Cardiovascular event was the main cause of late death both for Marfan patients (80%; 4/5) and for non-Marfan patients (86%; 6/7). In conclusion, independent of the presence of Marfan syndrome, careful follow-up is necessary for patients with cystic medial necrosis of the aorta to ...
MFS is notable for its variability in age of onset, tissue distribution, and severity of clinical manifestations. neonatal Marfan syndrome (nMFS) is consid- ered the most severe end of the spectrum of type I fibrilli- nopathies, but diagnostic criteria are still debated. Indeed, Booms et al...
White, T. L.et al. The Structural Role of Elastic Fibers in the Cornea Investigated Using a Mouse Model for Marfan Syndrome.Invest Ophthalmol Vis Sci58, 2106–2116 (2017). ArticlePubMedPubMed CentralCASGoogle Scholar Guler, M.et al. Short-term effects of intravitreal bevacizumab on cornea a...
sudden cardiac death may occur when the heart's electrical signals become erratic (arrhythmias). When the heart tissue that is responsible for regular electrical stimulus of heart muscle contractions is damaged, the heart stops effectively pumping blood. Death usually occurs in a few minutes after t...
Genetic anticipation occurs characteristically in all the conditions except a. myotonia dystrophica b. spinocerebellar ataxia type 1 c. Marfan's syndrome d. Huntingdon's disease e. Fragile X syndrome Explain the differences in the given ...
infantile Marfan syndromegiant atrial dilatationsevere mitral regurgitationdysplastic mitral valve.Our aim is to report a severe case of Infantile Marfan syndrome, which presented to the Emergency Department for shortness of breath, intense pallor and loss of appetite. Her particular physiognomy made us...
As shown in Case 1, double valve replacement in an infant with neonatal Marfan syndrome is feasible but its benefit on long term is uncertain. Excluding our patient, 3 infants with nMFS from the literature died early after cardiac surgery. Our second case is a unique patient who survives ...
Tuende Kertess-SzlaninkaPediatric PracticeSonja DippacherMartin Hulpke-Wette胸外科期刊(英文)Buchhorn R, Kertess-Szlaninka T, Dippacher S, Hulpke-Wette M. Neonatal Marfan syndrome: improving the bad prognosis with a strict conservative treatment with carvedilol? Open J Thorac Surg 2014;4:44-7....
Aetiology was identified in 26 patients (76.5%): causes were cerebral amyloid angiopathy in 8, ischaemic stroke in 5, vasculitis in 4, reversible posterior encephalopathy in 2, venous thrombosis in 2, reversible cerebral vasoconstriction syndrome in 2, carotid occlusion in 1, Marfan syndrome in 1...
Marfan's syndrome and other known genetically determined aortopathies.2 The study by Detaint et al3 confirms previously forwarded evidence that (1) BAV-related aortopathy is remarkably heterogeneous in terms of both phenotype and prognosis and (2) it should not be equated with Marfan aortopathy...