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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2025 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.