l and maternal ages were 35.6 and 28.8 years respectively, and the median ages 31 and 28 respectively. In 7 U.S. cases, the mean paternal age was 37.1. Brown favored autosomal dominant inheritance (most cases resulting from new mutation) because of the paternal age effect, the low frequency of parental consanguinity, and the report of progeric monozygotic twins with 14 normal sibs. Ayres and Mihan suggested that a fault in vitamin E metabolism may be at the root of progeria and recommended vitamin E therapy for its antioxidant effect. In cultured skin fibroblasts of patients with progeria,Goldstein and Moermandemonstrated an increased fraction of heat-labile enzymes and other altered proteins. Freshly obtained cells, namely, erythrocytes, showed similar heat-lability of G6PD and 6-phosphogluconate dehydrogenases in a girl with progeria. Both parents showed intermediate values, consistent with recessive inheritance. The primary source of the multiple protein defects is unknown. Normal HLA antigens were found by Brown. Brown described identical twins with progeria who developed heart failure at the age of 8 and died within 1 month of each other. Cytogenetic analysis showed aninverted insertion in the long arm of chromosome 1 in 70% of cells. Brown suggested that a gene for progeria may be located on chromosome 1. Evidence for possible bioinactive growth hormone was presented with a suggestion of treatment of progeria with growth hormone. Signs of infant progeria, the Hutchinson-Gilford syndrome, appear at about age one, after an evidently normal infancy. Affected individuals seldom exceed the size of a normal 5-year-old, although they have the physical appearance of 60- year-old adults by the time they are 10. Many of the superficial aspects of aging, such as baldness, thinning of the skin, prominence of blood vessels of the scalp, and vascular diseases, occur. Sex organs remain small and underdeveloped. A few individuals with progeria ar...
