Progeria and Atherosclerosis

Keay, A. J.; Oliver, M.F.; Boyd, George S.

doi:10.1136/adc.30.153.410

Cited by 27 publications

(8 citation statements)

References 16 publications

(9 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Of the 36 cases reviewed by Ghosh and Varma (1964), raised serum cholesterol was reported in 14. Raised ,-lipoprotein levels have been reported in some cases (Keay, Oliver, and Boyd, 1955;Rosenthal et al, 1956), and Keay et al also found the ratio of cholesterol to phospholipid to be increased. In some patients, however, cholesterol and lipoproteins have been found to be normal (Villee et al, 1969;Kaiman, Lambie, and Metzl, 1969).…”

mentioning

confidence: 92%

Progeria: Case Report with Long-term Studies of Serum Lipids

Machamara¹,

Farn²,

Mitra³

et al. 1970

Archives of Disease in Childhood

View full text Add to dashboard Cite

A. S. (1970). Archives of Disease in Childhood, 45, 553. Progeria: case report with long-term studies of serum lipids. A girl with the typical features of progeria died at the age of 10 years from cardiac failure following repeated myocardial infarctions. Serial studies of the serum lipids were made from the age of 2 years 3 months. Variable hyperlipidaemia, increased f,-and pre-fl-ipoproteins, and impaired clearing of absorbed dietary fat were shown. A polyunsaturated fat diet, which was started at the age of 2 years 5 months and continued throughout the rest of her life, controlled the hyperlipidaemia and the clearing of fat became normal. Pre-,B-lipoprotein could still, however, be seen occasionally. The dietary regimen did not delay the development of atherosclerosis, the pathogenesis of which, in progeria, may be due to factors other than hyperlipidaemia.Progeria was first described by Hutchinson in 1886 and named by Hastings Gilford in 1897. The term means prematurely old. Individuals appear normal at birth and weight gain is usually satisfactory during the first year of life, but thereafter virtually ceases. Growth in height is retarded and rarely exceeds that of a normal 4-or 5-year-old. Intelligence is normal. The appearance has been likened to a 'plucked bird' or very old man because of baldness and lack of subcutaneous fat; the skin is atrophic and often shows brown pigmentation. The nails are atrophic or absent. The eyes are prominent and the nose beaked; the chin recessive and the maxilla hypoplastic. The chest is narrow and the abdomen protrudes. Clinical manifestations of osteoarthritis and arteriosclerosis may appear before the sixth year. The commonest cause of death is myocardial infarction and survival beyond the second decade is rare. The radiological features, which consist of hypoplastic facial bones, delay of cranial suture and fontanelle closure, thin short clavicles, coxa valgus, and progressive acro-osteolysis of the terminal phalanges have been recently reviewed by Margolin and Steinbach (1968), Ozonoff and Clemett (1967), and Macleod (1966.

show abstract

mentioning

confidence: 92%

Progeria: Case Report with Long-term Studies of Serum Lipids

Machamara¹,

Farn²,

Mitra³

et al. 1970

Archives of Disease in Childhood

View full text Add to dashboard Cite

show abstract

“…That aging is indeed an independent risk factor for coronary artery disease and stroke is perhaps best evident from patients suffering from Werner syndrome or Hutchinson-Gilford progeria. These patients experience much accelerated aging [84,89,135] and usually die within 20 years due to myocardial infarction or stroke [68,108]. A causal link between defective lamin genes and accelerated vascular aging in these conditions has been recently demonstrated [91,103].…”

Section: Current and Future World Demographics Of Aging And Obesitymentioning

confidence: 99%

Obesity and aging: determinants of endothelial cell dysfunction and atherosclerosis

Barton

2010

Pflugers Arch - Eur J Physiol

105

View full text Add to dashboard Cite

Endothelial cells are both the source and target of factors contributing to atherosclerosis. After the discovery of the endothelium-derived relaxing factor (EDRF) by Robert F. Furchgott in 1980 it soon became clear that endothelial cells also release vasoactive factors distinct from nitric oxide (NO) namely, endothelium-derived contracting factors (EDCF) as well as hyperpolarizing factors (EDHF). Vasoactive factors derived from endothelial cells include NO/EDRF, reactive oxygen species, endothelins and angiotensins which have either EDRF or EDCF functions, cyclooxygenase-derived EDCFs and EDRFs, and EDHFs. Endothelial factors are formed by enzymes such as NO synthase, cyclooxygenase, converting enyzmes, NADPH oxidases, and epoxigenases, among others, and participate in the regulation of vascular homeostasis under physiological conditions; however, their abnormal regulation due to endothelial cell dysfunction contributes to disease processes such as atherosclerosis, arterial hypertension, and renal disease. Because of recent changes in world demographics and the declining health status of the world's population, both aging and obesity as independent risk factors for atherosclerosis-related diseases such as coronary artery disease and stroke, will continue to increase in the years to come. Obesity and associated conditions such as arterial hypertension and diabetes are now also some of the primary health concerns among children and adolescents. The similarities of pathomechanisms activated in obesity and aging suggest that obesity-at least in the vasculature-can be considered to have effects consistent with accelerated, "premature" aging. Pathomechanisms as well as the clinical issues of obesity-and aging-associated vascular changes important for atherosclerosis development and prevention are discussed.

show abstract

“…Rosenthal et al [19] reported an abnormally high concentration of serum li poprotein in a case of progeria. Atherosclero sis was found in progeria as well as in Wer ner's syndrome [6].…”

Section: Discussionmentioning

confidence: 99%

A Disturbance of Serum Lipids in Three Cases of Werner’s Syndrome

Murata¹

1983

Gerontology

View full text Add to dashboard Cite

3 cases of Werner’s syndrome were studied on serum lipid metabolism in comparison with clinical manifestation. These 3 cases showed hyperlipemia all with hypertriglycemia and increased level of very low density lipoprotein. The lipoprotein lipase activity was shown after intravenous injection of heparin. These results indicate a possible pathogenic facet of Werner’s syndrome from the lipid metabolic point of view.

show abstract

Progeria and Atherosclerosis

Cited by 27 publications

References 16 publications

Progeria: Case Report with Long-term Studies of Serum Lipids

Progeria: Case Report with Long-term Studies of Serum Lipids

Obesity and aging: determinants of endothelial cell dysfunction and atherosclerosis

A Disturbance of Serum Lipids in Three Cases of Werner’s Syndrome

Contact Info

Product

Resources

About