inheritance was suggested by the report from Egypt of affected sisters, children of first cousins. Paterson, in 1922 recorded the cases of two possibly affected brothers, photographs were not published and the diagnosis is not completely certain. The full report was simply the following: A boy, aged 8 years. Condition has been present since birth. The father and mother are first cousins. There are 4 children in the family, the girls are unaffected, both boys are affected. The senile condition of the skin and facies should be noted. The vessels show arteriosclerosis.(There is almost complete absence of subcutaneous fat.). Erecinski described photographically typical progeria in 2 brothers, and among the 9 offspring of 2 sisters, Rava in1967 found 6 affected. Khalifa in1989 described a consanguineous Libyan family in which 2 males and 1 female in 2 sibships related as cousins had seemingly typical Hutchinson-Gilford progeria. Repeated nonhealing fractures were the presenting manifestation in the proband. Maciel in1988 reported an inbred Brazilian family in which presumed Hutchinson-Gilford progeria had occurred in members of 2 sibships related as first cousins once removed. Although autosomal recessive inheritancewas unmistakable, it was by no means certain that this involved true progeria. The two brothers reported as having progeria by Parkash in1990 probably had mandibuloacral dysplasia. In 1972 DeBusk maintained that of 19 cases reported to that date in which consanguinity was sought, in only 3 were the parents related. Conceivably progeria is a dominant and the rare instances of affected sibs are the result of germinal mosaicism. In 1990 Fatunde described a family in which 3 of 6 sibs had progeria. A seventh sib, who had died before the time of study, may have been affected. In 1972 DeBusk and Jones reported a paternal age effect, supporting autosomal dominant inheritance. In 20 cases in which parental age was known, the mean paterna...
