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Ruptured abdominal aortic aneurysm in Marfan's syndrome. 1987

K Lafferty, and L McLean, and J Salisbury, and L T Cotton
Kings College Hospital, Denmark Hill, London, UK.

A rare case of ruptured abdominal aortic aneurysm in Marfan's syndrome is described. The patient was treated successfully with a straight aortic tube graft. Histology of the aneurysm wall showed changes typical of cystic medial necrosis.

UI MeSH Term Description Entries
D008297 Male Males
D008382 Marfan Syndrome An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2. Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001012 Aorta, Abdominal The aorta from the DIAPHRAGM to the bifurcation into the right and left common iliac arteries. Abdominal Aorta,Abdominal Aortas,Aortas, Abdominal
D001019 Aortic Rupture The tearing or bursting of the wall along any portion of the AORTA, such as thoracic or abdominal. It may result from the rupture of an aneurysm or it may be due to TRAUMA. Aortic Aneurysm, Ruptured,Ruptured Aortic Aneurysm,Aneurysm, Ruptured Aortic,Aneurysms, Ruptured Aortic,Aortic Aneurysms, Ruptured,Aortic Ruptures,Rupture, Aortic,Ruptured Aortic Aneurysms,Ruptures, Aortic

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