PEDIATRICS Vol. 43 No. 2 February 1969, pp. 207-216
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METABOLIC STUDIES IN TWO BOYS WITH CLASSICAL PROGERIA

Dorothy B. Villee M.D.1, George Nichols Jr. M.D.1, and Nathan B. Talbot M.D.1

1 Children's Service, Massachusetts General Hospital and the Departments of Pediatrics and Medicine, Harvard Medical School, Boston, Massachusetts

Two boys with classical progeria have been studied by a variety of laboratories in the Boston area. Evidence for unresponsiveness to growth hormone, relative insulin resistance, and highly cross-linked collagen is presented. The data support the concept that progeria represents a form of mesenchymal dysplasia in which connective tissue cells are unresponsive to growth influences. It is hypothesized that this failure to grow may account for the "older" collagen which may in turn produce structural abnormalities in vessel walls predisposing to atherosclerosis.

Submitted on July 1, 1968
Accepted on September 25, 1968




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