SUMMARY A pair of monozygous twins discordant for Wiedemann-Beckwith syndrome is described and published reports reviewed to establish the mode of inheritance. Single gene control seems unlikely and it is proposed that the condition arises from the interaction of 'at risk' genes in the fetus and metabolic factors in the mother, which suggests multifactorial control.The Wiedemann-Beckwith syndrome (WBS), also called exomphalos-macroglossia-gigantism syndrome, was first described in 1964 by Wiedemann," and his reported family included three affected sibs.

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