The individual forms of inherited immunodeficiency diseases are rare and the true incidence in the United Kingdom is not known. In countries where immunodeficiency registers have been established (Australia, Japan, Spain, and Sweden) an overall incidence of appreciable symptomatic antibody de- ficiency has been shown to be 1/1 1 000 live births, and that of severe combined immunodeficiency to be 14/million live births. In the United Kingdom many of the severest forms are not diagnosed until several early deaths have occurred in a family, but with increasing awareness among paediatricians and with better laboratory facilities more of these children will be identified.
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Lau et al. (1988) studied this question.
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