Marfan's Syndrome and Dissecting Aneurysm of Aorta

Atta, Antonio Gabriel

doi:10.1001/archinte.1961.03620110121016

Cited by 12 publications

(4 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Skeletal manifestations include tall stature, long limbs and phalanges (arachnodactyly). Cardiovascular complications, particularly aneurysmal dilatation of the ascending aorta and dissections, are a major cause of death in the adult (Atta and Hoch, 1961 ;McKusick, 1955). The aortic lesion is thought to result from elastic fibre degeneration in the tunica media, producing cystic foci commonly termed medionecrosis (Erdheim, 1930).…”

Section: Introductionmentioning

confidence: 99%

Fetal marfan syndrome: Prenatal ultrasound diagnosis with pathological confirmation of skeletal and aortic lesions

et al. 1981

View full text Add to dashboard Cite

Sonography was performed during the second trimester in two pregnancies at risk for the Marfan syndrome. In one, the limbs were significantly longer than expected ( p < 0.0025) and a diagnosis of the Marfan syndrome was established. Pathological examination of the fetus after pregnancy interruption showed typical foci of cystic medial necrosis in the ascending aorta.KEY WORDS Marfan, fetus Ultrasound Marfan Fetal Marfan

show abstract

Section: Introductionmentioning

confidence: 99%

Fetal marfan syndrome: Prenatal ultrasound diagnosis with pathological confirmation of skeletal and aortic lesions

et al. 1981

View full text Add to dashboard Cite

show abstract

“…Case 1. Female, age 15, with clinical findings of arachnodactyly, pigeon breast, ectopia lentis, aortic regurgitation and great stature (175 cm) and with autopsy findings of diffuse and dissecting aneurysm in the ascending aorta and marked hypertrophy and dilatation of the left ventricle (550 gm).…”

Section: Methodsmentioning

confidence: 99%

“…Marfan's syndrome as (1) conglomerate, disorganized masses of hyperplastic and hypertrophied smooth muscle cell fibers, and (3) formation of greatly dilated vascular channels. One way or the other, a series of these regressive changes are the cause of dissecting or diffuse aneurysm frequently seen in Marfan's syndrome.…”

Section: Introductionmentioning

confidence: 99%

“…In the present study, ultrastructural and histochemical observations were conducted for vascular change in Marfan's syndrome, particularly for the structure of media smooth muscle cells, elastic fibers, collagen fibers and ground substances (AMPS, etc), in comparison with that of normal aorta, arteriosclerosis, mesoaortitis ~yphilitica and aortitis syndrome (TAKAYASU'S disease). Case 1. Female, age 15, with clinical findings of arachnodactyly, pigeon breast, ectopia lentis, aortic regurgitation and great stature (175 cm) and with autopsy findings of diffuse and dissecting aneurysm in the ascending aorta and marked hypertrophy and dilatation of the left ventricle (550 gm).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

ULTRASTRUCTURAL and HISTOCHEMICAL STUDIES OF VASCULAR LESIONS IN MARFAN'S SYNDROME, WITH REPORT OF 4 AUTOPSY CASES

Takebayashi

Kubota

Takagi

1973

Acta Pathologica Japonica

View full text Add to dashboard Cite

Histochemical observation was conducted on arterial lesion in 4 cases of Marfan's syndrome and electron microscopic observation was also carried out on the aorta and gastroepiploic artery in 2 of the 4 cases. Acid mucopolysaccharide (AMPS) noted In cystic medionecrosis demonstrated meshwork of spheres and threads, and showed the same structure as the ground substances widely distributed in the mesenchymal connective tissues including normal arteries and as AMPS in cystic medionecrosis‐like lesions such as arteriosclerosis, mesoaortitis syphilitica, and aortitis syndrome (Takayasu's disease). In histochemical observation, there was found no distinct qualitative difference between AMPS in Marfan's syndrome and that in controls. The overaccumulation of AMPS is not a direct cause of vascular lesions in Marfan's syndrome. The pattern of degeneration of elastic fibers was peculiar to Marfan's syndrome but it also resembled senile change. Degeneration of elastic fibers was accompanied by regeneration in the same area. There was observed no electron microscopic anomaly in collagen fibers nor was found anomaly of fibrlllogenesis in media smooth muscle cells. From the point of view of cell pathology, attribution of the cause of vascular lesion merely to some enzyme deficiency results in much contradiction.

show abstract

Acute dissecting aneurysms of the aorta: diagnosis and treatment—1979

Wheat

1980

American Heart Journal

150

View full text Add to dashboard Cite

Marfan's Syndrome and Dissecting Aneurysm of Aorta

Cited by 12 publications

References 10 publications

Fetal marfan syndrome: Prenatal ultrasound diagnosis with pathological confirmation of skeletal and aortic lesions

Fetal marfan syndrome: Prenatal ultrasound diagnosis with pathological confirmation of skeletal and aortic lesions

ULTRASTRUCTURAL and HISTOCHEMICAL STUDIES OF VASCULAR LESIONS IN MARFAN'S SYNDROME, WITH REPORT OF 4 AUTOPSY CASES

Acute dissecting aneurysms of the aorta: diagnosis and treatment—1979

Contact Info

Product

Resources

About