Imaging-Based 4D Aortic Pressure Mapping in Marfan Syndrome Patients: A Matched Case-Control Study (Commentary)

Publication date: Available online 13 December 2019Source: The Annals of Thoracic SurgeryAuthor(s): Marc R. Moon
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research

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Publication date: February 2020Source: The Annals of Thoracic Surgery, Volume 109, Issue 2Author(s): Sherene Shalhub, Mary J. Roman, Kim A. Eagle, Scott A. LeMaire, Qianzi Zhang, Artur Evangelista, Dianna M. Milewicz, Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) ConsortiumBackgroundTo investigate clinical course of patients with type B aortic dissection (TBAD) occurring at a young age with confirmed or suspected heritable thoracic aortic disease.MethodsIndividuals with TBAD occurring at an age
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
ConclusionAlthough compound heterozygosity or homozygosity is rare in MFS, it should be considered when there is an unusually severe phenotype in a subset of family members.
Source: Molecular Genetics & Genomic Medicine - Category: Genetics & Stem Cells Authors: Tags: CLINICAL REPORT Source Type: research
CONCLUSIONS: All together, our results suggest sterile inflammation as a novel paradigm to disease progression, and we identify, for the first time, monocytes as a viable candidate for targeted therapy in MVD. PMID: 31928435 [PubMed - in process]
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
Jeremy Norton, from Wigston, Leicestershire, has Marfan syndrome, where the connective tissue throughout the body is too fragile. As a result, the walls of the aorta can become weakened.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
Marfan Syndrome (MFS) is a heritable connective tissue disorder with a high degree of clinical variability including respiratory diseases; a rare case of MFS with massive intrathoracic bleeding has been report...
Source: BMC Pulmonary Medicine - Category: Respiratory Medicine Authors: Tags: Case report Source Type: research
CONCLUSION: The development of the third episode of pneumothorax led to the diagnosis of Marfan's syndrome thanks to the patient's combination of symptoms. To avoid diagnostic delay, criteria were established in 1996 and revised in 2010 to ensure an early diagnosis of this condition. PMID: 31883816 [PubMed - as supplied by publisher]
Source: Revue des Maladies Respiratoires - Category: Respiratory Medicine Tags: Rev Mal Respir Source Type: research
In conclusion, in human MFS aortic SMCs: (a) ERK activation is enhanced but not specific to the site of aneurysm formation; (b) ERK opposes TGF-β-dependent negative effects on SMC phenotype; (c) multiple distinct SMC subtypes contribute to a 'mixed' contractile-synthetic phenotype in MFS aortic aneurysm; and (d) ERK drives Notch3 overexpression, a potential pathway for tissue remodelling in response to aneurysm formation. PMID: 31886938 [PubMed - as supplied by publisher]
Source: J Cell Mol Med - Category: Molecular Biology Authors: Tags: J Cell Mol Med Source Type: research
AbstractPregnant patients with Marfan syndrome (MFS) are at high risk of developing aortic dissection or rupture during the third trimester and early postpartum period. This increased likelihood is the consequence of the hyperdynamic and hypervolemic cardiocirculatory state and/or pregnancy ‐mediated structural changes of the arterial wall in response to hemodynamic and hormonal changes. In this article, we report on the case of a 26‐year‐old pregnant woman with MFS in the 30th gestation week, who presented with type A aortic dissection at the emergency department. According to t he multidisciplinary team decision, a...
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CASE REPORT Source Type: research
Publication date: Available online 18 December 2019Source: Stem Cell ResearchAuthor(s): Baihui Ma, Mingyao Luo, Hang Yang, Tianjiao Li, Weiwei Liu, Faxiang Xu, Chang Shu, Guokai Chen, Zhou ZhouAbstractThe human induced pluripotent stem cell line NCCDFWi001-A was derived from peripheral blood mononuclear cells (PBMC) of a 26-year-old female Marfan syndrome patient carrying two compound heterozygous variants FBN1c.2613A>C, (p.Leu871Phe) and c.684_736+4del. The established patient-derived iPSC showed expression of pluripotent stem cell markers and had the ability to differentiate into all of the three germ layers and posse...
Source: Stem Cell Research - Category: Stem Cells Source Type: research
CONCLUSIONS The high-density pedicle screw construct contributed to the significantly improved correction rate of thoracic curves in MF-S patients with large thoracic curves (≥70°). Additionally, increasing of pedicle screw number could help to enhance the structural stability and reduce the correction loss during the follow-up period. PMID: 31846451 [PubMed - in process]
Source: Medical Science Monitor - Category: Research Tags: Med Sci Monit Source Type: research
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