A typical case of progeria (Hutchinson-Gilford syndrome) is reported including the findings of the necropsy. Death occurred at the age of 11 years as the result of an accident. Analysis of cephalometric roentgenograms of this patient revealed that the characteristic facies of progeria results from marked retardation of facial growth in the presence of relatively normal growth of the neurocranium, and is therefore not the result of premature aging. Atherosclerosis was found in this child at necropsy. Concentrations of lipoproteins in the serum determined by analytical ultracentrifugation during life were found to be abnormally high, suggesting a metabolic error rather than premature aging of the arteries as the cause of the atherosclerosis. Psychologic studies showed the patient to be of normal intelligence with no evidence of senilism. Analysis of these data leads to the conclusion that premature aging does not occur in progeria.
- Received January 27, 1956.
- Accepted February 26, 1956.
- Copyright © 1956 by the American Academy of Pediatrics