Serum cholesterol and triglyceride levels in progeria as a model of ageing

Szamosi, Tamás; Szollár, J; Meggyesi, Vera; Wilhelm, O; Bodánszky, H; Mátyus, József

doi:10.1016/0047-6374(84)90024-1

Cited by 10 publications

(2 citation statements)

References 15 publications

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“…However, most progeric subjects are not hypercholesterolemic in the conventional sense [3,33], and none exhibited the clinical features of monogenic hypercholesterolemia. Szamosi et al noted in two progeric brothers that the high-density lipoprotein cholesterol level was extremely low, and that of their parents was low [34]. The authors purported that these ¢nd-ings in homozygous children may explain the development of very early atherosclerosis described in progeric subjects and suggested progeria as a useful model in the study of the role of lipid metabolism in normal aging.…”

Section: Discussionmentioning

confidence: 94%

Hutchinson-Gilford Progeria Syndrome: A Pathologic Study

Ackerman

Gilbert‐Barness

2002

Pediatric Pathology & Molecular Medicine

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Hutchinson-Gilford progeria syndrome is an extremely rare condition with features of premature and accelerated aging.The pattern of inheritance is unclear, although both autosomal recessive and autosomal dominant modes have been proposed. The children usually present in late infancy and early childhood with a characteristic phenotype of alopecia; short stature; abnormal skin, teeth, and nails; beaked nose; loss of subcutaneous fat; and failure to thrive.This condition has been reported on all inhabited continents and has been described in all major races. Laboratory ¢ndings note an increased urinary excretion of hyaluronic acid. Death results from cardiovascular abnormalities in the majority of cases and usually occurs in the second decade of life. There is no e¡ective treatment.We report the pathologic changes noted at autopsy on a 20-year-old woman with classic features of Hutchinson-Gilford progeria syndrome.

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