Marfan Syndrome: The variability of operative management

JS Coselli, S Büket - Cardiovascular aspects of Marfan Syndrome, 1995 - Springer
Marfan syndrome is an autosomal dominant disorder of connective tissue with an incidence
of approximately 1 in 20 000 (1). The disorder is characterized by clinical manifestations in …

Recurrent and founder mutations in the Netherlands: extensive clinical variability in Marfan syndrome patients with a single novel recurrent fibrillin-1 missense …

JJJ Aalberts, AG Schuurman, G Pals, BJC Hamel… - Netherlands heart …, 2010 - Springer
Abstract Background/Methods. Marfan syndrome (MFS) is a heritable connective tissue
disorder usually caused by a mutation in the fibrillin 1 (FBN1) gene. Typical characteristics of …

Beneficial Outcome of Losartan Therapy Depends on Type of FBN1 Mutation in Marfan Syndrome

R Franken, AW den Hartog, T Radonic… - Circulation …, 2015 - Am Heart Assoc
Background—It has been shown that losartan reduces aortic dilatation in patients with
Marfan syndrome. However, treatment response is highly variable. This study investigates …

Pathophysiology and management of cardiovascular manifestations in Marfan and Loeys–Dietz syndromes

N Takeda, H Yagi, H Hara, T Fujiwara… - International heart …, 2016 - jstage.jst.go.jp
Marfan syndrome (MFS) is an autosomal dominant heritable disorder of connective tissue
that affects the cardiovascular, skeletal, ocular, pulmonary, and nervous systems and is …

[HTML][HTML] Clinical characteristics of Marfan syndrome in Korea

AY Lim, JS Song, EK Kim, SY Jang… - Korean circulation …, 2016 - synapse.koreamed.org
Background and Objectives Marfan syndrome (MFS) is a connective tissue disorder with
autosomal dominant inheritance and a highly variable clinical spectrum. However, there are …

[HTML][HTML] Strategies to prevent aortic complications in Marfan syndrome

L Sartor, A Forteza - Journal of Thoracic Disease, 2017 - ncbi.nlm.nih.gov
Marfan syndrome (MFS) is an autosomal dominant disorder of the body's connective tissue,
caused by mutations in the fibrillin-1 (FBN1) gene. The estimated prevalence of the …

A novel mutation in the neonatal region of the fibrillin (FBN) 1 gene associated with a classical phenotype of Marfan syndrome (MfS)

U Grau, HG Klein, C Detter, H Mair, A Welz… - Human …, 1998 - Wiley Online Library
Abstract Marfan Syndrome (MfS) is an autosomal dominant inherited connective tissue
disorder with variable phenotypic expression of cardiovascular, skeletal and ocular …

[HTML][HTML] Cardiovascular manifestations in Marfan syndrome

C Lazea, S Bucerzan, M Crisan… - Medicine and …, 2021 - ncbi.nlm.nih.gov
Marfan syndrome (MFS) is an autosomal dominant inherited disease of the connective
tissue with multiorgan involvement (skeleton, cardiovascular, eyes, skin, lungs) …

Marfan syndrome: prevalence and natural course of cardiovascular manifestations

Y Von Kodolitsch, M Raghunath… - Zeitschrift fur …, 1998 - europepmc.org
The Marfan syndrome is an autosomal dominant disorder of the connective tissue with
mutations on the fibrillin-1 gene encoding for fibrillin, a major component of the extracellular …

Marfan syndrome: an update of genetics, medical and surgical management

Y Von Kodolitsch, PN Robinson - Heart, 2007 - heart.bmj.com
Marfan syndrome is a heritable disorder of the connective tissue with an estimated
prevalence of 1 in 5000 individuals and no predilection for either sex. The syndrome is …