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Total 715932 results found since Jan 2013.

Effects of Age, Sex, and Extracellular Matrix Integrity on Aortic Dilatation and Rupture in a Mouse Model of Marfan Syndrome
CONCLUSIONS: Collagen fiber structure and function in the Marfan aorta are augmented, in part, by increased lysyl oxidase in female and especially male mice, which improves structural integrity, particularly via fibrils in the adventitia. Preserving or promoting collagen cross-linking may represent a therapeutic target for an otherwise vulnerable aorta.PMID:37470181 | DOI:10.1161/ATVBAHA.123.319122
Source: Arteriosclerosis, Thrombosis and Vascular Biology - July 20, 2023 Category: Cardiology Authors: Dar Weiss Bruno V Rego Cristina Cavinato David S Li Yuki Kawamura Nir Emuna Jay D Humphrey Source Type: research

Ten-year clinical and imaging follow-up of dural ectasia in adults with Marfan syndrome
Abstract: Background context: Dural ectasia in the lumbosacral spine is a common feature of Marfan syndrome and is associated with low back pain and surgical complications, but its natural history is unknown.Purpose: To evaluate the natural history of dural ectasia in adults with Marfan syndrome by determining if, over time, symptoms associated with dural ectasia worsen, dural ectasia imaging findings worsen, or spondylolisthesis/spondylolysis develops or worsens.Study design: Prospective cohort study.Patient sample: For our prospective follow-up study, we enrolled 20 patients with Marfan syndrome and dural ectasia who, fr...
Source: The Spine Journal - December 10, 2012 Category: Orthopaedics Authors: Addisu Mesfin, Nicholas U. Ahn, John A. Carrino, Paul D. Sponseller Tags: Clinical Studies Source Type: research

Assessment of aortic root dimensions in patients with suspected Marfan syndrome: Intraindividual comparison of contrast-enhanced and non-contrast magnetic resonance angiography with echocardiography
Abstract: Purpose: Our purpose was to evaluate intraindividually the performance of contrast-enhanced magnetic resonance angiography (MRA) and non-contrast MRA for aortic root diameter measurements and to compare the results with routinely performed echocardiography in patients with suspected Marfan syndrome.Methods and materials: Aortic roots were examined prospectively in 51 consecutive patients with suspected Marfan syndrome by using contrast-enhanced MRA and non-contrast MRA at 1.5T. Two readers independently measured aortic root diameters at the annulus, sinuses of Valsalva and sinutubular junction in both data sets a...
Source: International Journal of Cardiology - January 13, 2012 Category: Cardiology Authors: Peter Bannas, Michael Groth, Meike Rybczynski, Sara Sheikhzadeh, Yskert von Kodolitsch, Joachim Graessner, Gunnar Lund, Gerhard Adam, Christian R. Habermann Tags: Original Articles Source Type: research

Posterior iris-claw aphakic intraocular lens implantation in subluxated lenses due to Marfan syndrome.
Conclusions:</bold> The posterior implantation technique of aphakic iris-claw IOL provided good visual outcomes with a favorable complication rate and can be used as a reasonable alternative in subluxated lenses due to Marfan syndrome without adequate capsular support.</p> PMID: 24057936 [PubMed - as supplied by publisher]
Source: European Journal of Ophthalmology - September 19, 2013 Category: Opthalmology Authors: Gonnermann J, Torun N, Klamann MK, Maier AK, Sonnleithner CV, Bertelmann E Tags: Eur J Ophthalmol Source Type: research

Emergency Cesarean Section Due to Acute Aortic Dissection Type A (Debakey I) without Marfan Syndrome: A Case Report and Review of the Literature
Conclusion: This alarming result of AADA (DeBakey I) in late pregnancy without obvious warnings such as Marfan syndrome illustrates the importance of performing early imaging in similar cases.
Source: The Journal of Emergency Medicine - September 9, 2013 Category: Emergency Medicine Authors: Marc Beirer, Ingo J. Banke, Daniela Münzel, Heiko Wendorff, Nawid Khaladj, Chlodwig Kirchhoff, Stefan Huber-Wagner, Peter Biberthaler Tags: Clinical Communications: OB/GYN Source Type: research

Type A Aortic Dissection in Marfan Syndrome: A Case for More Aggressive and Extensive Surgery at the Time of Initial Surgical Operation.
Abstract In the manuscript by Rylski et al.(1) in this issue of Circulation, the authors investigate the long-term outcomes of repair for Type A aortic dissection in Marfan's patients. Marfan's syndrome (MFS) is one of the genetic syndromes associated with thoracic aortic aneurysms and aortic dissection. The syndrome is the results of fibrillin-1 gene mutations (FBN-1) which is an autosomal dominant mutation with variable expression. Fibrillin 1 is a large glycoprotein that serve as a structural component of calcium-binding microfibrils. These microfibrils are present in elastic connective tissue, such as the medi...
Source: Circulation - March 4, 2014 Category: Cardiology Authors: Byrne JG, Leacche M Tags: Circulation Source Type: research

Surgical management of lens subluxation in Marfan syndrome
Conclusions: Anterior lensectomy and limited vitrectomy with aphakic correction is safe and provides a consistent visual outcome in patients with lens subluxation secondary to Marfan syndrome. This is especially important in pediatric patients, in whom long-term follow-up for iris- and scleral-fixated intraocular lenses is limited.
Source: Journal of AAPOS - April 1, 2014 Category: Opthalmology Authors: Virginia Miraldi Utz, Razek Georges Coussa, Elias I. Traboulsi Tags: Major Articles Source Type: research

Aneurysmal dilatation of the aortic sinuses of Valsalva — beyond Marfan syndrome: a single centre experience and review of the literature
In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less well-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.
Source: Frontiers of Medicine - December 1, 2014 Category: Journals (General) Source Type: research

C596G mutation in FBN1 causes Marfan syndrome with exotropia in a Chinese family.
CONCLUSIONS: A C596G mutation in FBN1 was identified in a Chinese family with MFS. Our results expand the spectrum of FBN1 mutations and contribute to the understanding of the role of FBN1 in the pathogenesis of Marfan syndrome. PMID: 25729264 [PubMed - in process]
Source: Molecular Vision - March 5, 2015 Category: Molecular Biology Tags: Mol Vis Source Type: research

Marfan Syndrome Is Associated With Recurrent Dissection of the Dissected Aorta.
CONCLUSIONS: We conclude that medial degeneration, as seen in the Marfan aorta, represents a predisposing factor for recurrent dissection of the dissected aorta. Our data indicate that double aortic dissections cause significant arterial destabilization and a low threshold for surgical intervention is appropriate. PMID: 25818572 [PubMed - as supplied by publisher]
Source: The Annals of Thoracic Surgery - March 25, 2015 Category: Cardiovascular & Thoracic Surgery Authors: Ptaszek LM, Kim K, Spooner AE, MacGillivray TE, Cambria RP, Lindsay ME, Isselbacher EM Tags: Ann Thorac Surg Source Type: research

Pulmonary involvement in patients with Marfan Syndrome.
CONCLUSION: In the absence of early respiratory symptoms, pulmonary abnormalities should be detected and monitored before they aggravate. Particular attention should be paid to prevent pneumothorax. Our results support the importance of lung volume determination to identify patients in which pulmonary parenchyma require a careful chest CT evaluation. PMID: 24994580 [PubMed - indexed for MEDLINE]
Source: Panminerva Medica - June 4, 2015 Category: Journals (General) Tags: Panminerva Med Source Type: research

Distinct effects of losartan and atenolol on vascular stiffness in Marfan syndrome
We conducted a randomized, double-blind trial of losartan (100 mg QD) versus atenolol (50 mg QD) for 6 months in adults with Marfan syndrome. Carotid-femoral pulse wave velocity (PWV), central augmentation index (AIx), aortic diameter and left ventricular (LV) function were assessed with arterial tonometry and echocardiography. Thirty-four subjects (18 female; median age 35 years, IQR 27, 45) were randomized. Central systolic and diastolic blood pressure decreased comparably with atenolol and losartan (p = 0.64 and 0.31, respectively); heart rate decreased with atenolol (p = 0.02), but not with losartan. PWV decreased in p...
Source: Vascular Medicine - August 7, 2015 Category: Internal Medicine Authors: Bhatt, A. B., Buck, J. S., Zuflacht, J. P., Milian, J., Kadivar, S., Gauvreau, K., Singh, M. N., Creager, M. A. Tags: Original Articles Source Type: research

A Woman With Marfan Syndrome in Pregnancy: Managing High Vascular Risk With Multidisciplinary Care.
CONCLUSION: Having optimal maternal and neonatal outcomes for pregnant women with Marfan syndrome depends on a highly responsive and coordinated team effort, including meticulous planning for a vascular catastrophe. PMID: 26474229 [PubMed - in process]
Source: Journal of Obstetrics and Gynaecology Canada : JOGC - October 18, 2015 Category: OBGYN Tags: J Obstet Gynaecol Can Source Type: research

Acroangiodermatitis (Pseudo-Kaposi's Sarcoma) Seen in Conjunction with Marfan Syndrome and a Hypercoagulable State: Is There a Link?
Authors: Vazales R, Snyder RJ Abstract A case study of acroangiodermatitis is presented in conjunction with chronic venous disease, Marfan syndrome, and a hypercoagulable state. A possible link between acroangiodermatitis and Marfan syndrome is explored. PMID: 25860331 [PubMed]
Source: Wounds - November 30, 2015 Category: Journals (General) Tags: Wounds Source Type: research

Neonatal Marfan Syndrome: Report of a Case with an Inherited Splicing Mutation outside the Neonatal Domain
We report a child and her mother affected by Marfan syndrome. The child presented with a phenotype of neonatal Marfan syndrome, revealed by acute and refractory heart failure, finally leading to death within the first 4 months of life. Her mother had a common clinical presentation. Genetic analysis revealed an inherited FBN1 mutation. This intronic mutation (c.6163+3_6163+6del), undescribed to date, leads to exon 49 skipping, corresponding to in-frame deletion of 42 amino acids (p.Ile2014_Asp2055del). FBN1 next-generation sequencing did not show any argument for mosaicism. Association in the same family of severe neonatal ...
Source: Molecular Syndromology - February 1, 2016 Category: Molecular Biology Source Type: research