2002)
6.	Habashi, Jennifer P: 2 articles (06/2008 - 04/2006)
7.	Chung, Ada W Y: 2 articles (04/2008 - 08/2007)
8.	van Breemen, Cornelis: 2 articles (04/2008 - 08/2007)
9.	Carrel, Thierry: 2 articles (02/2008 - 08/2007)
10.	Huang, Yi: 2 articles (08/2007 - 12/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Marfan Syndrome:

1.	fibrillinIBA 11/01/2006 - "This review discusses mutant-fibrillin mouse models of Marfan syndrome and SSc (Tsk mice), and studies suggesting that alterations in microfibrils might contribute to human SSc" 08/01/2004 - "In order to estimate the contribution of mutations at the fibrillin-1 locus (FBN1) to classical Marfan syndrome (MFS) and to study possible phenotypic differences between patients with an FBN1 mutation vs. without, a comprehensive molecular study of the FBN1 gene in a cohort of 93 MFS patients fulfilling the clinical diagnosis of MFS according to the Ghent nosology was performed" 01/01/2003 - "This study strongly suggests that AD WMS and Marfan syndrome are allelic conditions at the fibrillin-1 locus and adds to the remarkable clinical heterogeneity of type I fibrillinopathies." 11/12/2001 - "Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome." 01/01/1998 - "A comparative histologic study of the fibrillin microfibrillar system in the lens capsule of normal subjects and subjects with Marfan syndrome." Order ALL the reference details at left...
2.	Transforming Growth Factor beta (TGF-beta)IBA 07/01/2008 - "Targeting TGF-beta and the extracellular matrix in Marfan's syndrome." 02/01/2008 - "RESULTS: It seems that many manifestations of Marfan syndrome are less related to a primary structural deficiency of the tissues than to altered morphogenetic and homeostatic programs that are induced by altered transforming growth factor-beta signaling" 01/29/2007 - "Altered TGFbeta signaling is a major contributor to the pathology of Marfan syndrome (MFS) and related diseases" 01/01/2007 - "LRP1 functions as an atheroprotective integrator of TGFbeta and PDFG signals in the vascular wall: implications for Marfan syndrome." 03/01/2003 - "Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome." Order ALL the reference details at left...
3.	DNA (Deoxyribonucleic Acid)IBA 10/01/2000 - "In this study, we performed segregation analysis of allelic DNA polymorphisms to support diagnosis of Marfan syndrome" 11/01/1996 - "The in vitro fertilization technology coupled with the ability to amplify DNA from a single cell has been used for the preimplantation genetic diagnosis of Marfan syndrome" 07/21/1994 - "These results document the usefulness of novel polymorphic DNA repeat sequences in the presymptomatic diagnosis of Marfan's syndrome" 01/01/1994 - "DNA diagnostics of the Marfan syndrome: application of amplifiable polymorphic markers." 09/01/1991 - "This information will provide the basis for the DNA diagnostics of Marfan syndrome in the future." Order ALL the reference details at left...
4.	Atenolol (Tenormin)FDA LinkGeneric 10/01/2006 - "METHODS: In 46 patients with Marfan syndrome (age, 17.4 +/- 11.1 years) and 46 healthy control subjects, ascending and descending aortic elastic parameters were determined noninvasively before and after 39 +/- 16 months of beta-blocker treatment with atenolol" 06/01/1999 - "CONCLUSIONS: There was a heterogeneous response in the aortic root elastic properties after long-term treatment with atenolol in asymptomatic patients with Marfan syndrome" 03/01/1995 - "The negative inotropic and chronotropic effects of beta-blocker therapy have been reported to reduce morbidity and mortality in patients with Marfan syndrome; however, little is known about the pharmacokinetics of atenolol after oral administration of multiple doses to patients with the Marfan syndrome" 03/01/1995 - "Absorption pharmacokinetics of atenolol in patients with the Marfan syndrome." 03/01/1993 - "Aortic biomechanical properties in pediatric patients with the Marfan syndrome, and the effects of atenolol." Order ALL the reference details at left...
5.	Polyethylene Terephthalates (Polyethylene Terephthalate)IBA 10/01/2004 - "David-V technique, we describe a patient with Marfan's syndrome who underwent valve-sparing aortic root replacement with 1 large and 1 small graft to create pseudosinuses in the Dacron graft, to facilitate suturing the valve inside the graft, and to make the distal graft-to-aorta anastomosis a better size match." 06/01/2004 - "METHODS: By using a time-resolved, 3-dimensional, phase-contrast magnetic resonance imaging technique, aortic root and aortic blood velocity data were acquired from 2 patients with Marfan syndrome 6 months after aortic valve-sparing surgery with straight Dacron grafts and contrasted with data from 6 normal volunteers" 10/01/1995 - "We describe a case of tight stenosis at the left coronary ostial anastomosis in a 38-year-old woman with Marfan's syndrome who had undergone a composite graft replacement that employed separate Dacron coronary grafts." 05/01/1983 - "A fatal thrombosis originating at the junction of an aortic bioprosthetic heart valve and a Dacron aortic graft was observed in a patient with the Marfan syndrome, who had had replacement of the ascending aorta with a valved conduit 29 months previously" 01/01/2005 - "Most of the improvement in life expectancy that has been achieved in Marfan syndrome is attributable to early recognition of aortic root aneurysms and prophylactic replacement with composite grafts (mechanical valve prostheses within Dacron conduits) before rupture or dissection occurs" Order ALL the reference details at left...
6.	Matrix Metalloproteinase 2 (Gelatinase A)IBA 08/31/2007 - "Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome." 04/25/2008 - "Long-term doxycycline is more effective than atenolol to prevent thoracic aortic aneurysm in marfan syndrome through the inhibition of matrix metalloproteinase-2 and -9." Order ALL the reference details at left...
7.	RNA (Ribonucleic Acid)IBA 09/01/1998