Echocardiographic assessment of cardiovascular abnormalities in the Marfan syndrome

1983 ◽  
Vol 74 (3) ◽  
pp. 465-474 ◽  
Author(s):  
Patricia C. Come ◽  
Nicholas J. Fortuin ◽  
Robert I. White, ◽  
Victor A. McKusick
Keyword(s):  
Marfan Syndrome ◽  
Cardiovascular Abnormalities ◽  
Echocardiographic Assessment

Echocardiographic Assessment of Cardiovascular Abnormalities in Patients and Their Families with Marfan's Syndrome

1986 ◽  
Vol 3 (3) ◽  
pp. 255-260
Author(s):  
CHIN C. CHEN ◽  
CHIH W. PAN ◽  
SHIH P. WANG ◽  
TSUI L. HSU ◽  
MING C. HSIUNG ◽  
...  
Keyword(s):  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Cardiovascular Abnormalities ◽  
Echocardiographic Assessment

Banded aggregates containing fibrillin are present in the cartilage matrix adjacent to chondrocytes in individuals affected with scoliosis

1992 ◽  
Vol 50 (1) ◽  
pp. 892-893
Author(s):  
Douglas R. Keene ◽  
Magaret Fairhurst ◽  
Catherine C. Ridgway ◽  
Lynn Y. Sakai
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Cross Section ◽  
Immunoelectron Microscopy ◽  
Cartilage Matrix ◽  
Negative Stain ◽  
Gene Coding ◽  
Rotary Shadowing

Matrix microfibrils are present in the connective tissue matrices of all tissues. Following standard TEM processing, they appear in cross section as cylindrical fibrils 8-10 nm in diameter, often associated with amorphous elastin. They are also seen in the absence of amorphous elastin, for example in the shallow papillary layer of skin, and also in cartilage matrix (Figure 1). Negative stain and rotary shadowing studies suggest that microfibrils are composed of laterally associated globular structures connected by fine filamentous strands (“ beaded strings”), and that they are extendable. Immunoelectron microscopy has demonstrated that fibrillin, a 350 Kd glycoprotein, is distributed along all microfibrils with a relaxed periodicity of about 54 nm The gene coding for fibrillin has recently been identified and is defective in the Marfan syndrome.

Marfan Syndrome Update

2011 ◽  
Vol 44 (8) ◽  
pp. 54-55
Author(s):  
HOWARD P. LEVY
Keyword(s):  
Marfan Syndrome
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